Henoch–Schönlein purpura

Henoch–Schönlein purpura
Other namesIgA vasculitis,[1] anaphylactoid purpura,[2] purpura rheumatica,[2] Schönlein–Henoch purpura[2]
Typical purpura on lower legs and buttocks
Pronunciation
  • /ˈhɛnək ˈʃɜːnln, ˈʃn-/
SpecialtyRheumatology, Immunology

IgA vasculitis (HSP), previously known as Henoch–Schönlein purpura, is an autoimmune disease that most commonly affects children. In the skin, the disease causes palpable purpura (small, raised areas of bleeding underneath the skin), often with joint pain and abdominal pain. With kidney involvement, there may be a loss of small amounts of blood and protein in the urine (hematuria and proteinuria), but this usually goes unnoticed; in a small proportion of cases, the kidney involvement proceeds to chronic kidney disease. HSP is often preceded by an infection, such as a throat infection.

HSP is a systemic vasculitis (inflammation of blood vessels) and is characterized by deposition of immune complexes containing the antibody immunoglobulin A (IgA); the exact cause for this phenomenon is unknown. In children, it usually resolves within several weeks and requires no treatment apart from symptom control but may relapse in a third of cases and cause irreversible kidney damage in about one in a hundred cases. In adults, the prognosis is different from in children. The average duration of cutaneous lesions is 27.9 months.[3] For many, it tends to be relapsing–remitting over a long period of time, rather than self-limiting and there tend to be more complications.[4]

  1. ^ Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. (January 2013). "2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides". Arthritis and Rheumatism. 65 (1): 1–11. doi:10.1002/art.37715. PMID 23045170.
  2. ^ a b c Rapini RP, Bolognia JL, Jorizzo JL (2007). Dermatology. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  3. ^ Sais G, Vidaller A, Jucglà A, Servitje O, Condom E, Peyri J (March 1998). "Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients". Archives of Dermatology. 134 (3): 309–315. doi:10.1001/archderm.134.3.309. PMID 9521029.
  4. ^ Collins TR (August 2016). "Treatment Challenges, Uncertainty Abound with IgA Vasculitis". The Rheumatologist.