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Epidermolysis bullosa acquisita in childhood
Authors:John C Su  George A Varigos  John Dowling
Institution:Department of Dermatology, Royal Children's Hospital, Parkville;Department of Pathology, Alfred Hospital, Prahran, Victoria, Australia
Abstract: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.
Keywords:autoimmune bullous disease  immuno-bullous disease  paediatric
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