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Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis

Authors:

Fatma Dedeoglu, MD

Susan Kim, MD, MMSc

Section Editor:

Robert Sundel, MD

Deputy Editor:

Elizabeth TePas, MD, MS

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Sep 2016. | This topic last updated: Dec 15, 2015.

INTRODUCTION — Henoch-Schönlein purpura (HSP), also called immunoglobulin A vasculitis (IgAV) [1], is the most common form of systemic vasculitis in children. Ninety percent of cases occur in the pediatric age group. In contrast to many other forms of systemic vasculitis, HSP (IgAV) is self-limited in the great majority of cases. The disease is characterized by a tetrad of clinical manifestations:

●Palpable purpura in patients with neither thrombocytopenia nor coagulopathy

●Arthritis/arthralgia

●Abdominal pain

●Renal disease

The clinical manifestations, pathogenesis, diagnosis, and differential diagnosis of HSP (IgAV) are presented here. The management of HSP (IgAV) and a more complete discussion of the renal manifestations of HSP (IgAV) are found elsewhere. (See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management" and"Renal manifestations of Henoch-Schönlein purpura (IgA vasculitis)".)

EPIDEMIOLOGY — HSP (IgAV) is primarily a childhood disease that occurs between the ages of 3 and 15 years [2]. In a population-based study from the United Kingdom, the annual incidence was about 20 per 100,000 in children ................
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