Volume 8, Issue 8 , Pages e83-e84, August 2010
Henoch-Schönlein Purpura
Article Outline
A 47-year-old man presented with multiple erythematous macules over bilateral lower extremities for 2 weeks. The skin lesions increased in size and number, and were painless and nonitching. Furthermore, abdominal pain and intermittent diarrhea developed, along with painful symmetric arthralgia that involved the wrists, elbows, and ankles. There was no relevant medical or family history and he took no medications. On physical examination, his abdomen was distended with diffuse tenderness but no rebound pain, the bowel sound was hypoactive. There were multiple foci of palpable purpura over the bilateral lower legs (Figure A). Abdominal computed tomography (CT) scans revealed edema of the small bowel (Figure B, arrows), consistent with the presence of intestinal vasculitis. Urinalysis showed proteinuria 2+ (normal, negative) with approximately 10–20 red blood cells in high power field (normal, <2). The serum immunoglobulin A (IgA) and creatinine levels were normal, as well as multiple immunologic studies. Endoscopy showed multiple hyperemic and ulcerative lesions at the second portion of duodenum (Figure C). A biopsy from skin lesions revealed leukocytoclastic vasculitis (Figure D; H&E, original magnification × 400), with swollen endothelial cells in the dermal small vessel (large arrow), and infiltration of neutrophils (small arrow). Histopathological examination of the tissue (Figure E; H&E, original magnification × 400) revealed leukocytoclastic vasculitis with diffuse inflammation and perivascular neutrophilic cell infiltration (small arrows) and swollen endothelial cells (large arrow) in the submucosal small vessels. Henoch-Schönlein purpura (HSP) was diagnosed. His condition improved after steroid treatment, and the patient was doing well at follow-up 6 months after the onset of his illness.
Henoch-Schönlein purpura, characterized by palpable purpura, abdominal pain, hematuria, and arthritis, is a systemic small-vessel vasculitis mediated by IgA. Although William Heberden first reported this disorder in 1801, the association between the skin purpura, arthralgia, gastrointestinal, and renal involvement was not recognized until described by Johann Schönlein in 1837 and his student, Eduard Henoch in 1868.1
HSP can affect all age groups, predominantly male, but most commonly in children. While deposition of IgA-containing immune complex may play an important role in HSP, the exact etiology and pathogenesis remain unclear and there is no specific test that establishes the diagnosis. Similar to IgA nephropathy, the postulated mechanism of HSP is abnormalities of immune response triggered by factors such as various drugs or infection episodes, which is supported by the fact that occurrence of HSP is more common in winter, autumn, and spring than in summer.1
Acceptable diagnostic criteria for HSP are palpable purpura in the presence of at least 1 of either diffuse abdominal pain, any biopsy showing predominant IgA deposition, arthritis, or arthralgia, and renal involvement (any hematuria and/or proteinuria).2 The typical skin lesion of HSP is nonthrombocytopenic palpable purpura with symmetrical pattern over buttocks, face, or extremities (as in this case). While IgA-mediated vasculitis can occur in any tissues, especially the kidney, gastrointestinal tract, and skin, it may develop prior to the purpura, concurrently, or during the course of HSP. Patients with HSP usually recover spontaneously without complications except those having renal involvement, persistent proteinuria, or hematuria which may lead to poor renal outcome. The treatment is mainly supportive and symptomatic, including intravenous fluid or nonsteroidal anti-inflammatory drugs. Steroids are reported to be beneficial in severe HSP, such as refractory abdominal pain, bleeding, intussusception, or nephritis.3
References
Conflicts of interest The authors disclose no conflicts.
PII: S1542-3565(10)00207-7
doi:10.1016/j.cgh.2010.02.011
© 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
Volume 8, Issue 8 , Pages e83-e84, August 2010
