Xeroderma pigmentosum variant or pigmented xerodermoid.

We describe herein a 29-year-old patient who was recognized as having a xeroderma pigmentosum variant or pigmented xerodermoid in the early adult life since sunlight sensitivity caused degenerative changes in the skin and cutaneous carcinomas. The patient had 11 skin tumours removed over an interval of two years, of which four proved to be squamous cell carcinoma, one basal cell carcinoma and six precancerous conditions. Then the patient was treated with isotretinoin at a dosage of 2 mg per kilogram of body weight per day for two years. During two years of treatment with isotretinoin a further three tumours were removed givind a histological result of basal cell carcinoma and Bowen's diseases. The patient tolerated well this low dosage retinoid treatment. Only 50 or so of these patients are described in the world literature.