Childhood epidermolysis bullosa acquisita. Detection in a 5-year-old girl.
A 5 1/2-year-old girl with a blistering disease involving the skin and the oral, ocular, and anogenital mucosa is described. The initial clinical, histologic, and immunofluorescence findings suggested a diagnosis of cicatricial pemphigoid. However, immunoelectron microscopy demonstrated linear deposits of several immunoreactants within the sub-lamina densa region of the dermoepidermal junction, consistent with the diagnosis of epidermolysis bullosa acquisita. Although epidermolysis bullosa acquisita is considered a disease of adult onset, it should be included in the differential diagnosis of blistering diseases in children.
D001485:Basement Membrane / Q000276:immunology; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D003622:Dapsone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004820:Epidermolysis Bullosa / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008854:Microscopy, Electron; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis; D012867:Skin / Q000276:immunology
D003176:Complement C3; D007074:Immunoglobulin G; D003622:Dapsone