[Acquired epidermolysis bullosa. A clinico-pathologic study].
Epidermolysis bullosa acquisita is an immunologically mediated mechano-bullous dermatosis presenting with a variety of clinical appearances. In mild cases the skin lesions, serous blisters, erosions and scars are restricted to the extremities. Scarring alopecia, dystrophy and even loss of nails as well as extensive erosions of mucous membranes may complicate severe cases. The characteristic histopathological feature is a subepidermal blister, which is located within the dermis by electron microscopy. Linear deposits of IgG and C3 along the basement membrane zone have been found on the dermal side of the lamina densa by immunoelectron microscopy. This close anatomical relationship with anchoring fibrils strongly suggests a functional disturbance of these anatomical structures, leading to dermolytic blistering. Treatment with sulphones, in combination with cortico-steroids in the more severe cases, has resulted in long-term improvement in two of our three patients.
D000328:Adult; D004820:Epidermolysis Bullosa / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D012867:Skin / Q000276:immunology / Q000473:pathology* / Q000648:ultrastructure
Epidermolysis bullosa acquisita. Eine klinisch-pathologische Studie.