Henoch-Schonlein purpura in Wiskott-Aldrich syndrome

Wiskott-Aldrich syndrome (WAS) is a rare immune deficiency disease. Sialoph orin glycosylation is defective in WAS. Although it is not very common, ren al involvement including IgA nephropathy (IgAN) was reported. Abnormal glyc osylation plays a key role in the pathogenesis of IgAN. We present an 8-yea r-old boy with WAS who had recurrent episodes of Henoch-Schonlein purpura w ith renal involvement following upper respiratory tract infections. His ren al function did not deteriorate. Both IgAN and WAS have glycosylation defec ts, but there must be some other factors (genetic and environmental) to exp lain their rare association.