Xeroderma pigmentosum variant associated with multiple cancers

A 62-year-old Japanese man with xeroderma pigmentosum (XP) variant is repor ted. The patient had developed at least 6 basal cell carcinomas, a squamous cell carcinoma, and a malignant melanoma on sun-exposed areas, and an atyp ical carcinoid on the right lung. In vivo phototesting showed a normal resp onse. The minimal erythema dose of ultraviolet B (UVB) was not lowered and no delayed peaking of the erythema reaction was observed. His skin fibrobla sts exhibited higher sensitivity to UV irradiation, but a normal level of u nscheduled DNA and RNA synthesis. Cell fusions with XP group A, C, D, E, F, and G cells after UV irradiation were all complemented. Previous reports t ogether with this case suggest that older XP variant patients have a high f requency of not only skin cancers, but also internal malignancies.