Epidermolysis bullosa dystrophica polydysplastica. A case of anesthetic management in oral surgery

Epidermolysis bullosa dystrophica is a rare disease that affects the skin and mucous membranes. Manifest at birth, it is characterized by poor dentition, esophageal strictures, syndactyly, and severe chronic anemia. Our 12-year-old patient required extensive dental treatment which necessitated overcoming problems of anesthesia as well as developing a technique of management that provided maximum safety and a minimum of discomfort. Transmission electron microscopy of sections of the gingiva revealed possible degenerative collagen fibers and an interrupted basement membrane. Anchoring fibrils normally found in the connective tissue beneath the epithelium were absent.