Chronic bullous disease of childhood and a paecilomyces lung infection in chronic granulomatous disease.

Sillevis Smitt, J H; Leusen, J H; Stas, H G; Teeuw, A H; Weening, R S · Arch Dis Child · 1997

case_report · Level V

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Abstract

A 12 year old boy suffering from p67-phox deficient chronic granulomatous disease presented with a bullous skin disease and a lung infection with paecilomyces species. The histopathology of a bullous lesion showed subepidermal blister formation and microabcesses containing eosinophils in the dermal papillae. By direct immunofluorescence, linear staining of IgA at the dermal-epidermal junction was detected which confirmed the clinical diagnosis of chronic bullous disease of childhood (linear IgA dermatosis).

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