Heberden (1802) was the first to describe the association of macroscopic heamaturia with a purpuric rash, colicky pain, bloody stools and arthralgia. Erythematous or purpuric rash, and joint pain was reported again by Schonlein (1847). Schonlein's former pupil, Henoch (1874), described four children with the combination of rash, colic, bloody diarrhoea and joint pain and in a later report added heamorrhagic nephritis to the list of components of the syndrome, thus completing the modern definition of the disease. The latter has been formulated by The International Consensus Conference on Nomenclature of Systemic Vasculitides as ''a vasculitis with IgA-dominant immune deposits affecting small vessels and typically involving skin, gut and glomeruli and associated with arthralgias or arthritis'' (Jennette et al., 1994).
Berger and Hinglais (1968) reported for the first time a form of glomerulonephritis characterized by mesangial accumulation of IgA, which led later on to the denomination of IgA nephropathy (IgAN). At the same time, Urizar and co-workers (1968) showed similar IgA deposits in renal biopsies of patients with Henoch-Schonlein purpura nephritis (HSPN).
HSPN and IgAN are considered nowadays as related diseases since both can be encountered consecutively in the same patient (Ravelli et al.,
Pediatric Nephrology Unit, Het EKZ/Kinder AMC, 9, Meib-ergdreef, 1105 AZ Amsterdam Z-O, The Netherlands.
1969), have been described in identical twins (Meadow and Scott, 1985) and bear identical pathological and biological abnormalities (for a review: Davin et al., 2001; Knight, 1990).
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