Henoch-Schonlein Purpura (HSP) or IgA Vasculitis, is the most common childhood vasculitis.
The underlying pathogenesis is uncertain however the structural end organ injury is caused by the deposition of IgA molecules due to a reduction in glycosylation. It is classified as a small vessel immune complex vasculitis.
There is a tetrad of the core clinical manifestations:
- Purpura – Most often present on areas of pressure or gravity dependence
- Arthritis or arthralgia
- Abdominal Pain – Often due to small bowel involvement and can be complicated by intussusception
- Renal involvement – It is possible for single organ deposition of IgA to occur, with IgA nephropathy not distinguishable histologically from a systemic IgA vasculitis. Renal failure is the most common long term sequelae of IgA vasculitis and can initially present with hypertension or evidence of nephrotic syndrome
IgA Vasculitis has a good prognosis and is most often self-limiting. The main stay of management is ensuring adequate hydration and analgesia, with any other complications investigated and addressed as indicated. There is often a history of a preceding infection affecting the gastrointestinal system or upper respiratory tract. Assessment should include monitoring of blood pressure and urinalysis for haematuria/proteinuria. If initially normal in an otherwise well child these are followed periodically with development of hypertension or haematuria/proteinuria prompting further investigation and management.
1802 – First described by William Heberden the elder, a London based physician who described a case of a 4 year old boy with all four hallmarks of HSP: gastrointestinal involvement, kidney involvement, arthritis, and purpura. Initially known as Heberden-Willan disease [Ch 78 De purpureis maculis; or ]
1837 –Johann Lukas Schönlein described an association between the presence of purpura and arthritis
1874 and 1899 – Eduard Heinrich Henoch, a student of Schönlein’s, added gastrointestinal and renal involvement respectively, to the description
1920 – Eduard Glanzmann reported on the allergic components of HSP as ‘Anaphylaktoide Purpura‘
1990 – American College of Rheumatology published diagnostic criteria for HSP including palpable purpura; age 20yrs or younger at onset; bowel angina; and wall granulocytes on biopsy. A patient requires at least 2 of these criteria for a diagnosis of HSP
2012 – The International Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed replacing the term Henoch-Schonlein Purpura with the term IgA Vasculitis to better reflect the underlying pathophysiology
- William Heberden (1710–1801)
- Johann Lukas Schönlein (1793-1864)
- Eduard Heinrich Henoch (1820-1910)
- Eduard Glanzmann (1887-1959)
- Schönlein-Henoch Purpura
- Schönlein–Henoch–Glanzmann Purpura
- IgA Vasculitis
- Heberden-Willan disease
- Schönlein JL. Allgemeine und specielle Pathologie und Therapie: Nach dessen Vorlesungen niedergeschrieben und herausgegeben von einigen seiner Zuhörer [General and Special Pathology and Therapy: After its lectures written down and published by some of its listeners] – [HSP description in 5th edition; Volume 1: S 41]
- Henoch EH. Über den Zusammenhang von Purpura und Intestinalstörungen. In: IV Verhandlungen ärztlicher Gesellschaften. Berliner Klinische Wochenschrift. 1868;5:517-519. [PDF]
- Henoch EH. Über eine eigentümliche Form von Purpura. Berliner klinische Wochenschrift. 1874;11:641-643. [Henoch-Schönlein Purpura]
- Glanzmann E. Die Konzeption der Anaphylaktoiden Purpura. Jahrbuch für Kinderheilkunde und physische Erziehung. 1920;91:371
- Matteson EL. Notes on the history of eponymic idiopathic vasculitis: the diseases of Henoch and Schönlein, Wegener, Churg and Strauss, Horton, Takayasu, Behçet, and Kawasaki. Arthritis Care Res. 2000 Aug;13(4):237-45.
- Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013 Oct;17(5):603-606
- Trnka P. Henoch-Schönlein purpura in children. J Paediatr Child Health. 2013 Dec;49(12):995-1003.
- Ortiz-Sanjuán F, Blanco R, Loricera J, Hernández JL, Pina T, Calvo-Río V, Alvarez L, González-Vela MC, Rueda-Gotor J, González-López MA, González-Gay MA. Reappraisal of the 1990 American College of Rheumatology criteria for the classification of cutaneous vasculitis: an analysis based on 766 patients. Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S51-4.
- Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schönlein Purpura: A Literature Review. Acta Derm Venereol. 2017 Nov 15;97(10):1160-1166.
the names behind the name