The clinical implications of adult-onset henoch-schonelin purpura

Warit Jithpratuck,Yasmin Elshenawy,Guha Krishnaswamy,David S Chi,George Youngberg,Hana Saleh

doi:10.1186/1476-7961-9-9

Abstract

Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis (LCV) involving the skin and other organs. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported. Management often involves the use of immunomodulatory or immune-suppressive regimens.

Highlights

Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition
In the cases reported in children, the majority of cases presented with an eruption, while up to 66% presented with abdominal pain and close to 50% the cases demonstrated renal involvement [2]
Skin biopsy revealed the presence of a perivascular inflammatory infiltrate in the superficial dermal blood vessels, with nuclear dust and focal fibrinoid necrosis, consistent with leukocytoclastic vasculitis(LCV) (Figure 2C), accompanied by the deposition of IgA on vascular walls detected by direct immunofluorescent staining, a pathognomonic feature of HSP

Summary

Introduction

Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement [1]. A skin biopsy showed an inflammatory infiltrate around superficial blood vessels with associated nuclear dust and focal fibrinoid necrosis, consistent with leukocytoclastic vasculitis This was accompanied by deposition of IgA on vascular walls seen on direct immunofluorescent staining, a pathognomonic feature of HSP. Skin biopsy revealed the presence of a perivascular inflammatory infiltrate in the superficial dermal blood vessels, with nuclear dust and focal fibrinoid necrosis, consistent with leukocytoclastic vasculitis(LCV) (Figure 2C), accompanied by the deposition of IgA on vascular walls detected by direct immunofluorescent staining, a pathognomonic feature of HSP.

Conclusion

Findings

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