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Childhood Bullous Pemphigoid Successfully Treated with Diaminodiphenyl Sulfone
Author(s) -
Motegi Seiichiro,
Abe Masatoshi,
Tamura Atsushi,
Ishii Norito,
Hashimoto Takashi,
Ishikawa Osamu
Publication year - 2005
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2005.tb00849.x
Subject(s) - bulla (seal) , bullous pemphigoid , basement membrane , pathology , medicine , infiltration (hvac) , autoantibody , antibody , antigen , epidermolysis bullosa acquisita , immunofluorescence , dermatology , immunology , physics , lung , thermodynamics
Bullous pemphigoid (BP) is an acquired autoimmune blistering disease which predominantly affects the elderly. It is rare in children and infants. We reported a 14‐year‐old girl presenting with a month history of relapsing tense bullae on the face and extremities. Histopathological examination of the lesional skin revealed a subepidermal bulla with infiltration of eosinophils, neutrophils, and lymphocytes. Direct immunofluorescence showed linear deposits of IgM and C3 at the basement membrane zone. Indirect immunofluorescense using normal human skin sections as a substrate detected IgG anti‐basement membrane zone antibodies in the patient's serum and that using 1M NaCl split skin sections showed that the patient's antibodies bound to the epidermal side of the split skin. Immunoblot analysis using normal human epidermal extracts demonstrated the presence of autoantibodies against the 230‐kDa BP antigen. Furthermore, the patient's serum reacted with the recombinant protein of the NC16a domain of the 180‐kDa BP antigen by immunoblot analysis and enzyme‐linked immunosorbent assay. Our patient showed significant improvement of the skin lesions with systemic administration of diaminodiphenyl sulfone.

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