title: Chronic bullous disease of childhood and a paecilomyces lung infection in chronic granulomatous disease.
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).
authors.affiliation: Department of Dermatology, University of Amsterdam, Netherlands.
authors.forename: J H
authors.initials: JH
authors.lastname: Sillevis Smitt
authors.forename: J H
authors.initials: JH
authors.lastname: Leusen
authors.forename: H G
authors.initials: HG
authors.lastname: Stas
authors.forename: A H
authors.initials: AH
authors.lastname: Teeuw
authors.forename: R S
authors.initials: RS
authors.lastname: Weening
mesh_terms: D000666:Amphotericin B / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D002648:Child; D004359:Drug Therapy, Combination; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D017964:Itraconazole / Q000627:therapeutic use; D008172:Lung Diseases, Fungal / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D008297:Male; D010143:Paecilomyces; D010750:Phosphoproteins / Q000172:deficiency; D011239:Prednisolone / Q000627:therapeutic use; D012872:Skin Diseases, Vesiculobullous / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism
chemical_list: D000893:Anti-Inflammatory Agents; D000935:Antifungal Agents; D010750:Phosphoproteins; C079789:neutrophil cytosol factor 67K; D017964:Itraconazole; D000666:Amphotericin B; D011239:Prednisolone