Nevoid basal cell carcinoma syndrome: combined etretinate and surgical treatment.

Aromatic retinoids have been shown to be useful in the management of various genodermatoses, including nevoid basal cell carcinoma syndrome (NBCCS). A patient with this condition was treated by combined etretinate and surgical therapy. Over the course of 12 months, the patient was placed on oral etretinate therapy, with a dosage of 1 mg/kg/day for 3 months, and 0.5 mg/kg/day for 9 months. In the first period, notable tumor reduction was observed in the larger tumors. Complete clinical regression was seen in only 26% of BCCs with an overall tumor size of less than 1 cm in diameter. In the second period, no tumor changes were observed, but no new tumors appeared. Surgical excisions of the remaining tumors were carried out 3 months after the end of the etretinate treatment, with excellent results and no recurrences after 6 months of follow-up. Treatment with etretinate permits a less aggressive surgical excision and recurrence rate than surgical excision alone.