How is Henoch-Schönlein purpura (IgA vasculitis) characterized?

Updated: Jan 08, 2021
  • 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

IgA vasculitis (IgAV) was first described in 1802 by the English physician William Heberden, in two boys who presented with abdominal pain, purpuric rash, and arthralgia. [8]  The historical term for the condition honors the German pediatrician Eduard Heinrich Henoch and his teacher Johann Lukas Schönlein, who described the association of non-thrombocytopenic purpura with joint pain in 1837 and called the condition purpura rheumatica. [9] Henoch added GI and kidney involvement in 1874. [10]  In addition to Henoch-Schönlein purpura, IgAV has also been referred to as Schönlein-Henoch purpura, anaphylactoid purpura.

IgAV is an acute 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). [1, 11] It is a subset of necrotizing vasculitis characterized by fibrinoid destruction of blood vessels and leukocytoclasis.


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