Introduction Henoch-Schonlein Purpura (HSP) is the most common form of systemic vasculitis in children. It is characterized by a tetrad of palpable purpura, arthralgia, abdominal pain and renal disease. It rarely presents with significant pericardial disease, especially in adults, and is a rare entity with only a few reported cases.
Case Description A 49 year old Male presented with a two month history of progressive dyspnea, polyarthralgia, fatigue, and skin rash. Physical exam showed cardiac dullness beyond the apical point of maximal impulse, elbow joints with limited range of motion and swelling, periungal erythema and palpable purpura over his extremities. He was found to have a large pericardial effusion on an echocardiogram with respiratory variation requiring drainage. Laboratory evaluation revealed markedly elevated C-Reactive Protein of 225. Biopsy of the skin purpura was consistent with IgA leukocytoclastic vasculitis with intense deposition of IgA within the superficial dermal blood vessel walls. Pericardial fluid was consistent with acute inflammation with several neutrophils but no growth on culture. The patient was started on high dose steroids with marked improvement in his symptoms including a decreased pericardial effusion. A repeat echocardiogram was obtained which showed only trivial pericardial effusion with no signs of tamponande.
Discussion HSP is a rather common disease in children with systemic hypersensitivity vasculitis in the skin and other organs including the kidneys. It rarely involves the myocardium or pericardium and is even rarer to present as a large pericardial effusion, especially in an adult. HSP presenting as pericardial disease has only been reported in a few cases. Although the pericardial involvement of Henoch-Schonlein Purpura seen in this patient is rare, any collagen vascular disease can present with pericardial effusion and might respond to appropriate anti-inflammatory therapies including steroids if recognized promptly.
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