Hypergammaglobulinemic purpura of Waldenstrom: Report of 3 cases with a short review

Benign hypergammaglobulinemic purpura of Waldenstrom (HGPW) is an uncommon cause of non-thrombocytopaenic purpura that may create diagnostic difficult ies. The presence of constitutional symptoms associated with prominent immu nological abnormalities may raise alarm, leading to extensive and often unn ecessary investigations. This report describes 3 young women with HGPW. Cli nical features were characterised by recurrent episodes of bilateral asymme trical palpable purpuric lesions on the lower extremities that were precipi tated by a prolonged increase in hydrostatic pressure (e.g. prolonged stand ing, tight stockings etc.) associated with constitutional features. In one patient the condition was secondary to Sjogren's syndrome with type IV rena l tubular acidosis. Laboratory abnormalities included a persistently elevat ed erythrocyte sedimentation rate, marked polyclonal hypergammaglobulinemia , and high titers of rheumatoid factor and anti-nuclear antibody of the ant i-SSA (anti-Ro)/anti-SSB(anti-La) subsets. This topic is reviewed briefly w ith the emphasis that in its 'primary' form this condition could be conside red a 'benign' systemic immunoinflammatory disease that requires neither ex tensive investigation nor any aggressive form of therapy. Greater awareness of HGPW may increase the frequency of its diagnosis, especially in the pat ient group with non-thrombocytopenic purpura or the so-called cutaneous vas culitic syndromes with 'palpable purpura'.