Squamous cell carcinoma complicating recessive dystrophic epidermolysis bullosa-Hallopeau-Siemens: a report of four cases.

Background  Recessive dystrophic epidermolysis bullosa (RDEB), an autosomal-recessive genodermatosis, is one of the more severe forms of the epidermolysis bullosa dystrophica group, and is characterized by generalized blistering of the skin and mucous membranes. Cutaneous squamous cell carcinoma is one of the most serious complications of this disease. Methods  We report four patients (three females and one male), two of whom were under 20 years of age, suffering from RDEB-Hallopeau–Siemens. Results  All patients developed well-differentiated squamous cell carcinoma. No metastases were detected. All cases were treated surgically. Fatal evolution was noted in one patient. A second tumor was detected in another patient during the follow-up period. No further tumors or metastases were observed in the other patients. Conclusions  Regular clinical and histologic examination of any nodular lesions or non healing ulcers of all patients suffering from RDEB-Hallopeau-Siemens to detect an early malignancy is recommended.