Henoch-Schönlein purpura (HSP)

• HSP is the most common form of systemic vasculitis in children (90% of cases occur in the pediatric age-group)
• HSP is an immune-mediated vasculitis associated with IgA deposition
• A variety of infectious and chemical triggers have been proposed, but the underlying cause of HSP remains unknown
• In contrast to many other forms of systemic vasculitis, HSP is self-limited in the great majority of cases
• The disease is characterized by the following of clinical manifestations:

o   Palpable purpura (in patients without thrombocytopenia or DIC)

o   Arthritis/arthralgia

o   Abdominal pain (bowel angina, GI bleeding); can be associated with intussuception in children

o   Renal disease (hematuria, renal failure)

 

HSP and IgA nephropathy seem to be part of a similar disease spectrum: HSP is distinguished clinically from IgA nephropathy by prominent systemic symptoms, a younger age (generally <20 years old), preceding infection, and abdominal complaints

(Victoria Kelly MD, 12/7/10)