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Epidermolysis bullosa acquisita in childhood
Author(s) -
Su John C,
Varigos George A,
Dowling John
Publication year - 1998
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/j.1440-0960.1998.tb01240.x
Subject(s) - epidermolysis bullosa acquisita , medicine , dermatology , epidermolysis bullosa , immunology , autoantibody , antibody
SUMMARY This case report of an 11‐year‐old girl describes a juvenile form of epidermolysis bullosa acquisita, an autoimmune disease of IgG antibodies to basement membrane type 7 collagen. Our case illustrates an unusually severe, acute inflammatory presentation of this condition with prominent mucosal and constitutional features requiring admission to a paediatric burns unit. The treatment consisted of supportive topical and systemic agents, prednisolone and dapsone. She responded to dapsone alone and the course of the illness was uneventful.