Henoch-Schönlein purpura

Abstract

Although Henoch-Schönlein purpura (HSP) can occur at any age from infancy to adulthood, it is overwhelmingly a disease of childhood. Indeed, HSP is the most common vasculitis syndrome affecting children. The clinical features of HSP have been well documented, and the diagnosis is generally not difficult. However, there are substantial gaps in our understanding of the etiology, pathogenesis, and treatment of HSP. This article briefly reviews the clinical aspects of HSP and new information concerning therapy. The major focus of this review is recent information concerning abnormalities of immunoglobulin A1 glycosylation and the role of aberrantly glycosylated immunoglobulin A1 in the pathogenesis of HSP.