The dystrophic form of congenital epidermolysis bullosa causes skin lesions resembling those of patients with severe bums. The loosening of the skin is permanent and occurs repeatedly, in a series of flare-ups. The mucous membranes are particularly severely affected. Skin care requires numerous personnel and pain management is of prime importance. The management of this condition is therefore highly specialised. The prognosis is very poor with dystrophic epidermolysis bullosa typically resulting in the death of the patient. (C) 2001 Editions scientifiques et medicales Elsevier SAS.