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Ming-Jen Chen,
Tsang-En Wang, Wen-Hsiung Chang, Shu-Jung Tsai, Wen-Shen Liao,
Division of Gastroenterology, Department of Internal Medicine,
Mackay Memorial Hospital, Mackay Medicine, Nursing and Management
College, Taipei, Taiwan, China
Correspondence to: Dr. Ming-Jen Chen, Division of
Gastroen-terology, Department of Internal Medicine, Mackay Memorial
Hospital, No. 92, Sec. 2, Chung-Shan N. Road, Taipei, Taiwan, China.
mingjen.ch@msa.hinet.net
Telephone: +886-2-25433535-2260
Fax: +886-2-25433642
Received: 2004-07-12
Accepted: 2005-01-21
Abstract
Henoch-SchÖnlein
purpura (HSP) is a systemic vasculitis of the small vessels of the
skin, joints, GI tract, and kidney. It preferentially affects
children but may also occur in adults. We report a 60-year-old man
with HSP who prese-nted with colicky abdominal pain, bloody
diarrhea, arth-ralgia, and skin rash. The gastrointestinal tract was
viewed by upper endoscopy and colonoscopy. We found charac-teristic
endoscopic findings in the stomach, cecum and sigmoid colon, the
combination of which has rarely been demonstrated in one patient.
Histologic examination of skin biopsy specimens revealed
leukocytoclastic vasculitis with positive staining for IgA in the
capillaries. Endoscopy appears to have substantial diagnostic
utility in patients suspected of having HSP, especially when
abdominal symptoms precede the cutaneous lesions.
© 2005 The WJG
Press and Elsevier Inc. All rights reserved.
Key words: Henoch-SchÖnlein
purpura; Purpura; Endoscopy
Chen MJ, Wang TE, Chang WH, Tsai SJ, Liao WS. Endoscopic findings in
a patient with Henoch-SchÖnlein
purpura. World J Gastroenterol
2005; 11(15): 2354-2356
http://www.wjgnet.com/1007-9327/11/2354.asp
INTRODUCTION
Henoch-SchÖnlein
purpura (HSP) is a systemic vasculitic disorder of the small vessels
of the skin, joints, GI tract, and kidney. The clinical
manifestations usually include arthralgia, a purpuric skin rash,
nephritis, abdominal pain, and GI bleeding. It commonly affects
young children, with the peak age of onset between 4 and 7 years.
The pathologic changes are mediated by IgA deposition[1],
complement activation, granulocyte recruitment, and destruction of
endothelial cells. The American College of Rheumatology published
diagnostic criteria for HSP in 1990[2] including (1) age
less than or equal to 20 years at disease onset, (2) the presence of
palpable purpura, (3) GI bleeding, and (4) a biopsy showing
granulocytes in the walls of small arterioles or venules. The
diagnosis is made when three or more of those criteria are
fulfilled.
CASE REPORT
A 60-year-old man presented with three days of colicky abdominal
pain with bloody diarrhea, arthralgia in the knees, and ankle edema.
His medical history was unremarkable except for a self-limited upper
respiratory infection one week previously. Physical examination
revealed diffuse lower abdominal rebound tenderness. There was no
fever and no skin rash on admission. The initial leukocyte count was
17 830 /mm3, and the C-reactive protein was 12 mg/dL. A
plain abdominal film showed generalized ileus and no evidence of
free air. Stool cultures were negative for enteric pathogens, and no
ova or parasites were found. Upper endoscopy revealed multiple
discrete coin-like petechiae and hemorrhagic erosions in the gastric
antrum and lower body (Figure 1). Colonoscopy revealed patches of
hyperemic and ecchymotic lesions in the cecum (Figure 2), ileocecal
valve, and sigmoid colon. The patient was managed with fasting and
intravenous fluids. The day after admission, he developed numerous
erythematous, purpuric lesions, predominantly on the lower
extremities and buttocks. The lesions rapidly became palpable
(Figure 3). A platelet count, prothrombin time and partial
thromboplastin time were normal. Based on this constellation of
findings, HSP was suspected. Tissue from a skin biopsy of the
lesions was compatible with leukocytoclastic vasculitis with
deposition of IgA, confirming the diagnosis of HSP. The urinalysis
and renal function were normal. Parenteral steroid therapy (5 mg/(kg·d)
of hydrocortisone) was instituted initially, resulting in relief of
the abdominal pain and arthralgia and gradual resolution of the skin
lesions within 5 d. The treatment was switched to oral prednisolone
20 mg/d with gradual tapering over 4 wk. The patient has remained
well over 2 years of follow up.
Figure
1 Upper
endoscopic view of multiple discrete coin-like petechiae in the
lower gastric body.
Figure
2 Colonscopic
view of hyperemic and ecchymotic lesions in the cecum.
Figure
3 Numerous
erythematous papules and palpable purpura on the lower extremities
and buttocks.
DISCUSSION
The above-mentioned patient fulfilled three of four criteria for
the diagnosis of HSP. The only exception was his age. The diagnostic
sensitivity and specificity of three out of the four criteria are
89.4% and 88.1%, respectively[2]. Although the cause of
HSP is unknown, immunizations, certain food allergies, insect bites,
infection[3], and some medications[4] may play
a role in the development of the disease. It may be that our patient's
recent cold predisposed him to HSP. The final diagnosis of HSP was
confirmed by histologic examination of a skin biopsy specimen
demonstrating a vasculitis with leukocyte infiltration and
deposition of IgA.
The diagnosis of HSP can be difficult, especially
when abdominal symptoms precede cutaneous lesions. A skin rash is
nearly always present, although not necessarily in the earliest
stages. Lanzkowsky reported that 14% of patients had abdominal
symptoms preceding the skin rash[5]. As in our case, the
skin rash is typically on the lower extremities and buttocks, less
commonly on the face and trunk. It starts as erythematous papules
that quickly become palpable purpura. Normal platelets counts,
prothrombin time, and partial thromboplastin time exclude a platelet
or hemorrhagic disorder as the cause of purpura. Other laboratory
tests are usually not conclusive.
Abdominal symptoms reportedly occur in 50-85% of
HSP patients, possibly secondary to edema and hemorrhage within the
bowel wall and mesentery. The abdominal pain is always colicky and
poorly localized. On physical examination the abdomen may be rigid
or distended, occasionally mimicking an acute abdomen and resulting
in unnecessary exploratory laparotomy. Signs suggestive of
intussusception including an abdominal mass may be present in
children. GI bleeding occurs in one-third of cases and may be overt,
with hematemesis, melena, or bloody stools[6,7]. While
bloody diarrhea is common in HSP, it may also occur in eosinophilic
gastroenteritis[8], systemic lupus erythematosus,
parasitic infection, and drug-induced vasculitis. Distinguishing
between these different vasculitic disorders depends on clinical,
serological, hematologic, and histologic findings. Arthralgia is
usually symmetrical, with the ankles being the most commonly
affected joints. Joint involvement is considered as an indication
for systemic steroids. Proteinuria and hema-turia indicate renal
involvement and generally occur within the first 3 mo after the
onset of the disease.
The hallmarks of HSP on computed tomography are
multiple focal areas of bowel thickening with skip lesions,
mesenteric edema, vascular engorgement, and non-specific
lymphadenopathy[9]. However, similar thickening may also
be seen in eosinophilic gastroenteritis[8], lymphoma,
Crohn's
disease, and granulomatous disease. It is thus not a specific sign
for HSP. Ultrasound may reveal generalized thickening of the
intestinal wall, ascites, and sometimes intussusception.
GI involvement in HSP is seen predominantly in
the small bowel[10,11] but may also affect the esophagus,
stomach, terminal ileum[12,13] and colon[14].
Our patient had diffuse, poorly localized abdominal pain and bloody
diarrhea, prompting us to visualize both the upper and lower GI
tract. The endoscopic findings in our patient included discrete
coin-like petechiae, hemorrhagic erosions, and skip hyperemic and
ecchymotic lesions. These were seen in the gastric antrum, cecum,
ileocecal valve, and sigmoid colon. It is important to recognize
these characteristic endoscopic findings when a previously healthy
patient presents with the sudden onset of an acute abdomen. When
encountered, they should suggest the possibility of HSP. Although we
did not biopsy the lesions in the GI tract, we expected to find
histologic abnormalities similar to those found on skin biopsy.
However, endoscopic biopsies often miss the subm-ucosal vessels and
only reveal nonspecific inflammation.
HSP is primarily a medical disease and requires
only supportive treatment once other acute surgical conditions have
been excluded. Simple analgesics or non-steroidal anti-inflammatory
drugs are used as first-line therapy for relief of arthralgia.
Parenteral steroids have been advocated for more severe abdominal or
joint pains, or painful angioedema, but they should be used with
caution if there is active GI bleeding. All patients with HSP should
have their urine analyzed on several occasions during the initial
stages of the disease. Proteinuria and hematuria indicate possible
renal involvement which, if it progresses to renal insufficiency,
has a poor long-term outcome[15,16]. Our patient's
urinalysis was normal, and his renal function has been remained
normal over two years of follow up.
In conclusion, the majority of patients with HSP
recover fully. The prognosis is best in the absence of renal
involvement. The diagnosis of HSP may be difficult, especially when
abdominal symptoms precede the characteristic palpable purpura.
Typical endoscopic findings may alert gastroent-erologists to
consider this disease early and thus avoid unnecessary laparotomy.
REFERENCES
1
Uchiyama K, Yoshida N, Mizobuchi M, Higashihara H,
Naito Y, Yoshikawa T. Mucosal IgA deposition in
Henoch-Schonlein purpura with
duodenal ulcer. J Gastroenterol Hepatol 2002; 17: 728-729
2
Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG,
Arend WP, Edworthy SM, Fauci AS, Leavitt RY, Lie JT.
The American College of Rheumatology
1990 criteria for the classification of Henoch-Schonlein purpura.
Arthritis Rheum 1990; 33:
1114-1121
3
Gimenez-Esparza, Vich JA, Arguelles BF, Martin IH,
Gutierrez Fernandez MJ, Porras Vivas JJ. Recurrence of
Henoch-Schonlein purpura in
association with colitis. J Clin Gastroenterol 2002; 34:
492-493
4
Borras-Blasco J, Enriquez R, Amoros F, Cabezuelo JB,
Navarro-Ruiz A, Perez M, Fernandez J. Henoch-Schonlein
purpura associated with
clarithromycin. Case report and review of literature. Int J Clin
Pharmacol Ther
2003; 41: 213-216
5
Lanzkowsky S, Lanzkowsky L, Lanzkowsky P.
Henoch-Schoenlein purpura. Pediatr Rev 1992; 13: 130-137
6
Nathan K, Gunasekaran TS, Berman JH. Recurrent
gastrointestinal Henoch-Schonlein purpura.
J Clin Gastroenterol 1999; 29:
86-89
7
Lippl F, Huber W, Werner M, Nekarda H, Berger H,
Weigert N, Life-threatening gastrointestinal bleeding due to a
jejunal lesion of Henoch-Schonlein
purpura. Endoscopy 2001; 33: 811-813
8
Chen MJ, Chu CH, Lin SC, Shih SC, Wang TE.
Eosinophilic gastroenteritis: Clinical experience with 15
patients.
World J Gastroenterol 2003; 9:
2813-2816
9
Jeong YK, Ha HK, Yoon CH, Gong G, Kim PN, LeeNG, Min
YI, Auh YH. Gastrointestinal involvement in Henoch
Schonlein syndrome: CT findings. Am
J Roentgenol 1997; 168: 965-968
10
Esaki M, Matsumoto T, Nakamura S, Kawasaki M, Iwai K,
Hirakawa K, Tarumi K, Yao T, Iida M. GI involvement
in Henoch-Schonlein purpura. Gastrointest
Endosc 2002; 56: 920-923
11
Pore G. GI lesions in Henoch-Schonlein purpura. Gastrointest
Endosc 55: 283-286
12
Ortego-Centeno N, Callejas-Rubio JL, Lopez-Manas JG,
Troncoso-Garcia E, de la Higuera Torres-Puchol J,
Ileitis terminalis in a patient
with Henoch-Schonlein purpura. Dig Dis Sci 1999; 44:
1590-1593
13
Kim HS, Lee DK, Baik SK, Kwon SO. Ileal vasculitis in
Henoch-Schonlein purpura. Gastrointest Endosc
2001; 54: 493-494
14
Nakasone H, Hokama A, Fukuchi J, Makishi T, Yamashiro
T, Sakugawa H, Kinjo F, Saito A. Colonoscopic findings in
an adult patient with
Henoch-Schonlein purpura. Gastrointest Endosc 2000; 52: 392
15
Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P,
Nochy D. Henoch-Schonlein purpura in adults: outcome and
prognostic factors. J Am Soc
Nephrol 2002; 13: 1271-1278
16
Garcia-Porrua C, Gonzalez-Louzao C, Llorca J,
Gonzalez-Gay MA. Predictive factors for renal sequelae in
adults
with Henoch-Schonlein purpura. J
Rheumatol 2001; 28: 1019-1024 Science
Editor Guo SY Language
Editor Elsevier HK
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