[A case of xeroderma pigmentosum treated by oral etretinate]

A case of xeroderma pigmentosum (XP) with neurological disorders is reported. The patient is a 16 year old male who presented a marked photosensitivity from his early infancy. The family history is negative for XP with no consanguinity being observed. He displayed neurological symptoms such as a low IQ and audiological disturbances. He developed six basal cell epitheliomas (BECEs) on his face at age of 14. We tried oral etretinate for 4 years at a initial dosage of 30 mg per day for the possible prevention of new skin cancers. Efficacy of the drug for prevention of cancers has not been confirmed, but evidence is obtained that the drug might prevent the growth of tumors. We also reviewed the chemoprevention of skin cancers with retinoids, in patients with xeroderma pigmentosum.