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CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD: DIFFERENTIATION FROM OTHER AUTOIMMUNE BLISTERING DISEASES IN CHILDREN
Author(s) -
TSE YARDY,
LIM HENRY W.
Publication year - 1994
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4362.1994.tb02868.x
Subject(s) - medicine , hyperpigmentation , dapsone , trunk , direct fluorescent antibody , dermatology , physical examination , pathology , antibody , surgery , immunology , ecology , biology
An 8‐year‐old girl presented with a 1‐week history of intensely pruritic vesicles and bullae on the trunk and extremities. The patient was otherwise in good health and was not taking any medications. Physical examination revealed multiple large, tense bullae on normal‐appearing skin on the trunk, extremities, and pelvic region. Some of the bullae were hemorrhagic, while others were grouped in circular clusters (Fig. 1A, B). The oral cavity and conjunctivae were spared. Complete blood cell count, electrolytes, serum urea nitrogen, creatinine, and liver function studies were normal. Histopathologic examination of perilesional skin demonstrated a subepidermal blister with neutrophils in the dermal papillae (Fig. 2). Direct immunofluorescence of the specimen showed heavy deposition of IgA in a linear pattern in the basement membrane zone (Fig. 3). Broad but faint deposits of IgM were also present. Blood obtained for indirect immunofluorescence revealed no circulating autoantibodies. The patient was diagnosed with chronic bullous dermatosis of childhood and treated with dapsone (2 mg/kg) with dramatic improvement. New blister formation and puritis were suppressed within several days. All lesions healed with mild hyperpigmentation but without scarring.