RADIATION-THERAPY FOR SQUAMOUS-CELL CARCINOMA IN DYSTROPHIC EPIDERMOLYSIS-BULLOSA - CASE-REPORTS AND LITERATURE-REVIEW

Dystrophic epidermolysis bullosa (DEB) is a debilitating systemic dise ase frequently associated with biologically aggressive secondary squam ous cell carcinomas arising from affected skin or mucosal surfaces. Tr eatment of these carcinomas with surgery, chemotherapy, or radiation i s complicated by inherently poor wound healing. We report on two DEB p atients treated with radiation therapy for locally advanced squamous c ell carcinoma, and retrospectively analyze 10 DEB patients treated wit h radiation, reported in the literature. Of the 11 fully available and described case results from radiation therapy, six (54%) patients dem onstrated a partial tumor response. All patients receiving > 4,500 cGy developed moist skin desquamation and delayed skin healing. Radiation therapy may be of benefit in palliating DEB patients who have locally advanced carcinoma, but has been associated with enhanced normal tiss ue toxicity, suggesting a narrow or absent therapeutic index between i rradiated carcinoma and skin.