Henoch Schonlein Purpura syndrome

Henoch Schonlein Purpura is a clinical syndrome comprises a characteristic skin rash, abdominal colic, joint pain and glomerulonephritis.

The rash is of purpuric type and the synonym Henoch-Schönlein purpura is often used. It occurs at all ages and in both sexes, but is mainly a disease of early childhood. Males are twice as often affected as females. A recent history of infection, often respiratory, is common.

The disease is rare in adults. Serum concentrations of IgA are increased in about half the patients during the first 3 months of the disease and IgA-containing immune complexes have been detected in serum in a high proportion of cases. The renal lesion is a focal segmental proliferative glomerulonephritis, sometimes with mesangial hypercellularity.

Epithelial crescents may be present. Immunoglobulin deposition, mainly of IgA, is seen in the glomerular mesangium and to a lesser extent in the capillary walls on immunofluorescence. IgG, IgM and components of the complement system may also be detectable.

Electron-dense deposits, presumably immune complexes, are seen in the mesangium and subendothelial position on electron microscopy. No treatment is of proven benefit.