SQUAMOUS-CELL CARCINOMA SECONDARY TO RECESSIVE DYSTROPHIC EPIDERMOLYSIS-BULLOSA - A REPORT OF 4 PATIENTS WITH 17 PRIMARY CUTANEOUS MALIGNANCIES

Four patients with recessive dystrophic epidermolysis bullosa and 17 invasive primary cutaneous squamous cell carcinomas (SCCs) are presented. All skin cancers arose at the site of scarring due to recessive dystrophic epidermolysis bullosa. Three of 17 (18%) primary cutaneous SCCs recurred locally following initial treatment with either surgical excision or wide surgical excision. Patients with recessive dystrophic epidermolysis bullosa are more likely to develop SCCs of the extremities than nonimmunocompromised patients or patients with epidermodysplasia verruciformis. Like patients with epidermodysplasia verruciformis, patients with recessive dystrophic epidermolysis bullosa are likely to suffer from invasive and metastatic SCCs at a much younger age than nonimmunocompromised patients.