What is Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Oct 28, 2020
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS). HSP has been renamed IgA vasculitis (IgAV), but the eponymic name remains in wide use. IgAV is the most common vasculitis in childhood, the incidence decreasing with age. [1] The disease is more benign in children than adults. See the image below.

Characteristic rash of IgA vasculitis (Henoch-Schönlein purpura).

Characteristic rash of IgA vasculitis (Henoch-Schö Characteristic rash of IgA vasculitis (Henoch-Schönlein purpura).

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