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Martinez Villarreal JD, Cardenas-de la Garza JA, Ionescu MA, Tatu AL, Busila C, Mokni M, Medina LMS, Jasso SMD, Poletti ED, Tomecki KJ. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Current Management and Innovative Therapies. Int J Dermatol 2025. [PMID: 40231717 DOI: 10.1111/ijd.17768] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/05/2025] [Revised: 03/23/2025] [Accepted: 03/24/2025] [Indexed: 04/16/2025]
Abstract
There is no consensus regarding the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Supportive care remains the preferred approach internationally, while the efficacy of topical/systemic therapies is subject to controversy. We reviewed published guidelines and recommendations on SJS/TEN management and assessed supportive care involving topical and systemic therapies in a series of SJS patients. We included several specialty departments from different countries to determine the consensus in the management of SJS/TEN. We also included SJS patients and provided supportive care including silver nitrate at 0.5% sprayed on denuded areas, in conjunction with a single dose of subcutaneous etanercept. Based on our literature review and experience, we propose a management algorithm for SJS/TEN. Our review confirmed the importance of supportive care, including specific topical and systemic treatments, for SJS/TEN. Systemic corticotherapy, cyclosporine A, intravenous immunoglobulin, tumor necrosis factor-alpha (TNF-α) blockers, or combinations are subject to controversies. In our pilot series of seven adult patients with SJS, we obtained good clinical results within 1 week, with mucosal and skin clearance at Week 2, along with a good safety profile. This was achieved by spraying topical silver nitrate at a concentration of 0.5% on denuded areas, following a single dose of etanercept. This consensus led to the recommendation of a combination of supportive care along with several possible topical and systemic therapies for SJS/TEN. Corticosteroids, immunoglobulins, cyclosporine A, and TNF-alpha blockers were used in our centers alone or in combination, with good results associated with 0.5% topical silver nitrate. In our series of SJS patients, silver nitrate at 0.5% associated with etanercept showed a good response.
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Affiliation(s)
| | | | - Marius-Anton Ionescu
- Department of Dermatology, Paris Cité University, Hospital Saint Louis, Paris, France
| | - Alin Laurentiu Tatu
- Clinical Medical Department, Faculty of Medicine and Pharmacy, "Dunarea de Jos" University of Galati, Galati, Romania
- Clinical Hospital of Infectious Diseases Saint Parascheva, Galati, Romania
- Multidisciplinary Integrated Center for Dermatological Interfase Research, Galati, Romania
| | - Camelia Busila
- Clinical Medical Department, Faculty of Medicine and Pharmacy, "Dunarea de Jos" University of Galati, Galati, Romania
| | - Mourad Mokni
- Dermatology Department, Faculty of Medicine, University of Tunis, Tunis, Tunisia
| | - Luis Manuel Saenz Medina
- Internal Medicine Department, University Hospital "Jose Eleuterio Gonzalez", University Autonomous of Nuevo Leon, Monterrey, Mexico
| | | | - Eduardo David Poletti
- Internal Medicine and Dermatology, University Autonomous of Aguascalientes, Aguascalientes, Mexico
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Barreto F, Jardim M, Graça C, Catanho T, Nóbrega JJ. Toxic Epidermal Necrolysis: A Rare Complication of Allopurinol. Cureus 2025; 17:e76848. [PMID: 39897187 PMCID: PMC11787793 DOI: 10.7759/cureus.76848] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/03/2025] [Indexed: 02/04/2025] Open
Abstract
Toxic epidermal necrolysis (TEN) is a rare, life-threatening, mucocutaneous disorder characterized by extensive epidermal detachment and necrosis, often triggered by medications. Prompt recognition and management are critical to improving outcomes. The present article reports a case of an 80-year-old patient, with a history of gout and essential hypertension, who presented to the emergency department with pyrexia and a generalized macular rash characterized by dark centers and vesicles, accompanied by severe pruritus. The patient had recommenced allopurinol therapy one week prior, following an acute gout episode. Clinical examination revealed an extensively distributed, highly painful dark macular rash with cutaneous desquamation and a positive Nikolsky's sign, vesiculation, plasmorrhexis, and mucositis. Laboratory findings indicated elevated inflammatory markers. The patient was placed in isolation due to suspected TEN and managed with paraffin gauze dressings, temperature regulation, and fluid resuscitation. The condition was further complicated by refractory septic shock secondary to nosocomial pneumonia, ultimately resulting in the patient's demise. This case underscores the importance of early recognition, discontinuation of the causative agent, and comprehensive supportive care in the management of TEN. The case highlights the need for a multidisciplinary approach and the role of adjunctive therapies in improving outcomes. Further studies are warranted to optimize treatment protocols for this devastating condition.
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Affiliation(s)
- Francisco Barreto
- Internal Medicine Department, Hospital Central do Funchal, SESARAM EPERAM (SErviço de SAúde da Região Autónoma da Madeira, EPERAM), Funchal, PRT
| | - Margarida Jardim
- Intensive Care Department, Hospital Central do Funchal, SESARAM EPERAM (SErviço de SAúde da Região Autónoma da Madeira, EPERAM), Funchal, PRT
| | - Carina Graça
- Intensive Care Department, Hospital Central do Funchal, SESARAM EPERAM (SErviço de SAúde da Região Autónoma da Madeira, EPERAM), Funchal, PRT
| | - Tiago Catanho
- Intensive Care Department, Hospital Central do Funchal, SESARAM EPERAM (SErviço de SAúde da Região Autónoma da Madeira, EPERAM), Funchal, PRT
| | - José J Nóbrega
- Intensive Care Department, Hospital Central do Funchal, SESARAM EPERAM (SErviço de SAúde da Região Autónoma da Madeira, EPERAM), Funchal, PRT
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Mahajan A, Chen R, Fischer GM, Xiong Y, Ashrafzadeh S, Said JT, Nambudiri VE. Pembrolizumab-induced Stevens-Johnson syndrome-like reaction: An atypical clinical presentation. JAAD Case Rep 2024; 54:69-72. [PMID: 39654850 PMCID: PMC11626045 DOI: 10.1016/j.jdcr.2024.10.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2024] Open
Affiliation(s)
- Arjun Mahajan
- Department of Dermatology, Brigham and Women’s Hospital, Boston, Massachusetts
- Harvard Medical School, Boston, Massachusetts
| | - Ryan Chen
- Department of Dermatology, Brigham and Women’s Hospital, Boston, Massachusetts
- University of Massachusetts Medical School, Worcester, Massachusetts
| | - Grant M. Fischer
- Harvard Medical School, Boston, Massachusetts
- Department of Pathology, Brigham and Women’s Hospital, Boston, Massachusetts
| | - Yuqing Xiong
- Harvard Medical School, Boston, Massachusetts
- Department of Pathology, Brigham and Women’s Hospital, Boston, Massachusetts
| | - Sepideh Ashrafzadeh
- Department of Dermatology, Brigham and Women’s Hospital, Boston, Massachusetts
- Harvard Medical School, Boston, Massachusetts
| | - Jordan T. Said
- Department of Dermatology, Brigham and Women’s Hospital, Boston, Massachusetts
- Harvard Medical School, Boston, Massachusetts
| | - Vinod E. Nambudiri
- Department of Dermatology, Brigham and Women’s Hospital, Boston, Massachusetts
- Harvard Medical School, Boston, Massachusetts
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Anuntrangsee T, Chanprapaph K, Iamsumang W. D-Penicillamine-Induced Stevens-Johnson Syndrome in a Patient with Gold Cyanide Intoxication: A Case Report. Clin Cosmet Investig Dermatol 2024; 17:2409-2415. [PMID: 39494039 PMCID: PMC11529279 DOI: 10.2147/ccid.s489028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2024] [Accepted: 10/07/2024] [Indexed: 11/05/2024]
Abstract
D-penicillamine is used as the mainstay of chelation therapy for Wilson's disease and for heavy metal intoxication. D-penicillamine itself has been noted to cause several systemic side effects as well as symptoms related to the skin. Common cutaneous side effects such as acute hypersensitivity reactions, elastic fiber abnormalities, and bullous diseases have been occasionally described. Herein, we report a case of a 23-year-old Thai female with gold intoxication who developed Stevens-Johnson syndrome (SJS) following the treatment of D-penicillamine. To our knowledge, D-penicillamine-induced SJS is exceptionally rare. To raise awareness of potentially fatal cutaneous adverse drug reaction triggered by D-penicillamine, published literature regarding SJS induced by this agent has also been reviewed. D-penicillamine should be regarded as a possible culprit in patients presenting with SJS following D-penicillamine administration and should be promptly discontinued.
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Affiliation(s)
- Tanaporn Anuntrangsee
- Division of Dermatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Kumutnart Chanprapaph
- Division of Dermatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Wimolsiri Iamsumang
- Division of Dermatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
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Hasegawa A, Abe R. Stevens-Johnson syndrome and toxic epidermal necrolysis: Updates in pathophysiology and management. Chin Med J (Engl) 2024; 137:2294-2307. [PMID: 39238098 PMCID: PMC11441865 DOI: 10.1097/cm9.0000000000003250] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2024] [Indexed: 09/07/2024] Open
Abstract
ABSTRACT Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions characterized by extensive detachment of the epidermis and mucous membranes. These severe disorders carry a high mortality rate, and their pathogenesis remains largely unclear. Furthermore, optimal therapeutic strategies for SJS/TEN remain a subject of ongoing debate. Early diagnosis of SJS/TEN is challenging, and reliable biomarkers for diagnosis or severity prediction have not been firmly established. Certain drugs, such as carbamazepine and allopurinol, have shown a strong association with specific human leukocyte antigen (HLA) types. Recently, the potential benefits of HLA screening prior to administering these drugs to reduce the incidence of SJS/TEN have been explored. Epidermal cell death in SJS/TEN lesions is caused by extensive apoptosis, primarily through the Fas-Fas ligand (FasL) and perforin/granzyme pathways. Our findings suggest that necroptosis, a form of programmed necrosis, also contributes to epidermal cell death. Annexin A1, released from monocytes, interacts with the formyl peptide receptor 1 to induce necroptosis. Several biomarkers, such as CC chemokine ligand (CCL)-27, interleukin-15, galectin-7, receptor-interacting protein kinases 3 (RIP3), and lipocalin-2, have been identified for diagnostic and prognostic purposes in SJS/TEN. Supportive care is recommended for treating SJS/TEN, but the efficacy of various therapeutic options-including systemic corticosteroids, intravenous immunoglobulin, cyclosporine, and tumor necrosis factor-α antagonists-remains controversial. Recent studies have investigated the potential benefits of tumor necrosis factor-α antagonists. In this review, we discuss recent advances in the understanding and management of SJS/TEN.
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Affiliation(s)
- Akito Hasegawa
- Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata 951-8510, Japan
| | - Riichiro Abe
- Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata 951-8510, Japan
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Zhang X, Huang D, Lou D, Si X, Mao J. Stevens-Johnson Syndrome/Toxic epidermal necrolysis complicated with fulminant type 1 diabetes mellitus: a case report and literature review. BMC Endocr Disord 2024; 24:172. [PMID: 39218880 PMCID: PMC11367887 DOI: 10.1186/s12902-024-01683-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/15/2024] [Accepted: 08/08/2024] [Indexed: 09/04/2024] Open
Abstract
BACKGROUND Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening skin lesion triggered by hypersensitive drug reaction. They are characterized by extensive epidermal necrosis and skin exfoliation. Fulminant type 1 diabetes mellitus (FT1DM) is featured by a rapid-onset of hyperglycemia with ketoacidosis due to severely destroyed β-cell function. Fulminant type 1 diabetes mellitus as a sequela of SJS/TEN has rarely been reported. CASE PRESENTATION We present a 73-year-old female patient who developed SJS/TEN skin allergic reaction after taking carbamazepine and phenytoin for 35 days. Then, hyperglycemia and diabetic ketoacidosis occurred 20 days after discontinuation of antiepileptic drugs. A very low serum C-peptide level (8.79 pmol/l) and a near-normal glycosylated hemoglobin level met the diagnostic criteria for fulminant T1DM. Intravenous immunoglobulin (IVIG) and insulin were promptly administered, and the patient recovered finally. CONCLUSIONS This rare case indicates that monitoring blood glucose is necessary in SJS/TEN drug reaction, and comprehensive therapy with rehydration, insulin, antibiotics, and IVIG may improve the prognosis.
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Affiliation(s)
- Xiaofang Zhang
- Department of Endocrinology and Metabolism, Shaoxing People's Hospital, Zhejiang Province, China, 312000
| | - Dihua Huang
- Department of Endocrinology and Metabolism, Shaoxing People's Hospital, Zhejiang Province, China, 312000
| | - Dajun Lou
- Department of Endocrinology and Metabolism, Shaoxing People's Hospital, Zhejiang Province, China, 312000
| | - Xuwei Si
- Department of Endocrinology and Metabolism, Shaoxing People's Hospital, Zhejiang Province, China, 312000
| | - Jiangfeng Mao
- Department of endocrinology, Peking Union Medical College Hospital, Beijing, China, 100730.
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Foo VHX, Yueh LH, Mehta JS, Ong HS. Acute and chronic ocular outcomes in SJS/TEN patients treated with oral ciclosporin vs intravenous immunoglobulin. Front Med (Lausanne) 2024; 11:1398506. [PMID: 39224611 PMCID: PMC11368074 DOI: 10.3389/fmed.2024.1398506] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2024] [Accepted: 07/29/2024] [Indexed: 09/04/2024] Open
Abstract
Background/Aim To evaluate differences in ocular complications of Stevens Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) patients receiving either systemic IVIG or Ciclosporin (CsA) as initial treatments. Methods Retrospective review of consecutive patients admitted for SJS/TEN at the Singapore General Hospital (SGH) from 2011 to 2017 who received either IVIG or Ciclosporin at the onset of the disease and had ophthalmological follow-up of at least 6 months were included. Acute ocular severity of SJS/TEN was graded using the Gregory grading score; chronic ocular complications were graded using the Sotozono system. Results A total of 18 subjects were included for analysis, with eight in the IVIG group and 10 in the CsA group. There were no significant differences in acute Gregory severity grading between the two groups. The CsA group had a trend towards worse overall chronic Sotozono grading scores compared to the IVIG group (median [IQR]: 2 [0-3] vs. 1 [0-6.5], p = 0.27), with a higher incidence of acute severe cornea involvement (60% vs. 25%, p = 0.93) and chronic corneal and eyelid involvement in the former than the latter. SJS/TEN patients with worse acute ocular involvement were more likely to have TEN and perianal mucosal involvement (50% vs. 0, p = 0.01). Conclusion Compared to those who received IVIG, SJS/TEN patients who received CsA at the acute disease stage, seemed to have worse acute corneal and chronic corneal and eyelid complications. Larger studies are needed to confirm this finding.
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Affiliation(s)
- Valencia Hui Xian Foo
- Corneal and External Diseases Department, Singapore National Eye Centre, Singapore, Singapore
- Tissue Engineering and Cell Therapy Department, Singapore Eye Research Institute, Singapore, Singapore
| | - Lee Haur Yueh
- Department of Dermatology, Singapore General Hospital, Singapore, Singapore
| | - Jodhbir S. Mehta
- Corneal and External Diseases Department, Singapore National Eye Centre, Singapore, Singapore
- Tissue Engineering and Cell Therapy Department, Singapore Eye Research Institute, Singapore, Singapore
- Ophthalmology and Visual Science Academic Clinical Research Program, Duke-NUS Medical School, Singapore, Singapore
- School of Material Science & Engineering and School of Mechanical and Aerospace Engineering, Nanyang Technological University, Singapore, Singapore
| | - Hon Shing Ong
- Corneal and External Diseases Department, Singapore National Eye Centre, Singapore, Singapore
- Tissue Engineering and Cell Therapy Department, Singapore Eye Research Institute, Singapore, Singapore
- Ophthalmology and Visual Science Academic Clinical Research Program, Duke-NUS Medical School, Singapore, Singapore
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Abedi F, Zarei B, Elyasi S. Albumin: a comprehensive review and practical guideline for clinical use. Eur J Clin Pharmacol 2024; 80:1151-1169. [PMID: 38607390 DOI: 10.1007/s00228-024-03664-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2023] [Accepted: 03/04/2024] [Indexed: 04/13/2024]
Abstract
PURPOSE Nowadays, it is largely accepted that albumin should not be used in hypoalbuminemia or for nutritional purpose. The most discussed indication of albumin at present is the resuscitation in shock states, especially distributive shocks such as septic shock. The main evidence-based indication is also liver disease. In this review, we provided updated evidence-based instruction for definite and potential indications of albumin administration in clinical practice, with appropriate dosing and duration. METHODS Data collection was carried out until November 2023 by search of electronic databases including PubMed, Google Scholar, Scopus, and Web of Science. GRADE system has been used to determine the quality of evidence and strength of recommendations for each albumin indication. RESULTS A total of 165 relevant studies were included in this review. Fluid replacement in plasmapheresis and liver diseases, including hepatorenal syndrome, spontaneous bacterial peritonitis, and large-volume paracentesis, have a moderate to high quality of evidence and a strong recommendation for administering albumin. Moreover, albumin is used as a second-line and adjunctive to crystalloids for fluid resuscitation in hypovolemic shock, sepsis and septic shock, severe burns, toxic epidermal necrolysis, intradialytic hypotension, ovarian hyperstimulation syndrome, major surgery, non-traumatic brain injury, extracorporeal membrane oxygenation, acute respiratory distress syndrome, and severe and refractory edema with hypoalbuminemia has a low to moderate quality of evidence and weak recommendation to use. Also, in modest volume paracentesis, severe hyponatremia in cirrhosis has a low to moderate quality of evidence and a weak recommendation. CONCLUSION Albumin administration is most indicated in management of cirrhosis complications. Fluid resuscitation or treatment of severe and refractory edema, especially in patients with hypoalbuminemia and not responding to other treatments, is another rational use for albumin. Implementation of evidence-based guidelines in hospitals can be an effective measure to reduce inappropriate uses of albumin.
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Affiliation(s)
- Farshad Abedi
- Department of Clinical Pharmacy, School of Pharmacy, Mashhad University of Medical Sciences, P.O. Box, Mashhad, 91775-1365, Iran
| | - Batool Zarei
- Department of Clinical Pharmacy, School of Pharmacy, Mashhad University of Medical Sciences, P.O. Box, Mashhad, 91775-1365, Iran.
| | - Sepideh Elyasi
- Department of Clinical Pharmacy, School of Pharmacy, Mashhad University of Medical Sciences, P.O. Box, Mashhad, 91775-1365, Iran.
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van Nispen C, Long B, Koyfman A. High risk and low prevalence diseases: Stevens Johnson syndrome and toxic epidermal necrolysis. Am J Emerg Med 2024; 81:16-22. [PMID: 38631147 DOI: 10.1016/j.ajem.2024.04.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2024] [Revised: 03/04/2024] [Accepted: 04/04/2024] [Indexed: 04/19/2024] Open
Abstract
INTRODUCTION Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions that carry a high rate of morbidity and mortality. OBJECTIVE This review highlights the pearls and pitfalls of SJS/TEN, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION SJS/TEN is a rare, delayed hypersensitivity reaction resulting in de-epithelialization of the skin and mucous membranes. The majority of cases are associated with medication or infection. Clinicians should consider SJS/TEN in any patient presenting with a blistering mucocutaneous eruption. Evaluation of the skin, mucosal, pulmonary, renal, genital, and ocular systems are essential in the diagnosis of SJS/TEN, as well as in the identification of complications (e.g., sepsis). Laboratory and radiological testing cannot confirm the diagnosis in the ED setting, but they may assist in the identification of complications. ED management includes stabilization of airway and breathing, fluid resuscitation, and treatment of any superimposed infections with broad-spectrum antibiotic therapy. All patients with suspected SJS/TEN should be transferred and admitted to a center with burn surgery, critical care, dermatology, and broad specialist availability. CONCLUSIONS An understanding of SJS/TEN can assist emergency clinicians in diagnosing and managing this potentially deadly disease.
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Affiliation(s)
- Christiaan van Nispen
- Department of Emergency Medicine, Brooke Army Medical Center, Fort Sam Houston, TX, United States of America.
| | - Brit Long
- Department of Emergency Medicine, Brooke Army Medical Center, Fort Sam Houston, TX, United States of America
| | - Alex Koyfman
- Department of Emergency Medicine, University of Texas Southwestern Medical Center, Dallas, TX, United States of America
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Green EA, Fogarty K, Ishmael FT. Penicillin Allergy: Mechanisms, Diagnosis, and Management. Med Clin North Am 2024; 108:671-685. [PMID: 38816110 DOI: 10.1016/j.mcna.2023.08.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/01/2024]
Abstract
Allergy to penicillin can occur via any of the 4 types of Gel-Coombs hypersensitivity reactions, producing distinct clinical histories and physical examination findings. Treatments include penicillin discontinuation, and depending on the type of reaction, epinephrine, antihistamines, and/or glucocorticoids. Most beta-lactams may be safely used in penicillin-allergic patients, with the possible exception of first-generation and second-generation cephalosporins. Penicillin testing includes skin testing, patch testing, and graded challenge. The selection of the type of testing depends on the clinical setting, equipment availability, and type of hypersensitivity reaction. Desensitization may be used in some cases where treatment with penicillins is essential.
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Affiliation(s)
- Estelle A Green
- Pennsylvania State University, College of Medicine University Park, 1850 East Park Avenue, State College, PA 16803, USA
| | - Kelan Fogarty
- Pennsylvania State University, College of Medicine University Park, 1850 East Park Avenue, State College, PA 16803, USA
| | - Faoud T Ishmael
- Pennsylvania State University, College of Medicine University Park, 1850 East Park Avenue, State College, PA 16803, USA; Mount Nittany Health, 1850 East Park Avenue, State College, PA 16803, USA.
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Tan YW, Liu LP, Zhang K. Double plasma molecular adsorption system for Stevens-Johnson syndrome/toxic epidermal necrolysis: A case report. World J Clin Cases 2024; 12:1371-1377. [PMID: 38524512 PMCID: PMC10955548 DOI: 10.12998/wjcc.v12.i7.1371] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/31/2023] [Revised: 01/19/2024] [Accepted: 02/08/2024] [Indexed: 02/29/2024] Open
Abstract
BACKGROUND Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are very serious skin allergies, with an etiology related to infections and medication. Since the coronavirus disease 2019 (COVID-19) pandemic, severe acute respiratory syndrome coronavirus-2 has also been considered to cause SJS/TEN. CASE SUMMARY We report the case of a woman in her thirties who took acetaminophen after contracting COVID-19. After 3 d of fever relief, she experienced high fever and presented with SJS/TEN symptoms, accompanied by intrahepatic cholestasis. Three days of corticosteroid treatment did not alleviate the skin damage; therefore, double plasma molecular adsorption system (DPMAS) therapy was initiated, with treatment intervals of 48 h. Her skin symptoms improved gradually and were resolved after seven DPMAS treatments. CONCLUSION DPMAS therapy is beneficial for abrogating SJS/TEN because plasma adsorption and perfusion techniques reduce the inflammatory mediators (e.g., tumor necrosis factor-alpha and interleukin-10 and-12) speculated to be involved in the pathology of the skin conditions.
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Affiliation(s)
- You-Wen Tan
- Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
| | - Li-Ping Liu
- Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
| | - Kai Zhang
- Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
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Enescu CD, Elder AJ, Deirawan H, Moossavi M. To Debride or Not to Debride: A Review of Wound Management for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Cureus 2024; 16:e55350. [PMID: 38559535 PMCID: PMC10981949 DOI: 10.7759/cureus.55350] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/29/2024] [Indexed: 04/04/2024] Open
Abstract
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immune-mediated skin reactions with high mortality as a result of severely compromised skin barrier function. Currently, there is no consensus on the topical management of these conditions. Some advocate for surgical debridement of affected skin as a means of preventing infection and facilitating reepithelialization with synthetic and biological wound coverage. Others prefer a conservative approach that relies on leaving the blistered skin in situ. A consensus is lacking, primarily due to the rarity of the disease and the lack of high-quality evidence supporting one particular form of management. The goal of this review is to explore and compare the two treatment approaches for SJS and TEN, namely conservative management and surgical debridement.
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Affiliation(s)
- Christina D Enescu
- Department of Dermatology, Wayne State University School of Medicine, Detroit, USA
| | - Adam J Elder
- Department of Medical Education, Wayne State University School of Medicine, Detroit, USA
| | - Hany Deirawan
- Department of Dermatology, Wayne State University School of Medicine, Detroit, USA
| | - Meena Moossavi
- Department of Dermatology, Wayne State University School of Medicine, Detroit, USA
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Ozhathil DK, Powell CM, Corley CV, Golovko G, Song J, El Ayadi A, Wolf SE, Kahn SA. A retrospective analysis of mortality risk and immunosuppressive therapy for Stevens-Johnson Syndrome and toxic epidermal necrolysis syndrome using the TriNetX research network. Burns 2024; 50:75-86. [PMID: 37734977 DOI: 10.1016/j.burns.2023.08.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2023] [Revised: 07/17/2023] [Accepted: 08/16/2023] [Indexed: 09/23/2023]
Abstract
Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) exist on a spectrum of autoimmune conditions which cause epidermal detachment and keratinocyte necrosis. Due to the rare incidence of these conditions, a dramatic heterogeneity in treatment algorithms exists. To better appreciate pharmacologic immunosuppressive therapies' impact on survival, the authors queried a multi-institutional data network. Data for this study was extracted from TriNetX Research Network, a platform that contains ICD-9/ICD-10 coding data from a consortium of international healthcare organizations. Seventy-one institutions were queried to identify adult patients diagnosed with SJS, TEN or SJS-TEN Overlap. Cohorts were created based on the therapy received: systemic steroids (SS), diphenhydramine (DH), cyclosporine (CS), intravenous immunoglobulin (IVIG), tumor necrosis factor alpha inhibitors (TNFαi), or a combination of treatments. Cohorts were then propensity matched with patients who received supportive care. Patients who only received one of the above treatments showed no significant reduction in 90-day mortality. Patients who received CS or IVIG as part of their multitherapy showed a significantly increased risk of death when compared to supportive care (CS: RR = 1.583, 95% CI [1.119, 2.240]; IVIG: RR = 2.132, 95% CI [1.485, 3.059]). Despite their frequent utilization, this study's analysis suggests that none of these therapies confer significant 90-day mortality survival over supportive care alone. These results highlight the heterogeneity of therapies and emphasize the need for critical prospective appraisal of their outcomes in SJS and TEN.
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Affiliation(s)
- Deepak K Ozhathil
- Department of Surgery, Medical University of South Carolina, 96 Jonathen Lucas Street CSB 420 MSC 613, Charleston, SC 29425, USA.
| | - Carter M Powell
- Department of Surgery, Medical University of South Carolina, 96 Jonathen Lucas Street CSB 420 MSC 613, Charleston, SC 29425, USA
| | - Caroline V Corley
- Department of Surgery, Medical University of South Carolina, 96 Jonathen Lucas Street CSB 420 MSC 613, Charleston, SC 29425, USA
| | - George Golovko
- 301 University Boulevard, Department of Pharmacology, University of Texas Medical Branch at Galveston, Galveston, TX 77555, USA
| | - Juquan Song
- 301 University Boulevard, Department of Surgery, University of Texas Medical Branch at Galveston, Galveston, TX 77555, USA
| | - Amina El Ayadi
- 301 University Boulevard, Department of Surgery, University of Texas Medical Branch at Galveston, Galveston, TX 77555, USA
| | - Steven E Wolf
- 301 University Boulevard, Department of Surgery, University of Texas Medical Branch at Galveston, Galveston, TX 77555, USA
| | - Steven A Kahn
- Department of Surgery, Medical University of South Carolina, 96 Jonathen Lucas Street CSB 420 MSC 613, Charleston, SC 29425, USA
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14
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Fabian IM, Maddox K, Robicheaux C, Islam RK, Anwar A, Dorius B, Robinson CL, Kaye AM, Varrassi G, Ahmadzadeh S, Shekoohi S, Kaye AD. Stevens-Johnson Syndrome From Combined Allopurinol and Angiotensin-Converting Enzyme Inhibitors: A Narrative Review. Cureus 2024; 16:e51899. [PMID: 38333456 PMCID: PMC10850002 DOI: 10.7759/cureus.51899] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2023] [Accepted: 01/08/2024] [Indexed: 02/10/2024] Open
Abstract
Stevens-Johnson syndrome (SJS) is a severe and potentially debilitating skin reaction frequently related to medication use. Allopurinol and angiotensin-converting enzyme (ACE) inhibitors are commonly prescribed medications for prevalent health conditions worldwide, and their interaction associated with SJS warrants further investigation. A comprehensive literature search was performed to investigate cases as studies related to SJS occurring in patients with concomitant use of allopurinol and ACE inhibitors. We identified case reports and studies detailing hypersensitivity reactions, including SJS, attributed to a combination of allopurinol and ACE inhibitors. Despite the drug-drug interactions or lack thereof seen in patient populations, there is no definitive evidence of a pharmacokinetic interaction between allopurinol and ACE inhibitors. We were only able to find one case report specifically detailing SJS in a patient on combined ACE inhibitors and allopurinol. While the exact mechanism of the interaction is unclear, those reported cases of severe hypersensitivity reactions suggest a previous history of impaired renal function as a predisposing factor in the development of SJS. The potential risk of SJS with coadministration of ACE inhibitors and allopurinol is a drug-drug interaction that physicians should be aware of. This topic requires additional attention to determine if this drug combination should be avoided entirely in certain patients.
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Affiliation(s)
- Isabella M Fabian
- School of Medicine, Louisiana State University Health Sciences Center, Shreveport, USA
| | - Kirsten Maddox
- School of Medicine, Louisiana State University Health Sciences Center, Shreveport, USA
| | - Cameron Robicheaux
- School of Medicine, Louisiana State University Health Sciences Center, Shreveport, USA
| | - Rahib K Islam
- School of Medicine, Louisiana State University Health Sciences Center (LSUHSC) New Orleans, New Orleans, USA
| | - Ahmed Anwar
- Department of Psychology, Quinnipiac University, Hamden, USA
| | - Bradley Dorius
- Department of Anesthesiology, Louisiana State University Health Sciences Center, Shreveport, USA
| | - Christopher L Robinson
- Department of Anesthesiology, Critical Care, and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, USA
| | - Adam M Kaye
- Department of Pharmacy Practice, Thomas J. Long School of Pharmacy and Health Sciences University of the Pacific, Stockton, USA
| | | | - Shahab Ahmadzadeh
- Department of Anesthesiology, Louisiana State University Health Sciences Center, Shreveport, USA
| | - Sahar Shekoohi
- Department of Anesthesiology, Louisiana State University Health Sciences Center, Shreveport, USA
| | - Alan D Kaye
- Department of Anesthesiology, Louisiana State University Health Sciences Center, Shreveport, USA
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15
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Hao L, Cai W, Zeng Z, Geng X, Li Q, Chen H, Zhang Y, Ding J. Toxic epidermal necrolysis after injection of sclerosing agent and medical adhesive into oesophageal variceal ligation in a patient with a malignant liver tumour: A case report. Exp Ther Med 2024; 27:20. [PMID: 38125343 PMCID: PMC10728904 DOI: 10.3892/etm.2023.12309] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2022] [Accepted: 10/13/2023] [Indexed: 12/23/2023] Open
Abstract
Chronic liver disease can cause an increase in portal sinus pressure, which may lead to rupture and bleeding of esophageal and gastric varices. Oesophageal variceal ligation, with use of sclerosing agent and tissue glue injection is commonly used in clinical practice to address oesophageal bleeding. A 58-year-old male patient with chronic liver disease was treated with oesophageal variceal ligation, sclerosing agent and tissue glue injection due to oesophageal and gastric variceal bleeding. After 2 days, the skin of the patient exhibited erythema to different degrees. After 10 days of dexamethasone treatment, the whole-body rash worsened, and a severe skin reaction appeared that was suggestive of toxic epidermal necrolysis (TEN). Strict mucosal care was provided, and corticosteroids, γ globulin and adalimumab were concurrently used for treatment. After 20 days, the patient recovered from the skin problems. To the best of our knowledge, TEN after endoscopic surgery has rarely been reported in the relevant literature. Furthermore, when patients being treated with multiple drugs have erythema multiforme, physicians should be alert to the possibility of its development into TEN. The present case report summarizes the treatment methods for patients with TEN, providing a practical clinical basis and direction for the future diagnosis and treatment of the condition.
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Affiliation(s)
- Liangchao Hao
- Department of Dermatology, Shaoxing People's Hospital, Shaoxing, Zhejiang 312000, P.R. China
- Department of Plastic Surgery, Shaoxing People's Hospital, Shaoxing, Zhejiang 312000, P.R. China
| | - Wei Cai
- Department of Dermatology, Shaoxing People's Hospital, Shaoxing, Zhejiang 312000, P.R. China
| | - Zhaomu Zeng
- Department of Neurosurgery, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi 330000, P.R. China
| | - Xiuchao Geng
- School of Medicine, Taizhou University, Taizhou, Zhejiang 318000, P.R. China
| | - Qiang Li
- School of Medicine, Taizhou University, Taizhou, Zhejiang 318000, P.R. China
| | - Hong Chen
- Department of Dermatology, Shaoxing People's Hospital, Shaoxing, Zhejiang 312000, P.R. China
| | - Yuhao Zhang
- Department of Neurosurgery, Cancer Centre, Zhejiang Provincial People's Hospital (Affiliated People's Hospital), Hangzhou Medical College, Hangzhou, Zhejiang 310000, P.R. China
| | - Juan Ding
- Department of Dermatology, Shaoxing People's Hospital, Shaoxing, Zhejiang 312000, P.R. China
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16
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Martinez JD, Cardenas JA, Soria M, Saenz LM, Estrada K, Delgado SM, Ionescu MA, Busila C, Tatu AL. Role of Silver Nitrate Spray for Skin Wound Care in Patients with Toxic Epidermal Necrolysis: Our Experience in 4 Patients. Life (Basel) 2023; 13:2341. [PMID: 38137943 PMCID: PMC10744695 DOI: 10.3390/life13122341] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2023] [Revised: 12/04/2023] [Accepted: 12/12/2023] [Indexed: 12/24/2023] Open
Abstract
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are examples of severe cutaneous adverse reactions to drugs (SCARs) with several international recommendations for global medical management, ranging from pharmacological systemic therapy to skin wound care. There is no defined best management of the skin wounds in SJS/TEN. The care of wounds is essential to initiate re-epithelialization. Our objective is to improve the cicatrization process, avoiding scarring due to deepening of the wounds, as well as prevent infections, achieve pain control, and avoid loss of serum proteins, fluids, and electrolytes. In this retrospective case series, we highlight the value of systemic therapy and the use of silver nitrate for wound management in four patients with TEN.
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Affiliation(s)
- Jose Dario Martinez
- Department of Internal Medicine, Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico
| | - Jesus Alberto Cardenas
- Department of Rheumatology, Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico;
| | - Manuel Soria
- Dermatology Department, Hospital Civil, Guadalajara 44630, Mexico;
| | - Luis Manuel Saenz
- Medical Students, Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 64460, Mexico; (L.M.S.); (K.E.); (S.M.D.)
| | - Kattya Estrada
- Medical Students, Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 64460, Mexico; (L.M.S.); (K.E.); (S.M.D.)
| | - Sergio Maximo Delgado
- Medical Students, Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 64460, Mexico; (L.M.S.); (K.E.); (S.M.D.)
| | - Marius-Anton Ionescu
- Department of Dermatology, University Hospital Saint Louis, 75010 Paris, France;
| | - Camelia Busila
- “Sf. Ioan” Emergency Clinical Paediatric Hospital, 800179 Galati, Romania
- Clinical Medical Department, Faculty of Medicine and Pharmacy, “Dunarea de Jos” University, 800008 Galati, Romania;
| | - Alin Laurentiu Tatu
- Clinical Medical Department, Faculty of Medicine and Pharmacy, “Dunarea de Jos” University, 800008 Galati, Romania;
- Dermatology Department, “SfantaCuvioasaParascheva” Hospital of Infectious Diseases, 800179 Galati, Romania
- Multidisciplinary Integrated Center of Dermatological Interface Research MIC-DIR (Centrul Integrat Multidisciplinar de Cercetare de InterfataDermatologica—CIM-CID), “Dunărea de Jos” University, 800201 Galati, Romania
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17
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Martínez JD, Franco R, Sáenz LM, Alvarado AG, García JA, Delgado SM, Ionescu MA, Busilă C, Tatu AL. DRESS and Stevens-Johnson Syndrome Overlap Secondary to Allopurinol in a 50-Year-Old Man-A Diagnostic and Treatment Challenge: Case Report. Life (Basel) 2023; 13:2251. [PMID: 38137852 PMCID: PMC10744808 DOI: 10.3390/life13122251] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2023] [Revised: 11/13/2023] [Accepted: 11/23/2023] [Indexed: 12/24/2023] Open
Abstract
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a drug reaction commonly related to eosinophilia, from uncertain epidemiology, and without consensus for diagnosis and treatment globally. It presents a great challenge in its management and is characterized by fever, lymphadenopathy, skin rash, and multisystemic involvement. An aggressive and difficult-to-manage clinical case is presented in a 50-year-old man with chronic kidney disease due to diabetes mellitus type 2 and systemic arterial hypertension, who developed an unusual variant similar to DRESS and Stevens-Johnson syndrome (SJS) overlap secondary to allopurinol, with skin manifestations without eosinophilia, but fulfilling clinical and laboratory criteria for DRESS and SJS syndrome.
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Affiliation(s)
- José Dario Martínez
- Department of Internal Medicine, Faculty of Medicine, Hospital Universitario José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico;
| | - Rodolfo Franco
- Department of Human Pathology, Faculty of Medicine, Hospital Universitario José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico; (R.F.); (J.A.G.)
| | - Luis Manuel Sáenz
- Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico; (L.M.S.); (S.M.D.)
| | - Americo Guadalupe Alvarado
- Department of Internal Medicine, Faculty of Medicine, Hospital Universitario José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico;
| | - José Antonio García
- Department of Human Pathology, Faculty of Medicine, Hospital Universitario José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico; (R.F.); (J.A.G.)
| | - Sergio Máximo Delgado
- Faculty of Medicine, University Hospital José Eleuterio González, University Autonomous of Nuevo León, Monterrey 66455, Mexico; (L.M.S.); (S.M.D.)
| | - Marius-Anton Ionescu
- Department of Dermatology, University Hospital Saint Louis, 63110 Paris, France;
| | - Camelia Busilă
- Clinical Medical Department, Faculty of Medicine and Pharmacy, “Dunarea de Jos” University, 800008 Galati, Romania;
| | - Alin Laurentiu Tatu
- Clinical Medical Department, Faculty of Medicine and Pharmacy, “Dunarea de Jos” University, 800008 Galati, Romania;
- Dermatology Department, “Sfanta Cuvioasa Paraschiva” Hospital of Infectious Diseases, 800179 Galati, Romania
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18
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Shaw B, Madden M, Crespo A, Madruga M, Carlan SJ. A Rare Case of Severe Stevens-Johnson Syndrome Triggered by Topical Ofloxacin. AMERICAN JOURNAL OF CASE REPORTS 2023; 24:e941992. [PMID: 37952083 PMCID: PMC10654684 DOI: 10.12659/ajcr.941992] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2023] [Revised: 10/04/2023] [Accepted: 09/27/2023] [Indexed: 11/14/2023]
Abstract
BACKGROUND Stevens-Johnson syndrome (SJS) is a rare dermatologic disorder that is characterized by nonspecific flu-like prodrome with fever, malaise, myalgia, cough, rhinitis, and sore eyes, followed by a characteristic rash and mucocutaneous manifestations. It is triggered by medications in up to 80% of cases in adults. In each of these cases, the medication is oral or parenteral. Severe and progressive SJS can result in life-threatening complications. Adult-onset medication-induced SJS presents within 8 weeks of exposure to the offending substance, lasting 8 to 12 days. Recovery of denuded skin generally is complete within a month. There is no consensus on treatment, but supportive care with corticosteroids is often the initial intervention. CASE REPORT A 36-year-old woman with a flare of allergic rhinitis and tearing resistant to over-the-counter options was treated with topical ophthalmic ofloxacin. She began experiencing a diffuse mucocutaneous rash, with oral desquamation, tongue swelling, vaginal desquamation, and rash of the palms and soles within 24 h, which suggested the possibility of SJS. A skin biopsy was obtained, and pathology confirmed this suspicion. She was treated with parenteral antibiotics, corticosteroids, and supportive care, and after 10 days was discharged from the hospital. She had a complete recovery in 30 days. CONCLUSIONS The clinical course of SJS induced by the ophthalmic application of medication can be just as severe as the oral or parenteral routes. This is, to the best of our knowledge, the first documented case of SJS being triggered by topical ofloxacin.
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Affiliation(s)
- Brian Shaw
- Department of Internal Medicine, Orlando Regional Medical Center, Orlando, FL, USA
| | - Melinda Madden
- Department of Internal Medicine, Orlando Regional Medical Center, Orlando, FL, USA
| | - Antonio Crespo
- Division of Infectious Diseases, Orlando Regional Medical Center, Orlando, FL, USA
| | - Mario Madruga
- Department of Internal Medicine, Orlando Regional Medical Center, Orlando, FL, USA
| | - Stephen J. Carlan
- Division of Academic Affairs and Research, Orlando Regional Medical Center, Orlando, FL, USA
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19
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Puri P, Aslam ZM, Komal F, Prachi F, Sardana P, Chitkara A. Phenytoin-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Overlap in Focus: A Case Report. Cureus 2023; 15:e46075. [PMID: 37900419 PMCID: PMC10604504 DOI: 10.7759/cureus.46075] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/27/2023] [Indexed: 10/31/2023] Open
Abstract
Stevens-Johnson syndrome and toxic epidermal necrolysis overlap is a rare but severe cutaneous hypersensitivity reaction that can lead to death if not treated aggressively and adequately. Drug-induced hypersensitivity reactions are often related to drug exposure, with sulfonamides, anti-epileptics, fluoroquinolones, cephalosporins, and nonsteroidal anti-inflammatory drugs being the most common culprits. This case report describes a 10-year-old boy who was administered phenytoin at a local clinic to manage his seizures. This treatment led to the onset of SJS-TEN overlap, ultimately resulting in his demise.
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Affiliation(s)
- Piyush Puri
- Internal Medicine, Adesh Institute of Medical Sciences and Research, Bathinda, IND
| | | | - Fnu Komal
- Internal Medicine, HCA Houston Healthcare Northwest, Houston, USA
| | - Fnu Prachi
- Internal Medicine, Guru Teg Bahadur Hospital, Delhi, IND
| | - Princy Sardana
- Internal Medicine, Saraswathi Institute of Medical Sciences, Hapur, IND
| | - Akshit Chitkara
- Internal Medicine, University of California Riverside School of Medicine, Los Angeles, USA
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20
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Marquart E, Kinaciyan T. Overlapping clinical presentation of Mycoplasma-induced rash and mucositis and drug-induced Stevens Johnson Syndrome: A case report. IDCases 2023; 33:e01888. [PMID: 37693950 PMCID: PMC10484956 DOI: 10.1016/j.idcr.2023.e01888] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2023] [Revised: 08/26/2023] [Accepted: 08/27/2023] [Indexed: 09/12/2023] Open
Abstract
Introduction Mycoplasma pneumoniae is a worldwide occurring common bacterial agent for community-acquired pneumonia especially in children and young people with high contagiousness. Extrapulmonary complications such as cardiopulmonary, gastrointestinal, neurological and mucocutaneous manifestations including Mycoplasma pneumoniae-induced rash and mucositis (MIRM) may occur especially in adults. MIRM is an important differential diagnosis of Stevens Johnson Syndrome (SJS). Both clinically present similar as mucocutaneous erosive eruptions but have different etiologies. Case presentation We present an atypical case of a 36-year-old female with overlapping clinical features of MIRM and SJS. The patient presented to our allergy-outpatient clinic after recovering from mucocutaneous erosive eruptions and receiving an allergy-passport upon discharge for all drugs administered during the course of treatment including a subsequent ban of all beta-lactam antibiotics and NSAIDs for the future resulting in a desperate patient and treating physicians. A positive result of Mycoplasma pneumoniae in the sputum culture upon discharge was unnoticed. An allergological work-up with skin testing and drug provocation testing with the culprit drugs and safe alternatives was performed which resulted negative. Therefore, a new allergy passport was issued with drug alternatives that the patient may use in the future. A diagnosis of MIRM was subsequently made. Discussion The present case report depicts the diagnostic algorithm in an atypical case with overlapping clinical features of a MIRM and SJS. Conclusion Patients with atypical mucocutaneous eruptions of possible allergological etiology should receive a careful allergological work-up in an experienced tertiary referral center to reduce the number of inadequate allergy passport distribution.
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Affiliation(s)
- Elias Marquart
- Department of Dermatology, Medical University of Vienna, Vienna, Austria
| | - Tamar Kinaciyan
- Correspondence to: Department of Dermatology, Medical University of Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
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21
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Carvajal-Elizondo M, Monge-Ortega OP, Chinchilla-Monge R, Quiros S. [Stevens-Johnson syndrome in a patient with positive lymphocyte transformation test]. REVISTA ALERGIA MÉXICO 2023; 70:38-42. [PMID: 37566754 DOI: 10.29262/ram.v70i1.1047] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/22/2021] [Accepted: 04/04/2023] [Indexed: 08/13/2023] Open
Abstract
BACKGROUND Stevens-Johnson syndrome is a severe drug reaction. Sulfonamides have been associated with drug reactions, complications, sequelae, even death. CASE REPORT A 40-year-old female patient with a medical history of endometriosis and recently diagnosed chronic inflammatory ulcerative colitis. She was treated at the Allergology service of the San Juan de Dios Hospital of the Costa Rican Social Security Fund, and after 20 days of treatment with sulfasalazine she had a severe drug reaction on the skin, compatible with Stevens-Johnson syndrome. The lymphocyte transformation test was positive, confirming sulfasalazine as the causative agent. CONCLUSION The lymphocyte transformation test is a useful method that can confirm the causative agent and prevent important complications in the future.
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Affiliation(s)
| | | | | | - Steve Quiros
- Facultad de Microbiología, Universidad de Costa Rica
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22
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Tóth G, Lukács A, Schirra F, Sándor GL, Killik P, Maneschg OA, Nagy ZZ, Szentmáry N. Ophthalmic Aspects of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Narrative Review. Ophthalmol Ther 2023:10.1007/s40123-023-00725-w. [PMID: 37140876 PMCID: PMC10157599 DOI: 10.1007/s40123-023-00725-w] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2023] [Accepted: 04/19/2023] [Indexed: 05/05/2023] Open
Abstract
The aim of our review article was to summarize the current literature on Stevens-Johnson syndrome (SJS) and its severe form, toxic epidermal necrolysis (TEN). SJS/TEN is a serious, rare multi-system, immune-mediated, mucocutaneous disease with a significant mortality rate that can lead to severe ocular surface sequelae and even to bilateral blindness. Restoration of the ocular surface in acute and chronic SJS/TEN is challenging. There are only limited local or systemic treatment options for SJS/TEN. Early diagnosis, timely amniotic membrane transplantation and aggressive topical management in acute SJS/TEN are necessary to prevent long-term, chronic ocular complications. Although the primary aim of acute care is to save the life of the patient, ophthalmologists should regularly examine patients already in the acute phase, which should also be followed by systematic ophthalmic examination in the chronic phase. Herein, we summarize actual knowledge on the epidemiology, aetiology, pathology, clinical appearance and treatment of SJS/TEN.
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Affiliation(s)
- Gábor Tóth
- Dr. Rolf M. Schwiete Center for Limbal Stem Cell and Congenital Aniridia Research, Saarland University, Kirrberger Str. 100, 66424, Homburg/Saar, Germany.
- Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary.
| | - Andrea Lukács
- Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Utca 41, 1085, Budapest, Hungary
| | - Frank Schirra
- Argos Augenzentrum, Faktoreistraße 4, 66111, Saarbrücken, Germany
| | - Gábor L Sándor
- Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary
| | - Petra Killik
- Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary
| | - Otto A Maneschg
- Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary
| | - Zoltán Z Nagy
- Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary
| | - Nóra Szentmáry
- Dr. Rolf M. Schwiete Center for Limbal Stem Cell and Congenital Aniridia Research, Saarland University, Kirrberger Str. 100, 66424, Homburg/Saar, Germany
- Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary
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23
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Li J, Miller M, Abu Khalaf S, Nelson TB. Weeding Out the Culprit: Cannabinoid-Associated Stevens-Johnson Syndrome. Cureus 2023; 15:e39454. [PMID: 37362520 PMCID: PMC10289885 DOI: 10.7759/cureus.39454] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/24/2023] [Indexed: 06/28/2023] Open
Abstract
We describe a case of Stevens-Johnson syndrome (SJS) in a 32-year-old female who initially presented with a several-day history of worsening rash. Diagnosis of cannabinoid-associated SJS was established following skin biopsy and detailed history-taking of medication and other recreational drug usages. The patient was treated with pain management, antihistamines, and topical steroids with no complications following discharge. There currently exists limited literature describing SJS due to recreational drug usage.
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Affiliation(s)
- Jessie Li
- Medicine, University of Missouri, Columbia, USA
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24
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Allopurinol induced severe cutaneous adverse reactions: Vigilance of prescription (31 cases). REVUE FRANÇAISE D'ALLERGOLOGIE 2023. [DOI: 10.1016/j.reval.2022.103275] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
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25
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Green EA, Fogarty K, Ishmael FT. Penicillin Allergy. Prim Care 2023; 50:221-235. [PMID: 37105603 DOI: 10.1016/j.pop.2022.11.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/27/2023]
Abstract
Allergy to penicillin can occur via any of the 4 types of Gel-Coombs hypersensitivity reactions, producing distinct clinical histories and physical examination findings. Treatments include penicillin discontinuation, and depending on the type of reaction, epinephrine, antihistamines, and/or glucocorticoids. Most beta-lactams may be safely used in penicillin-allergic patients, with the possible exception of first-generation and second-generation cephalosporins. Penicillin testing includes skin testing, patch testing, and graded challenge. The selection of the type of testing depends on the clinical setting, equipment availability, and type of hypersensitivity reaction. Desensitization may be used in some cases where treatment with penicillins is essential.
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Affiliation(s)
- Estelle A Green
- Pennsylvania State University, College of Medicine University Park, 1850 East Park Avenue, State College, PA 16803, USA
| | - Kelan Fogarty
- Pennsylvania State University, College of Medicine University Park, 1850 East Park Avenue, State College, PA 16803, USA
| | - Faoud T Ishmael
- Pennsylvania State University, College of Medicine University Park, 1850 East Park Avenue, State College, PA 16803, USA; Mount Nittany Health, 1850 East Park Avenue, State College, PA 16803, USA.
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Surowiecka A, Barańska-Rybak W, Strużyna J. Multidisciplinary Treatment in Toxic Epidermal Necrolysis. INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH 2023; 20:2217. [PMID: 36767584 PMCID: PMC9916139 DOI: 10.3390/ijerph20032217] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/10/2023] [Accepted: 01/18/2023] [Indexed: 06/18/2023]
Abstract
Toxic epidermal necrolysis, Leyll's syndrome (TEN), is a rare mucocutaneous blistering disease burdened with high mortality rates. The diagnosis of TEN is based on clinical symptoms and histopathological findings. In approximately 90% of cases, it is a severe adverse reaction to drugs. In TEN, not only is the skin affected, but also mucosa and organs' epithelium. There are no unequivocal recommendations in regard to systemic and topical treatment of the patients. The aim of this paper is to review available literature and propose unified protocols to be discussed. Early management and multidisciplinary treatment are necessary to improve patients' outcome. Treatment of patients with TEN suspicions should be initiated with early drug withdrawal. TEN patients, like patients with burns, require intensive care and multidisciplinary management. Each patient with TEN should be provided with adequate fluid resuscitation, respiratory support, nutritional treatment, pain control, infection prophylaxis, anticoagulant therapy, and gastric ulcer prophylaxis. The key to local treatment of patients with TEN is the use of nonadherent dressings that do not damage the epidermis during the change. The aim of the systemic treatment is purification of the blood stream from the causative agent. The most efficient way to clarify serum of TEN patients' is the combination of plasmapheresis and IVIG. Immunomodulatory therapy can reduce the mortality five times in comparison with the patients with immunosuppression or lack of full protocol.
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Affiliation(s)
- Agnieszka Surowiecka
- East Center of Burns Treatment and Reconstructive Surgery, Medical University of Lublin, 20-059 Lublin, Poland
| | - Wioletta Barańska-Rybak
- Department of Dermatology, Venereology and Allergology, Faculty of Medicine, Medical University of Gdańsk, 80-210 Gdańsk, Poland
| | - Jerzy Strużyna
- East Center of Burns Treatment and Reconstructive Surgery, Medical University of Lublin, 20-059 Lublin, Poland
- Department of Plastic Surgery, Reconstructive Surgery and Burn Treatment, Medical University of Lublin, 20-059 Lublin, Poland
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Dastgheib L, Rostami F, Gharesi-Fard B, Asadi-Pooya AA, Namjoo S, Tahmasebi F, Hadibarhaghtalab M. Association of Human Leukocyte Antigen Alleles with Carbamazepine- or Lamotrigine-Induced Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in an Iranian Population: A Case-control Study. IRANIAN JOURNAL OF MEDICAL SCIENCES 2023; 48:70-76. [PMID: 36688194 PMCID: PMC9843459 DOI: 10.30476/ijms.2022.91192.2241] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Subscribe] [Scholar Register] [Received: 05/26/2021] [Revised: 11/21/2021] [Accepted: 01/30/2022] [Indexed: 01/24/2023]
Abstract
Background Genetic diversity in human leukocyte antigen (HLA) alleles across populations is a significant risk factor for drug-induced severe cutaneous adverse reactions (SCARs), e.g., carbamazepine (CBZ)- and lamotrigine (LTG)-induced Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). The present study aimed to investigate the frequency of different HLA alleles in Iranian patients with CBZ- and LTG-induced SJS/TEN. Methods A case-control study was conducted from 2011 to 2018 at various hospitals affiliated with Shiraz University of Medical Sciences (Shiraz, Iran). A total of 31 patients receiving anticonvulsant drugs (CZB or LTG) were recruited and divided into two groups. The drug-induced group (n=14) included hospitalized patients due to CBZ- or LTG-induced SJS/TEN. The drug-tolerant group (n=17) included individuals receiving CBZ or LTG for at least three months with no adverse effects. In addition, 46 healthy individuals (control group) were recruited. The frequency of HLA-A, -B, and -DRB1 alleles in patients with CZB- or LTG-induced SJS/TEN was investigated. HLA typing was performed using the allele-specific polymerase chain reaction method. The Chi square test and Fisher's exact test were used to determine a potential association between SJS/TEN and HLA alleles. P<0.05 was considered statistically significant. Results CBZ- or LTG-induced SJS/TEN was not significantly associated with HLA alleles. However, HLA-DRB1*01 showed a significantly higher frequency in patients with CBZ-induced SJS/TEN than the CBZ-tolerant patients (30% vs. 9%, P=0.07). Conclusion Overall, no significant association was found between CBZ- or LTG-induced SJS/TEN and HLA alleles. Further large-scale studies are required to substantiate our findings.
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Affiliation(s)
- Ladan Dastgheib
- Molecular Dermatology Research Center, Department of Dermatology, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Farima Rostami
- Student Research Committee, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Behrouz Gharesi-Fard
- Department of Immunology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Ali Akbar Asadi-Pooya
- Epilepsy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran,
Jefferson Comprehensive Epilepsy Center, Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA
| | - Saba Namjoo
- Blood Transfusion Research Center, High Institute for Education and Research in Transfusion Medicine, Tehran, Iran
| | - Foroozan Tahmasebi
- Department of Immunology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Maryam Hadibarhaghtalab
- Molecular Dermatology Research Center, Department of Dermatology, Shiraz University of Medical Sciences, Shiraz, Iran
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Wang S, Li T, Wang Y, Wang M, Liu Y, Zhang X, Zhang L. 5-Fluorouracil and actinomycin D lead to erythema multiforme drug eruption in chemotherapy of invasive mole: Case report and literature review. Medicine (Baltimore) 2022; 101:e31678. [PMID: 36451432 PMCID: PMC9704884 DOI: 10.1097/md.0000000000031678] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/05/2022] Open
Abstract
RATIONALE 5-Fluorouracil (5-FU) and actinomycin D (ActD) are often used in chemotherapy for various cancers. Side effects are more common in bone marrow suppression, liver function impairment, and gastrointestinal responses. Skin effects are rare and easy to be ignored by doctors and patients, which can lead to life-threatening consequence. PATIENT CONCERNS We reported a 45-year-old woman patient developed skin erythema and fingernail belt in chemotherapy of 5-FU and ActD. DIAGNOSIS Erythema multiforme drug eruption. INTERVENTIONS Laboratory tests including blood and urine routine, liver and kidney function, electrolytes and coagulation function and close observation. OUTCOMES The rash was gone and the nail change returned. LESSONS Delays in diagnosis or treatment may lead to serious consequence. We should pay attention to the dosage of 5-FU and ActD, monitor adverse reactions strictly, to reduce occurrence of skin malignant events.
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Affiliation(s)
- Shan Wang
- Departments of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China
| | - Tengfei Li
- Departments of General Surgery, The Second Hospital of Lanzhou University, Lanzhou, Gansu Province, China
| | - Yuan Wang
- Departments of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China
| | - Mengdi Wang
- Departments of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China
| | - Yibin Liu
- Departments of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China
| | - Xiaoguang Zhang
- Departments of Dermatology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China
| | - Lijuan Zhang
- Departments of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China
- * Correspondence: Lijuan Zhang, Departments of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China (e-mail: )
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Accuracy of SCORTEN in predicting mortality in toxic epidermal necrolysis. BMC Med Inform Decis Mak 2022; 22:273. [PMID: 36261833 PMCID: PMC9583545 DOI: 10.1186/s12911-022-02013-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2022] [Revised: 09/16/2022] [Accepted: 10/04/2022] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Toxic epidermal necrolysis (TEN) patients require multi-directional and multi-disciplinary treatment. In most cases, they are hospitalised at intensive care units and require multi-directional, burn-complication preventive care. Choosing the most appropriate treatment option might be troublesome even when predicting scores are used. SCORTEN is the most renowned prognostic score for TEN patients, however, there are some data indicating that the accuracy of this test may be limited. The credibility of not just the predicted mortality risk, but also componential laboratory results and clinical features subject to debate. The aim of this study was to evaluate the efficacy and credibility of SCORTEN in clinical practice, on proprietary material. METHODS A retrospective analysis of 35 patients with diagnosed in histopathology TEN was performed. The inclusion criteria were as follows: day of submission before 5th day from the onset of the symptoms, full protocol of plasmaphereses and IVIGs according to our scheme. Our protocol includes cycle of plasmapheresis with frozen fresh plasma twice daily for the first 2 days following admission, and once daily for the subsequent 5 to 7 days. IVIGs were administered after the first two sessions of plasmapheresis, for 4 to 7 days. The dosage was calculated according to body weight, at 0.4 to 0.5 g/kg per dose. RESULTS The sensitivity of SCORTEN for the analysed cohort was 100%, with a specificity of 24%. The estimated death was 41,9%, while the actual death rates were 12,5%. Our protocol improved the survival, OR = 26,57, RR = 6,34, p = 0,022. Decrease in mortality was caused by a combined treatment protocol we use- plasmaphereses with IVIGs. No independent risk factor was significant in death evaluation. CONCLUSION Our data suggest that the scoring system for predicting death among TEN patients are reliable when they are high. New prognostic factors should be found to improve the evaluation of patients with low SCORTEN.
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Borg L, Buhagiar M, La Ferla E, Pisani D, Said J, Boffa MJ. Pembrolizumab-Induced Toxic Epidermal Necrolysis. Case Rep Oncol 2022; 15:887-893. [PMID: 36825108 PMCID: PMC9941788 DOI: 10.1159/000526931] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2022] [Accepted: 08/30/2022] [Indexed: 11/19/2022] Open
Abstract
Immunotherapy has nowadays become part of the mainstay of treatment for several cancers. In this report, we present a case of toxic epidermal necrolysis (TEN) occurring as a relatively rare complication of pembrolizumab, which is a humanized monoclonal anti-programmed cell death-1 (anti-PD1) IgG4 antibody that targets tumours expressing PDL-1. In our case, this monoclonal antibody (MAB) was being used for the treatment of metastatic non-small cell lung cancer, before being withheld because of this potentially life-threatening reaction. There are no guidelines on the optimal management of TEN, with varying reports on the efficacy on steroids, intravenous immunoglobulin (IVIG), and cyclosporin. In this report, we describe a case of TEN following the second dose of three-weekly pembrolizumab in a 50-year-old man, comparing it to four similar cases reported in the literature. It is important that oncologists are aware of this potentially life-threatening adverse reaction of pembrolizumab, as early recognition and treatment of TEN are essential to improve clinical outcome.
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Affiliation(s)
- Luca Borg
- aDepartment of Medicine, Mater Dei Hospital, Msida, Malta,*Luca Borg,
| | - Malcolm Buhagiar
- bDepartment of Oncology, Sir Anthony Mamo Oncology Centre, Msida, Malta
| | - Elisa La Ferla
- aDepartment of Medicine, Mater Dei Hospital, Msida, Malta
| | - David Pisani
- cDepartment of Pathology, Mater Dei Hospital, Msida, Malta
| | - Janabel Said
- bDepartment of Oncology, Sir Anthony Mamo Oncology Centre, Msida, Malta
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O’Reilly P, Meskell P, Whelan B, Kennedy C, Ramsay B, Coffey A, Fortune DG, Walsh S, Ingen-Housz-Oro S, Bunker CB, Wilson DM, Delaunois I, Dore L, Howard S, Ryan S. Psychotherapeutic interventions for burns patients and the potential use with Stevens-Johnson syndrome and toxic epidermal necrolysis patients: A systematic integrative review. PLoS One 2022; 17:e0270424. [PMID: 35759493 PMCID: PMC9236256 DOI: 10.1371/journal.pone.0270424] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2021] [Accepted: 06/09/2022] [Indexed: 11/18/2022] Open
Abstract
Background
The existing evidence demonstrates that survivors of SJS/TEN have reported long-lasting psychological effects of their condition. Burns patients experience similar psychological effects. It is important to look at ways to help allay the psychological complications of SJS/TEN. As there is an absence of evidence on SJS/TEN psychotherapeutic interventions, it was judged to be beneficial to determine the evidence underpinning psychotherapeutic interventions used with burns patients.
Aims and objectives
The aim of this systematic integrative review was to synthesize the evidence relating to psychotherapeutic interventions used with adult burns patients and patients with SJS/TEN.
Method
The systematic review was guided by Whittemore and Knafl’s integrative review process and the PRISMA guidelines. Nine databases were searched for English and French language papers published January 2008 to January 2021. The protocol for the review was registered with PROSPERO.
Results
Following a screening process, 17 studies were included in the review. Two themes were identified using content analysis, (i) Empirically supported psychotherapeutic treatments, (ii) Alternative psychotherapeutic treatments. This review revealed no evidence on specific psychotherapeutic interventions for patients with SJS/TEN. Some of the interventions used with burns patients, viz. relaxation therapy, hypnosis and cognitive behavioral therapy showed some significant benefits. However, the evidence for burns patients is mainly focused on pain and pain anxiety as outcomes.
Conclusion
Following further research, some of the interventions deployed in burns patients may be applicable to SJS/TEN patients, particularly stress reduction techniques. In addition, the caring behaviours such as compassion, respect, and getting to know the patient as a person are important components to psychological care.
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Affiliation(s)
- Pauline O’Reilly
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- Health Research Institute, University of Limerick, Limerick, Ireland
- Health Implementation Science and Technology (HIST) Research Cluster, University of Limerick, Limerick, Ireland
- * E-mail:
| | - Pauline Meskell
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- Health Research Institute, University of Limerick, Limerick, Ireland
| | - Barbara Whelan
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- Health Research Institute, University of Limerick, Limerick, Ireland
| | - Catriona Kennedy
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- School of Nursing, Midwifery and Paramedic Practice, Robert Gordon University, Aberdeen, Scotland
| | - Bart Ramsay
- Charles Centre for Dermatology, University Hospital Limerick, ULHG, Limerick, Ireland
- School of Medicine, University of Limerick, Limerick, Ireland
| | - Alice Coffey
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- Health Research Institute, University of Limerick, Limerick, Ireland
- Health Implementation Science and Technology (HIST) Research Cluster, University of Limerick, Limerick, Ireland
| | - Donal G. Fortune
- Health Research Institute, University of Limerick, Limerick, Ireland
- Department of Psychology, University of Limerick, Limerick, Ireland
| | - Sarah Walsh
- Dermatology Department, King’s College Hospital, London, United Kingdom
| | - Saskia Ingen-Housz-Oro
- Dermatology Department, AP-HP, Henri Mondor Hospital, Créteil, France
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France
- Univ Paris Est Créteil EpiDermE, Créteil, France
| | - Christopher B. Bunker
- Department of Dermatology, University College London Hospitals NHS Foundation Trust, London, United Kingdom
| | - Donna M. Wilson
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada
| | - Isabelle Delaunois
- Regional Medical Library, University Hospital Limerick, Limerick, Ireland
| | - Liz Dore
- Glucksman Library, University of Limerick, Limerick, Ireland
| | - Siobhan Howard
- Health Research Institute, University of Limerick, Limerick, Ireland
- Department of Psychology, University of Limerick, Limerick, Ireland
| | - Sheila Ryan
- Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
- Charles Centre for Dermatology, University Hospital Limerick, ULHG, Limerick, Ireland
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Chi MH, Chung WH, Hui RCY, Chen CB, Lu CW, Chiu TM, Ma DHK, Wang CW, Yang CY. Clinical features and outcomes in children with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Dermatol 2022; 49:895-902. [PMID: 35715971 DOI: 10.1111/1346-8138.16476] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2021] [Revised: 04/08/2022] [Accepted: 05/10/2022] [Indexed: 11/29/2022]
Abstract
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening cutaneous conditions. However, studies of pediatric SJS/TEN are limited. To investigate the causes, clinical course, outcomes and complications of SJS and TEN in children. This retrospective study included 47 pediatric patients (aged < 18 years) with SJS, SJS/TEN, or TEN treated at Chang Gung Memorial Hospital, Taiwan, between January 2009 and December 2019. ALDEN scores and serological tests were used to assess causes and SCORTEN scores were applied to evaluate disease severity. Forty-seven patients, including 30 with SJS, 6 with SJS/TEN, and 11 with TEN were included. Median age was 8 years (range 1-17 years); 51.1% were male. Thirty-three cases (70.2%) were caused by drugs and infectious pathogens were suspected in 14 cases (29.8%). Oxcarbazepine (5/47, 10.6%) and amoxicillin (5/47, 10.6%) were the most often-implicated drugs, and Mycoplasma infection (9/47, 19.1%) was the predominant infectious cause. Only one TENS patient died (mortality rate 1/47, 2.1%) due to septic shock with ARDS, acute renal failure and cardiopulmonary shock. Median hospital stay was 15.5 (3-42) days. Pulmonary involvement (2/39, 5.1%), including pneumonia and ARDS, was noted in acute stage. Long-term sequelae were ocular involvement (6/39, 15.4%), nail dystrophy (4/39, 10.3%) and post-inflammatory hypo-/hyperpigmentation (3/39, 7.7%). In the present study, pediatric patients with SJS, SJS/TEN, or TEN have good outcomes with few long-term complications and low mortality. Mycoplasma is the most common infectious cause in pediatric SJS/TEN. Ocular discomfort, nail dystrophy and skin dyschromia are common long-term sequelae requiring regular follow-up.
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Affiliation(s)
- Min-Hui Chi
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Institute of Molecular Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Wen-Hung Chung
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Taiwan.,Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkuo, Taiwan.,Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | - Rosaline Chung-Yee Hui
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Taiwan.,Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkuo, Taiwan.,Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Taoyuan, Taiwan.,Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Chun-Bing Chen
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Taiwan.,Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkuo, Taiwan.,Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | - Chun-Wei Lu
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Taiwan.,Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkuo, Taiwan.,Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | - Tsu-Man Chiu
- Department of Dermatology, Changhua Christian Hospital, Changhua, Taiwan
| | - David Hui-Kang Ma
- Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | - Chuang-Wei Wang
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Taiwan.,Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkuo, Taiwan.,Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | - Chin-Yi Yang
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taipei,Linkuo and Keelung, Taiwan.,College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan.,Department of Dermatology, New Taipei Municipal TuCheng Hospital, New Taipei City, Taiwan
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Abuzneid YS, Alzeerelhouseini HIA, Rabi D, Hilail I, Rjoob H, Rabee A, Amro N, Qafisheh Q, Kharraz M. Carbamazepine Induced Stevens-Johnson Syndrome That Developed into Toxic Epidermal Necrolysis: Review of the Literature. Case Rep Dermatol Med 2022; 2022:6128688. [PMID: 35572158 PMCID: PMC9106509 DOI: 10.1155/2022/6128688] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2021] [Revised: 03/09/2022] [Accepted: 04/11/2022] [Indexed: 11/17/2022] Open
Abstract
Background Stevens-Johnson syndrome and toxic epidermal necrolysis are both skin diseases believed to be following the pattern of a type IV hypersensitivity mechanism, which can be triggered by infectious agents or administration of a variety of drugs as part of the spectrum of severe cutaneous adverse reactions (SCARs). Fever and blisters, that peel forming painful raw areas, are early symptoms of this condition, and complications such as dehydration, sepsis, pneumonia, and multiple organ failure are typically seen during the course of the disease. Case Presentation. We present a case of a 23-year-old female patient referred to our hospital after taking carbamazepine and developing high-grade fever and ulcers that appeared initially in her mouth and face but then progressed despite treatment, extending all over her body and involving about 90% of her BSA. Conclusion The use of IVIG and plasmapheresis was a good management for our case, helping in our patient's well-being and recovery. Even if there is no stipulated guideline treatment for cases of SJS and TEN, we think that further investigations about IVIG and plasmapheresis should be investigated as a possible way to treat both conditions.
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Affiliation(s)
| | | | - Duha Rabi
- Al-Quds University Faculty of Medicine, Jerusalem, State of Palestine
| | - Ihab Hilail
- Al-Quds University Faculty of Medicine, Jerusalem, State of Palestine
| | - Hatem Rjoob
- Al-Quds University Faculty of Medicine, Jerusalem, State of Palestine
| | - Abdelrahman Rabee
- Al-Quds University Faculty of Medicine, Jerusalem, State of Palestine
| | - Naser Amro
- Al-Quds University Faculty of Medicine, Jerusalem, State of Palestine
| | - Qutaiba Qafisheh
- Al-Quds University Faculty of Medicine, Jerusalem, State of Palestine
| | - Mohammad Kharraz
- An-Najah National University Hospital, Nablus, State of Palestine
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Sukri A, Salleh MZ, Masimirembwa C, Teh LK. A systematic review on the cost effectiveness of pharmacogenomics in developing countries: implementation challenges. THE PHARMACOGENOMICS JOURNAL 2022; 22:147-159. [PMID: 35319010 DOI: 10.1038/s41397-022-00272-w] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/04/2021] [Revised: 02/16/2022] [Accepted: 03/01/2022] [Indexed: 01/02/2023]
Abstract
The major challenges that delay the implementation of pharmacogenomics based clinical practice in the developing countries, primarily the low- and middle-income countries need to be recognized. This review was conducted to systematically review evidence of the cost-effectiveness for the conduct of pharmacogenomics testing in the developing countries. Studies that evaluated the cost-effectiveness of pharmacogenomics testing in the developing countries as defined by the United Nations were included in this study. Twenty-seven articles met the criteria. Pharmacogenomics effectiveness were evaluated for drugs used in the treatment of cancers, cardiovascular diseases and severe cutaneous adverse reactions in gout and epilepsy. Most studies had reported pharmacogenomics testing to be cost-effective (cancers, cardiovascular diseases, and tuberculosis) and economic models were evaluated from multiple perspectives, different cost categories and time horizons. Additionally, most studies used a single gene, rather than a gene panel for the pharmacogenomics testing. Genotyping cost and frequency of risk alleles in the populations influence the cost-effectiveness outcome. Further studies are warranted to examine the clinical and economic validity of pharmacogenomics testing in the developing countries.
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Affiliation(s)
- Asif Sukri
- Integrative Pharmacogenomics Institute, Universiti Teknologi MARA Cawangan Selangor, Puncak Alam Campus, 42300, Puncak Alam, Selangor, Malaysia
| | - Mohd Zaki Salleh
- Integrative Pharmacogenomics Institute, Universiti Teknologi MARA Cawangan Selangor, Puncak Alam Campus, 42300, Puncak Alam, Selangor, Malaysia
| | - Collen Masimirembwa
- African Institute of Biomedical Science & Technology, Wilkins Hospital, Corner J Tongogara and R Tangwena, Harare, Zimbabwe
| | - Lay Kek Teh
- Integrative Pharmacogenomics Institute, Universiti Teknologi MARA Cawangan Selangor, Puncak Alam Campus, 42300, Puncak Alam, Selangor, Malaysia. .,Faculty of Pharmacy, Universiti Teknologi MARA Cawangan Selangor, Puncak Alam Campus, 42300, Puncak Alam, Selangor, Malaysia.
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Mitani K, Hida S, Fujino H, Sumimoto S. Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication. BMJ Case Rep 2022; 15:e249224. [PMID: 35470164 PMCID: PMC9039386 DOI: 10.1136/bcr-2022-249224] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/01/2022] [Indexed: 11/04/2022] Open
Abstract
A young girl in her teens presented with fever, rashes and various mucocutaneous symptoms. Flat erythematous macules were seen mainly on the limbs, without blisters or skin detachments. The lips were swollen with crusts and haemorrhage. The oral cavity and pharynx showed ulcerative lesions with exudate. Severe bilateral ocular lesions with pseudomembrane formation and corneal epithelial defects were present. Also, urogenital lesion and gastrointestinal symptoms with frequent haematochezia were observed. Her symptoms and pathological findings were consistent with Stevens-Johnson syndrome. She was treated with prednisolone and methylprednisolone pulse therapy. Her ocular and cutaneous symptoms improved without severe chronic complications. However, 1 month later, she developed dyspnoea, and a pulmonary function test revealed severe obstructive ventilation disorder. After discharge, she was regularly followed up for respiratory complications. High-resolution chest CT performed 9 months after onset revealed mosaic perfusions and bronchiectasis, consistent with bronchiolitis obliterans.
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Affiliation(s)
- Kazuki Mitani
- Department of Pediatrics, Osaka Red Cross Hospital, Osaka, Japan
| | - Shinya Hida
- Department of Pediatrics, Osaka Red Cross Hospital, Osaka, Japan
| | - Hisanori Fujino
- Department of Pediatrics, Osaka Red Cross Hospital, Osaka, Japan
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Hidayati E, Wijayanti DN, Rahayu DA, Nurhidayati T, Mariyam M, Alfiyanti D. Supportive Therapy to Reduce Anxiety Levels of COVID-19 Nurses in Isolation ICU Room. Open Access Maced J Med Sci 2022. [DOI: 10.3889/oamjms.2021.7837] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
BACKGROUND: COVID-19 for this year has had a negative impact on life, particularly the psychological impact of anxiety. Anxiety occurs in the general public and in health workers, one of which is a nurse. If anxiety continues, it will affect the quality of life and performance of nurses. Supportive therapy is one of the non-pharmacological treatments to reduce anxiety.
AIM: This study was to determine the effectiveness of supportive therapy in reducing the anxiety of COVID-19 nurses in isolation ICU.
METHODS: This study used quantitative research with a quasi-experimental design. The researcher wanted to know the effectiveness of a treatment, namely, supportive therapy, against the anxiety of the COVID-19 nurses of isolation ICU room. This study used a pre-post-test one-group intervention method. The therapy mechanism applied 1–4 sessions of supportive therapy by dividing each session into 3 days. Forty respondents participated in this study. The data obtained were analyzed using paired t-test.
RESULTS: The results show that 55% of respondents experience moderate anxiety before supportive therapy, and 60% are not anxious after supportive therapy. It is also found that p = 0.000 is considered and lower than the alpha value of 0.05 (0.000 < 0.05). Therefore, there is a statistically significant difference before and after supportive therapy.
CONCLUSION: The conclusion that can be drawn is the influence of supportive therapy on reducing anxiety in COVID-19 isolation ICU nurses.
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Primisawitri PP, Mawardi P. The Correlation of Neutrophil-Lymphocyte Ratio and Eosinophil Count with SCORTEN in SJS/TEN. CLINICAL, COSMETIC AND INVESTIGATIONAL DERMATOLOGY 2022; 15:547-556. [PMID: 35387203 PMCID: PMC8978353 DOI: 10.2147/ccid.s356450] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/12/2022] [Accepted: 03/15/2022] [Indexed: 11/29/2022]
Abstract
Introduction Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute severe cutaneous adverse reactions commonly caused by medication. Precise evaluation of disease severity in initial setting must be obtained to start appropriate treatment. Neutrophil and lymphocyte ratio (NLR) plays a role in displaying inflammatory reaction while eosinophils count (EC) influences immunological dysregulation including the proliferation of cytotoxic cells in early onset of SJS/TEN. Objective To evaluate whether NLR and EC serve as prognostic markers of disease severity in patients with SJS/TEN using SCORTEN. Methods A single center study with retrospective study included SJS/TEN patients at Dr. Moewardi General Hospital Surakarta in January 1st 2018–December 31st 2020. The required laboratory data was assessed at the beginning of the patient’s admission through medical records. The significance analysis were performed using one-way ANOVA and Spearman while the receiver-operator curve were used to evaluate the prognostic value of variables for severity in SJS/TEN patients. Results The total sample in this study was 24 patients with majority female (58%) and range from 25 to >50 years (54%). The results demonstrated of significant difference and positively correlated between NLR and EC with severity of SJS/TEN (p<0.01; r>0.05). The specificity and sensitivity of 51%;61% and 70%;60%, respectively. Conclusion NLR and EC can be used as prognosticators of severity in SJS/TEN while further research on other inflammatory markers with increased number of samples and study centers are needed to provide more actual data.
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Affiliation(s)
- Pratiwi Prasetya Primisawitri
- Department of Dermatology and Venereology Faculty of Medicine, Sebelas Maret University/Dr. Moewardi General Hospital Surakarta, Central Java, Indonesia
| | - Prasetyadi Mawardi
- Department of Dermatology and Venereology Faculty of Medicine, Sebelas Maret University/Dr. Moewardi General Hospital Surakarta, Central Java, Indonesia
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Eyer GC, Heidemeyer K, Exadaktylos A, Ziaka M. Fuchs Syndrome with Isolated Oral Mucosa Lesions due to Severe Herpes Simplex Cheilitis in a Patient with Idiopathic Thrombocytopenic Purpura. Eur J Case Rep Intern Med 2022; 9:003278. [PMID: 35520365 PMCID: PMC9067420 DOI: 10.12890/2022_003278] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2022] [Accepted: 03/02/2022] [Indexed: 11/29/2022] Open
Abstract
UNLABELLED Stevens-Johnson syndrome (SJS) is a severe dermatological disease classically characterized by erythematous target lesions and mucosal involvement. Fuchs syndrome is an incomplete presentation of SJS which has oral, conjunctival and genital manifestations but no skin lesions. To the best of our knowledge, our case of Fuchs syndrome in an 80-year-old man is the first such case related to herpes simplex virus (HSV)-1 infection to be described. Our patient quickly recovered following IVIG therapy, although specific treatment is still a topic of discussion. Research is required on this poorly understood dermatological disease to determine optimum therapy. LEARNING POINTS We report a case of Fuchs syndrome in an elderly man after HSV-1 cheilitis.Therapy always includes discontinuation of the causative drug.Specific therapy for Stevens-Johnson syndrome and Fuchs syndrome is still a topic of discussion, although we noted marked improvement following the administration of IVIG therapy.
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Affiliation(s)
| | - Kristine Heidemeyer
- Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Aristomenis Exadaktylos
- Department of Emergency Medicine, Inselspital, University Hospital, University of Bern, Bern, Switzerland
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Yang W, Xu X, Xia D, Wang H, Jiang J, Yang G. Toxic epidermal necrolysis associated with chemoimmunotherapy for lymphoma: case report and literature review. Immunotherapy 2022; 14:275-282. [PMID: 35128931 DOI: 10.2217/imt-2021-0074] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
Abstract
Aim: The emergence of antitumor immunotherapy has been beneficial for patients with tumors, but more attention should be paid to the toxic side effects of chemoimmunotherapy. Here we describe a patient with NK/T-cell lymphoma who developed toxic epidermal necrolysis (TEN) during treatment with a regimen consisting of sintilimab combined with pegaspargase, gemcitabine and oxaliplatin (P-GemOx). Case presentation: A patient received six cycles of P-GemOx chemotherapy as first-line treatment; 1 year later, he received the same dose of P-GemOx combined with sintilimab as chemoimmunotherapy due to recurrence of NK/T-cell lymphoma. He developed a massive rash that quickly developed into TEN after the fourth chemoimmunotherapy. Conclusion: Although rare, cases of fatal TEN caused by single-agent PD-1 inhibitor or gemcitabine have been reported. Careful attention to drug-related cutaneous toxicities is needed when these two agents are combined. This report highlights the significance of TEN as a rapid and serious adverse event induced by chemoimmunotherapy.
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Affiliation(s)
- Wei Yang
- Department of Oncology, Hangzhou Red Cross Hospital, Hangzhou, China
| | - Xiaofeng Xu
- Department of Hematology, Hangzhou Red Cross Hospital, Hangzhou
| | - Dajing Xia
- Department of Toxicology of School of Public Health, & Center of Immunology & Infection, School of Medicine, Zhejiang University, Hangzhou, China
| | - Huaichong Wang
- Department of Pharmacy, Hangzhou Red Cross Hospital, Hangzhou, China
| | - Jing Jiang
- Department of Oncology, Hangzhou Red Cross Hospital, Hangzhou, China
| | - Guoliang Yang
- Department of Hematology, Hangzhou Red Cross Hospital, Hangzhou
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Yang L, Shou YH, Li F, Zhu XH, Yang YS, Xu JH. Intravenous Immunoglobulin Combined With Corticosteroids for the Treatment of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Propensity-Matched Retrospective Study in China. Front Pharmacol 2022; 12:750173. [PMID: 35115922 PMCID: PMC8804212 DOI: 10.3389/fphar.2021.750173] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2021] [Accepted: 11/25/2021] [Indexed: 11/13/2022] Open
Abstract
Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening severe adverse drug reactions. The use of corticosteroids and intravenous immunoglobulin (IVIg) in SJS/TEN remains controversial. Methods: In this single-center, observational, propensity-matched, retrospective study, we collected a total of 224 patients with SJS/TEN who were hospitalized in our department from 2008 to 2019; according to treatment with IVIg combined with corticosteroids or with corticosteroids alone, patients were divided into combination therapeutic group (163 patients) and monotherapeutic group (61 patients). Patients from the two groups were matched by their propensity score in blocks of 2:1. Comparisons of the clinical characteristics and prognoses between propensity-matched SJS/TEN patients treated with IVIg combined with corticosteroids and corticosteroids alone were made. Results: After our propensity matching, a total of 145 patients were yielded, including 93 patients treated with IVIg and 52 patients not treated with IVIg. All of the 23 variables reflected good matching between patients treated with/without IVIg, and no significant difference was observed. Although there was no significant difference between the totally predicted and actual mortality in both of our groups, the actual mortality was lower than it was predicted in patients treated with IVIg [p > 0.250, the standardized mortality ratio (SMR) was 0.38, 95% CI 0.00-0.91] and patients treated without IVIg (p = 1.000, the SMR was 0.75, 95% CI 0.00-1.76). IVIg tended toward reducing the time to arrest of progression by 1.56 days (p = 0.000) and the length of hospital stay by 3.37 days (p = 0.000). The mortality rate was 45% lower for patients treated with IVIg combined with corticosteroids than those only treated with corticosteroid therapy, although it was not statistically significant (p = 0.555). The incidence of skin infections was significantly lower in the combined therapy group (p < 0.025), and the total infection rate of patients treated with combination therapy tended to decrease by 67% compared to patients treated with corticosteroids alone (p = 0.047). Conclusion: The actual mortality rate of patients treated with corticosteroids alone or IVIg combined with corticosteroids tended to be lower than those predicted by TEN-specific severity-of-illness score (SCORTEN), although there was no significance. Compared with those treated by corticosteroids alone, combination therapy was prone to bring a better prognosis for SJS/TEN patients.
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Affiliation(s)
- Lu Yang
- Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China
| | - Yan-Hong Shou
- Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China
| | - Feng Li
- Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China
| | - Xiao-Hua Zhu
- Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China
| | - Yong-Sheng Yang
- Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China
| | - Jin-Hua Xu
- Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China
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Blevins K, Parsh B. Clinical Queries. Nursing 2022; 52:10-11. [PMID: 35085187 DOI: 10.1097/01.nurse.0000816344.08793.0f] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Affiliation(s)
- Kristina Blevins
- Kristina Blevins is a recent graduate at the Sacramento State School of Nursing, where Bridget Parsh is a professor. Dr. Parsh is also a member of the Nursing2022 editorial board
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Naik PP. A contemporary snippet on clinical presentation and management of toxic epidermal necrolysis. Scars Burn Heal 2022; 8:20595131221122381. [PMID: 36118413 PMCID: PMC9476246 DOI: 10.1177/20595131221122381] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
Introduction Toxic epidermal necrolysis (TEN) is one of the most severe cutaneous adverse reactions with a mortality rate of 30%. Due to a lack of consensus regarding the treatment and management of TEN, therapy is individualized on a case-to-case basis. Purpose The scientific literature about Stevens-Johnson Syndrome (SJS) and TEN is summarized and assessed to aid and assist in determining the optimal course of treatment. Methods PubMed and Google Scholar, among others, were searched with the keywords: “Toxic Epidermal Necrolysis”, “corticosteroids”, “cyclosporine”, “etanercept”, “intravenous immunoglobulin”, “Stevens-Johnson syndrome” and filtered by year. The research articles generated by the search, and their references, were reviewed. Results TEN is a severe dermatological condition that is mainly caused by medicines. World-wide guidelines differ in care plans. As there is no consensus on the management of TEN, this article aims to summarize the efficacy and feasibility of the management aspect of TEN from previous studies. Supportive care is highly accepted, along with early discontinuation of all medicines (hydration & electrolytes). Corticosteroids and cyclosporine have been used in therapy. Intravenous immunoglobulin (IVIG) is currently being administered; however, their efficacy by themselves and in combination remains uncertain. Conclusion Current evidence predominantly from retrospective studies suggests no individual treatment has sufficient efficacy and a multi-faceted regimen stands to be favored. Therapeutic regimens from corticosteroids to IVIG are under constant evaluation. The life-threatening nature of TEN warrants further confirmation with more extensive, robust randomized, controlled trials. Lay Summary Toxic epidermal necrolysis (TEN) is a serious skin reaction with a 30% chance of mortality. Commonly TEN is caused by medicines and results in a burn like appearance and sensation in patients. Usually administered medicine is cleared effectively by the human body but when the clearance of few metabolites from medicine is disrupted due to few genes, it leads to an ominous response by the body. This response involves several intermediate chemicals that primarily attack skin cells. Treatment guidelines differ globally. Supportive care is highly accepted, along with early discontinuation of all medicine. Currently, a multi-faceted treatment regimen is favored. Treatments like corticosteroids to immunoglobulins are under constant evaluation. Identification of the perfect combination of treatment needs confirmation from robust randomized controlled trials.
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Affiliation(s)
- Piyu Parth Naik
- Department of Dermatology, Saudi German Hospital and Clinic, Dubai, United Arab Emirates
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Case report of a patient with toxic epidermal necrolysis with complications and review of literature. SRP ARK CELOK LEK 2022. [DOI: 10.2298/sarh210830012p] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
Introduction. Toxic epidermal necrolysis (TEN), also known as Lyell?s
syndrome, is rare exfoliative disorder with high mortality rate. This entity
was first described by Lyell in 1956, who termed the condition ?toxic
epidermal necrolysis', pointing out that drug sensitization was generally
considered to be the mechanism leading to this syndrome. The drugs most
frequently involved are nonsteroidal anti-inflammatory drugs (NSAID),
chemotherapeutic agents, antibiotics, and anticonvulsants, although virus,
bacterial, and fungal infections, as well as immunization, have been
described. Case outline. We present a 72-year-old man with the following
history. Five days before he was admitted patient have had high fiver and
pain throat. He was treated with antibiotics and nonsteroidal
anti-inflammatory drugs (NSAID) because had had bronchopneumonia and after
that he developed itchy skin rash over all body following with sensation of
slight sore throat with conjunctival hyperaemia and hard breathing and high
fiver and because of this he was hospitalized in the local hospital. After
worsening of symptomatology following with urticaria like plaques and then
bullae with progress all over the body patient was moved to our Institution
and positioned in intensive care unit, under suspicion of TEN. The aim of
the paper presented here is to give a thorough summary of our literature
review searching for the best therapy modalities for our patient with TEN.
Conclusion. We decided to present this case because our patient had been
affected with lesions of multiorgan system and with affecting 80% TBSA and
SCROTEN score 4. With early diagnosed of TEN patient was successfully
treated.
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Houschyar KS, Tapking C, Borrelli MR, Puladi B, Ooms M, Wallner C, Duscher D, Pförringer D, Rein S, Reumuth G, Schulz T, Nietzschmann I, Maan ZN, Grieb G, Philipp-Dormston WG, Branski LK, Siemers F, Lehnhardt M, Schmitt L, Yazdi AS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis. J Wound Care 2021; 30:1012-1019. [PMID: 34881995 DOI: 10.12968/jowc.2021.30.12.1012] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
OBJECTIVE Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and severe skin and mucosal reactions that are associated with high mortality. Despite the severity, an evidence-based treatment protocol for SJS/TEN is still lacking. METHOD In this systematic review and meta-analysis, the PubMed database was searched using the following terms: [Stevens-Johnson syndrome] OR [toxic epidermal necrolysis] AND [therapy] OR [treatment] over a 20-year period (1999-2019) in the German and English language. All clinical studies reporting on the treatment of SJS/TEN were included, and epidemiological and diagnostic aspects of treatment were analysed. A meta-analysis was conducted on all comparative clinical studies that met the inclusion criteria. RESULTS A total of 88 studies met the inclusion criteria, reporting outcomes in 2647 patients. Treatment was either supportive or used systemic corticosteroid, intravenous immunoglobulin, plasmapheresis, cyclosporine, thalidomide or cyclophosphamide therapy. The meta-analysis included 16 (18%) studies, reporting outcomes in 976 (37%) patients. Systemic glucocorticoids showed a survival benefit for SJS/TEN patients in all analyses compared with other forms of treatment. Cyclosporine treatment also showed promising results, despite being used in a small cohort of patients. No beneficial effects on mortality could be demonstrated for intravenous immunoglobulins. CONCLUSION Glucocorticoids and cyclosporine may be tentatively recommended as the most promising immunomodulatory therapies for SJS/TEN, but these results should be investigated in future prospective controlled trials.
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Affiliation(s)
- Khosrow S Houschyar
- Department of Dermatology and Allergology, University Hospital Aachen, Germany
| | - Christian Tapking
- Department of Surgery, Shriners Hospitals for Children-Galveston, University of Texas Medical Branch, 815 Market Street, Galveston, TX 77550, US.,Department of Hand, Plastic and Reconstructive Surgery, Burn Trauma Center, BG Trauma Center Ludwigshafen, University of Heidelberg, Germany
| | - Mimi R Borrelli
- Division of Plastic and Reconstructive Surgery, Department of Surgery, Stanford School of Medicine, Stanford, CA 94305, US
| | - Behrus Puladi
- Department of Oral and Maxillofacial Surgery, University Hospital RWTH, Aachen
| | - Mark Ooms
- Department of Oral and Maxillofacial Surgery, University Hospital RWTH, Aachen
| | - Christoph Wallner
- Department of Plastic Surgery, BG University Hospital Bergmannsheil, Ruhr University Bochum, Bürkle-de-la-Camp Platz 1, 44789, Bochum, Germany
| | - Dominik Duscher
- Department of Plastic Surgery and Hand Surgery, Technical University Munich, Munich, Germany
| | - Dominik Pförringer
- Clinic and Policlinic of Trauma Surgery, Klinikum Rechts der Isar, Technische Universität München, Germany
| | - Susanne Rein
- Department of Plastic and Hand Surgery-Burn Center-Clinic St. Georg, Leipzig, Germany
| | - Georg Reumuth
- Department of Plastic Surgery and Hand Surgery, Evangelische Elisabeth Klinik, Luetzowstraße 26, 10785 Berlin, Germany
| | - Torsten Schulz
- Department of Plastic and Hand Surgery, Burn Unit, Trauma Center Bergmannstrost Halle, Halle, Germany
| | - Ina Nietzschmann
- Department of Plastic and Hand Surgery, Burn Unit, Trauma Center Bergmannstrost Halle, Halle, Germany
| | - Zeshaan N Maan
- Division of Plastic and Reconstructive Surgery, Department of Surgery, Stanford School of Medicine, Stanford, CA 94305, US
| | - Gerrit Grieb
- Department of Plastic Surgery and Hand Surgery, Gemeinschaftskrankenhaus Havelhoehe, Teaching Hospital of the Charité Berlin, Kladower Damm 221, 14089 Berlin, Germany
| | | | - Ludwik K Branski
- Department of Surgery, Shriners Hospitals for Children-Galveston, University of Texas Medical Branch, 815 Market Street, Galveston, TX 77550, US
| | - Frank Siemers
- Department of Plastic and Hand Surgery, Burn Unit, Trauma Center Bergmannstrost Halle, Halle, Germany
| | - Marcus Lehnhardt
- Department of Plastic Surgery, BG University Hospital Bergmannsheil, Ruhr University Bochum, Bürkle-de-la-Camp Platz 1, 44789, Bochum, Germany
| | - Laurenz Schmitt
- Department of Dermatology and Allergology, University Hospital Aachen, Germany
| | - Amir S Yazdi
- Department of Dermatology and Allergology, University Hospital Aachen, Germany
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Chen SY, Chen QW, Shou LM, Pan H, Ruan SM, Liang ZH, Shu QJ. Stevens-Johnson syndrome/toxic epidermal necrolysis successfully treated with Chinese herbal medicine Pi-Yan-Ning: A case report. JOURNAL OF INTEGRATIVE MEDICINE 2021; 19:555-560. [PMID: 34696996 DOI: 10.1016/j.joim.2021.10.001] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/26/2020] [Accepted: 09/18/2021] [Indexed: 12/27/2022]
Abstract
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare adverse cutaneous reaction with a low incidence and high mortality. Despite posing a serious threat to patients' health and lives, there is no high-quality evidence for a standard treatment regimen. Here we report the case of a 62-year-old man with stage IV pancreatic cancer who experienced immunotherapy-induced SJS/TEN. After consensus-based regular treatments at a local hospital, his symptoms became worse. Thus, he consented to receive Chinese herbal medicine (CHM) therapy. The affected parts of the patient were treated with the CHM Pi-Yan-Ning which was applied externally for 20 min twice a day. After 7 days of treatment, the dead skin began peeling away from the former lesions that had covered his hands, feet, and lips, indicating that skin had regenerated. After 12 days of treatment, the patient's skin was completely recovered. In this case, SJS/TEN was successfully treated with Pi-Yan-Ning, suggesting that there might be tremendous potential for the use of Pi-Yan-Ning in the treatment of severe skin reactions to drug treatments. Further basic investigations and clinical trials to explore the mechanism and efficacy are needed.
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Affiliation(s)
- Shu-Yi Chen
- Department of Oncology, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China
| | - Qun-Wei Chen
- Department of Oncology, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China
| | - Liu-Mei Shou
- Department of Oncology, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China
| | - Hong Pan
- Department of Oncology, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China
| | - Shan-Ming Ruan
- Department of Oncology, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China
| | - Zhe-Hao Liang
- Department of Medical Ultrasonics, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China
| | - Qi-Jin Shu
- Department of Oncology, the First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China.
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Kashfi S, Radparvar AA, Ventura Y, Sharma S, Sharma S. Bullous Erythema Multiforme Secondary to Trimethoprim-Sulfamethoxazole Use, Treated With Cyclosporine in a 91-Year-Old Male. Cureus 2021; 13:e18239. [PMID: 34712524 PMCID: PMC8542345 DOI: 10.7759/cureus.18239] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/23/2021] [Indexed: 11/29/2022] Open
Abstract
Erythema multiforme is an acute, immune-mediated, mucocutaneous condition in which 90% of cases are triggered by infection. The second most common cause is drug-induced. It classically presents with itchy, burning targetoid lesions on the skin and mucous membranes. The lesions may be mistaken for other conditions, and thus, rapid and correct diagnosis is crucial. It is most often treated with corticosteroids, though non-responders or those with weakened immune systems may require immunomodulatory therapy. We present the case of a 91-year-old male who developed bullous erythema multiforme after treatment with trimethoprim-sulfamethoxazole who was successfully treated with cyclosporine.
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Affiliation(s)
- Simon Kashfi
- Internal Medicine, CUNY School of Medicine, New York, USA
| | | | | | - Sapna Sharma
- Internal Medicine, Mahatma Gandhi Mission Institute of Health Sciences, Navi Mumbai, IND
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Hoyer D, Atti C, Nuding S, Vogt A, Sedding DG, Schott A. Toxic Epidermal Necrolysis Caused by Allopurinol: A Serious but Still Underestimated Adverse Reaction. AMERICAN JOURNAL OF CASE REPORTS 2021; 22:e932921. [PMID: 34634004 PMCID: PMC8522529 DOI: 10.12659/ajcr.932921] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Patient: Female, 75-year-old
Final Diagnosis: Toxic epidermal necrolysis
Symptoms: Exanthema • rash • shivers • weakness
Medication: —
Clinical Procedure: Analgesia • ciclosporine • corticosteroids • topical and systemic treatment
Specialty: Critical Care Medicine • Dermatology
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Affiliation(s)
- Daniel Hoyer
- Department of Medicine III, University Clinics of Halle (Saale), Halle (Saale), Germany
| | - Carlo Atti
- Department of Medicine III, University Clinics of Halle (Saale), Halle (Saale), Germany
| | - Sebastian Nuding
- Department of Medicine III, University Clinics of Halle (Saale), Halle (Saale), Germany
| | - Alexander Vogt
- Department of Medicine III, University Clinics of Halle (Saale), Halle (Saale), Germany
| | - Daniel G Sedding
- Department of Medicine III, University Clinics of Halle (Saale), Halle (Saale), Germany
| | - Artjom Schott
- Department of Medicine III, University Clinics of Halle (Saale), Halle (Saale), Germany
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48
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Zander E, Hintze TD, Sallee B, Allen P, Miller JL, Sagdeo M. Treatment of Toxic Epidermal Necrolysis with Etanercept in a Pediatric Patient. J Pediatr Pharmacol Ther 2021; 26:758-761. [PMID: 34588942 DOI: 10.5863/1551-6776-26.7.758] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2020] [Accepted: 01/08/2021] [Indexed: 12/18/2022]
Abstract
Toxic epidermal necrolysis is a rare, life-threatening skin disease with no consensus on adjunctive treatment, particularly in pediatric patients. We present the case of a 13-year-old previously healthy patient with drug-associated toxic epidermal necrolysis who experienced significantly shortened length of hospital stay and duration of symptoms compared with published literature when treated with 2 doses of etanercept 50 mg during 5 days.
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49
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Al Rajaibi R, Al Rumhi T, Al Abri AM. Carbamazepine-Induced Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Overlap Treated Successfully with Oral Cyclosporin: Case report and literature review. Sultan Qaboos Univ Med J 2021; 21:491-494. [PMID: 34522420 PMCID: PMC8407905 DOI: 10.18295/squmj.4.2021.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2020] [Revised: 08/12/2020] [Accepted: 10/21/2020] [Indexed: 11/30/2022] Open
Abstract
Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life-threatening mucocutaneous drug reactions. Several therapies have been used in the treatment of SJS/TEN but none of them have yet been established as the gold standard treatment. Studies have shown that cyclosporine (CsA) can be used off-label in TEN/SJS, which has shown promising therapeutic effectiveness in such diseases. Here we report a 38-year-old woman who presented to Ar Rustaq Hospital, Rustaq, Oman in 2019 with SJS/TEN overlap and was treated successfully with CsA along with supportive management. This case report also includes a literature review on the use of CsA in the treatment of SJS/TEN.
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Affiliation(s)
- Raqiya Al Rajaibi
- Department of Dermatology, Rustaq Extended Health Institute, Rustaq, Oman
| | - Thuraiya Al Rumhi
- Department of Dermatology, Rustaq Extended Health Institute, Rustaq, Oman
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50
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Frantz R, Huang S, Are A, Motaparthi K. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management. MEDICINA (KAUNAS, LITHUANIA) 2021; 57:895. [PMID: 34577817 PMCID: PMC8472007 DOI: 10.3390/medicina57090895] [Citation(s) in RCA: 101] [Impact Index Per Article: 25.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/19/2021] [Revised: 08/23/2021] [Accepted: 08/24/2021] [Indexed: 12/15/2022]
Abstract
Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpta Medica dataBASE and PubMed were searched for publications that addressed recent advances in the diagnosis and management of the disease. Multiple proteins (galectin 7 and RIP3) were identified that are promising potential biomarkers for SJS/TEN, although both are still in early phases of research. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN. Due to the rare nature of the disease, there is a lack of prospective, randomized controlled trials and conducting these in the future would provide valuable insights into the management of this disease.
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Affiliation(s)
- Robert Frantz
- College of Medicine, University of Florida, Gainesville, FL 32606, USA; (R.F.); (A.A.)
| | - Simo Huang
- Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, PA 19140, USA;
| | - Abhirup Are
- College of Medicine, University of Florida, Gainesville, FL 32606, USA; (R.F.); (A.A.)
| | - Kiran Motaparthi
- College of Medicine, University of Florida, Gainesville, FL 32606, USA; (R.F.); (A.A.)
- Department of Dermatology, College of Medicine, University of Florida, Gainesville, FL 32606, USA
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