Management of squamous cell carcinoma in a patient with recessive-type epidermolysis bullosa dystrophica

BACKGROUND. Surgical treatment of a recessive dystrophic epidermolysis bullosa patient is very difficult for both the surgeon and the anesthetist because of the fragility of the skin and abnormal nature of the tumor bed. OBJECTIVE. We report a case of 54-year-old Japanese recessive dystrophic epidermolysis bullosa patient with squamous cell carcinoma (SCC) of the lateral malleolus. METHODS. A tumor measuring 5.0 x 5.5 cm was surgically excised. The defect was then reconstructed by full-thickness skin grafting. To avoid airway complications, general anesthesia was administered using a face mask. Because the regional lymph nodes were swollen before surgery, the patient underwent sentinel lymph node biopsy. RESULTS. The patient remains well with no sign of recurrence or metastasis 7 months after surgery. CONCLUSION. To preserve activities of daily living, surgery should be performed for squamous cell carcinomas arising in recessive dystrophic epidermolysis bullosa patients.