EPIDERMOLYSIS BULLOSA and A rare and severe dystrophic and congenital case.

Epidermolysis bullosa is a group of acquired or inherited mechanobullous skin disorder, characterized by blister formation in response to physical traumas and hot weather. They differ according to severity, clinical feature, prognosis, histology and heredity pattern. Case report: Miss J is a 24 years old girl. She was admitted at Emam-Reza hospital, Mashhad University of Medical Sciences, Iran due to confusion, severe anemia, respiratory distress, sepsis, general weakness and generalized skin bullae. The skin bullae have appeared since the third day of her life. The patient looks like a severely pale small child. Her body weight was 24 kg and her physical growth was far less than her chronological age. Her skin biopsy was compatible with epidermolysis bullosa. Her general feature improved after administration of fluid, blood products and wide spectrum antibiotics. While the patients might have life threatening medical problems, physicians should be familiar with manifestations and treatment of rare and severe cases of epidermolysis bullosa.   Key word: Epidermolysis bullosa,Dystrophic type, Gastrointestinal