Wang YT, Zhang Y, Tang T, Luo C, Liu MY, Xu L, Wang L, Tang XM. Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review. World J Clin Cases 2022; 10(11): 3579-3586 [PMID: 35611208 DOI: 10.12998/wjcc.v10.i11.3579]
Corresponding Author of This Article
Xue-Mei Tang, PhD, Chief Doctor, Department of Rheumatology and Immunology; Ministry of Education Key Laboratory of Child Development and Disorders; National Clinical Research Center for Child Health and Disorders (Chongqing); China International Science and Technology Cooperation base of Child Development and Critical Disorders; Children’s Hospital of Chongqing Medical University, No. 136 Zhongshan 2nd Road, Yuzhong District, Chongqing 400014, China. tangxuemei2008@163.com
Research Domain of This Article
Pediatrics
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Apr 16, 2022; 10(11): 3579-3586 Published online Apr 16, 2022. doi: 10.12998/wjcc.v10.i11.3579
Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review
Ya-Ting Wang, Yu Zhang, Tao Tang, Chong Luo, Ming-Yue Liu, Li Xu, Li Wang, Xue-Mei Tang
Ya-Ting Wang, Yu Zhang, Tao Tang, Chong Luo, Ming-Yue Liu, Li Xu, Li Wang, Xue-Mei Tang, Department of Rheumatology and Immunology; Ministry of Education Key Laboratory of Child Development and Disorders; National Clinical Research Center for Child Health and Disorders (Chongqing); China International Science and Technology Cooperation base of Child Development and Critical Disorders; Children’s Hospital of Chongqing Medical University, Chongqing 400014, China
Ya-Ting Wang, Yu Zhang, Tao Tang, Chong Luo, Ming-Yue Liu, Li Xu, Li Wang, Xue-Mei Tang, Chongqing Key Laboratory of Child Infection and Immunity, Children’s Hospital of Chongqing Medical University, Chongqing 400014, China
Author contributions: Tang XM conceived and designed the study and revised the manuscript; Wang YT collected medical records and wrote the manuscript; Zhang Y collected medical records and participated in its design; Tang T collected medical records and provided pictures; Luo C participated in study design and coordination; Liu MY, Xu L, and Wang L collected and organized the literature; All authors read and approved the final manuscript.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xue-Mei Tang, PhD, Chief Doctor, Department of Rheumatology and Immunology; Ministry of Education Key Laboratory of Child Development and Disorders; National Clinical Research Center for Child Health and Disorders (Chongqing); China International Science and Technology Cooperation base of Child Development and Critical Disorders; Children’s Hospital of Chongqing Medical University, No. 136 Zhongshan 2nd Road, Yuzhong District, Chongqing 400014, China. tangxuemei2008@163.com
Received: December 7, 2021 Peer-review started: December 7, 2021 First decision: January 25, 2022 Revised: February 7, 2022 Accepted: February 27, 2022 Article in press: February 27, 2022 Published online: April 16, 2022
Abstract
BACKGROUND
Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy that occurs in childhood. It is characterized by muscle weakness and a characteristic rash. Previous literature reports have rarely described JDM with severe skin ulcers and infections.
CASE SUMMARY
Herein, we describe a case of a 2-year-old female patient who suffered from JDM, whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody, with progressively worsening skin ulcers and severe infections. The patient was treated with glucocorticoids and various immunosuppressants. Nevertheless, further progression of the disease and the combination of primary disease and severe infection in the later period were fatal.
CONCLUSION
In children, anti-nuclear matrix protein 2+ JDM combined with skin ulcers often indicates severe disease. In such cases, personalized treatment for the primary disease and infection prevention and control are essential.
Core Tip: Juvenile dermatomyositis (JDM) is a rare systemic autoimmune disease characterized by specific skin lesions, chronic muscle inflammation, and systemic vasculitis. We report a very rare case of JDM with severe skin ulcers and infections. By reporting the disease development and treatment of this case of a patient positive for anti-nuclear matrix protein 2 (NXP2) antibody combined with skin ulcers and performing a comprehensive literature review, we summarize JDM with skin ulcers, the clinical characteristics of JDM combined with positivity for anti-NXP2 antibody, and treatment measures for severe JDM.
