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December 1974

Epidermolysis Bullosa Dystrophica With Epidermal Neoplasms

Arch Dermatol. 1974;110(6):894-902. doi:10.1001/archderm.1974.01630120044009

Patients with epidermolysis bullosa dystrophica (EBD) tend to develop epidermal neoplasms, usually low-grade squamous cell carcinoma of the skin and less commonly that of the mouth. There have been 21 reported cases in the literature, and we are adding seven new cases and providing a further history in a previously reported case.

The average EBD patient develops carcinomas after 30 years of age, and some of these patients live to be 60 years of age before they die from the carcinoma. More than two thirds of the patients die from metastases.

In our patients, carcinoma developed in the mouth, ostium cardiacum, and in the midesophagus, with extension into the lungs. The cause remains unknown, but abnormal collagen scarring is the most likely cause. Carcinoma can probably develop in all the severe dystrophic types of EBD.