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Epidermolysis Bullosa Dystrophica With Epidermal Neoplasms
William B. Reed, MD;
Joseph College, Jr., MD;
Martin J. O. Francis, PhD;
Hugh Zachariae, MD;
Frederic Mohs, MD;
Mary Ann Sher, BM, BCH;
Ian B. Sneddon, MD, FRCP
Arch Dermatol. 1974;110(6):894-902.
Abstract
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.
Author Affiliations
From the University of California, Irvine (Dr. Reed) and San Diego (Dr. College); the Nuffield Orthopedic Hospital, Oxford, England (Dr. Francis); the University of Aarhus Medical School, Aarhus, Denmark (Dr. Zachariae); the University of Wisconsin Medical School, Madison (Dr. Mohs); University of Johannesburg Medical School, South Africa (Dr. Sher); Rupert Hallam Hospital, Department of Dermatology, Sheffield, England (Dr. Sneddon).
Footnotes
Accepted for publication May 21, 1974.
Reprint requests to 1013 W Olive Ave, Burbank, CA 91505 (Dr. Reed).
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