Xeroderma pigmentosum variant associated with multiple cancers.
A 62-year-old Japanese man with xeroderma pigmentosum (XP) variant is reported. The patient had developed at least 6 basal cell carcinomas, a squamous cell carcinoma, and a malignant melanoma on sun-exposed areas, and an atypical carcinoid on the right lung. In vivo phototesting showed a normal response. The minimal erythema dose of ultraviolet B (UVB) was not lowered and no delayed peaking of the erythema reaction was observed. His skin fibroblasts exhibited higher sensitivity to UV irradiation, but a normal level of unscheduled 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 together with this case suggest that older XP variant patients have a high frequency of not only skin cancers, but also internal malignancies.
Department of Dermatology, Kansai Medical University, Osaka, Japan.
K
K
Kuwamoto
H
H
Miyauchi-Hashimoto
T
T
Isei
T
T
Horio
D002276:Carcinoid Tumor / Q000150:complications; D002280:Basal Cell Carcinoma / Q000150:complications; D002294:Carcinoma, Squamous Cell / Q000150:complications; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications*; D008297:Male; D008545:Melanoma / Q000150:complications; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology; D014983:Xeroderma Pigmentosum / Q000150:complications* / Q000473:pathology