Cancer protection in xeroderma pigmentosum variant (XP-V)

We describe herein a brother and sister diagnosed with xeroderma pigmentosu m variant (XP-V) in early adult life, who presented with increased sensitiv ity to sunlight and with cutaneous carcinomas on sun-damaged skin. The 27-y ear-old male farmer (Case 1.) was diagnosed with advanced squamous cell car cinoma (SCC) and multiple actinic lesions. Surgical removal of these lesion s was performed Three months later he died of multiple pelvic metastases of SCC. His 29-year-old sister (Case 2.) was operated on for different tumors , histologically SCC-s or basal cell carcinomas (BCC), or praecancerous con ditions many times. After a two year interval she was treated with low dose isotretinoin (2 mg/body weight). Diagnosis of XP-V was based on unschedule d DNA analysis (USD) and on clinical symptoms. We observed that during the long lasting isotretinoin treatment the tumor frequency dropped to a quarte r. Therefore, the isotretinoin treatment seems to be a good approach for ca ncer prevention in conditions with high predisposition to skin cancer, such as in XP-V.