A Guide to Clinical Differential Diagnosis of Oral Mucosal Lesions
Hereditary Diseases: Epidermolysis Bullosa

Epidermolysis bullosa* refers to a group of mostly inherited diseases which cause blisters and ulcers of skin and sometimes oral mucosa. In almost all patients the lesions begin at birth or early childhood, and there is often a familial history of the condition. A Nikolsky sign may be present. Lesions of skin are consistently present. Some forms of epidermolysis bullosa can result in scarring and restricted mouth opening. Enamel defects of the teeth can also be present.

There is no cure for this group of diseases. The severity varies from mild (in the simplex form) to fatal (in the junctional and recessive forms). Antibiotics may be necessary to control infection associated with blisters and ulcers.