Henoch-Schönlein Purpura in children

Abstract

Henoch-Schlein purpura (HSP) is an IgA-mediated systemic small-vessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. Approximately 75% of cases occur in children aged between 2 to 11 years, with a peak incidence at 4 to 7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria: diffuse abdominal pain, arthritis or arthralgia, renal involvement (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most cases are selflimited. The average duration of the condition is 4 weeks. Recurrences are reported in up to 33% of patients. Therapy consists of general and supportive measures as well as treatment of sequelae of the vasculitis. Oral steroids may be considered for patients with severe gastrointestinal involvement. Unfortunately, early steroid treatment does not reduce the incidence and severity of nephropathy in patients with HSP.