Henoch-Schönlein purpura (HSP) is the most common vasculitis seen in children with 90% of the presentations occurring before the age of 10 years. Pathogenesis involves a systemic, immune-mediated small vessel vasculitis that commonly affects the vessels supplying the skin, GI tract, kidney, and joints. Patient’s symptoms are directly related to the organ systems involved. All patients will have a palpable purpuric rash most commonly involving the lower extremities, more than 75% of patients will have a migratory arthritis of the knees and ankles as well as colicky abdominal pain, and roughly half of all patients diagnosed with HSP will have varying degrees of renal manifestations from mild proteinuria to severe, progressive glomerulonephritis requiring dialysis. Diagnosis may be difficult when patients present with vague symptoms such as abdominal pain before the rash becomes apparent. Treatment is mostly supportive with Tylenol for pain or fever; H2 blockers may be effective in some patients with mild GI symptoms. The role of steroids in the treatment of HSP is somewhat controversial. Steroids may decrease discomfort but does not impact the disease course of HSP or decrease sequelae.
KeywordsHenoch-Schönlein purpura (HSP) Vasculitis Palpable purpuric rash Proteinuria Progressive glomerulonephritis
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