色素性乾皮症 (XP) 患者における基底細胞上皮腫

概要

論文の詳細を見る
Clinical and histopathological examinations were carried out on 133 basal cell epitheliomas (BCEs) from 23 patients with xeroderma pigmentosum (XP) . A total of 69 BCEs occurred in 10 patients with XP-A (age at first excision: 6-22y, average: 11.5y) ; 34 in 8 XP-variant (22-47y, 30.8y) ; 16 in 1 XP-E; 9 in 2 XP-F; 4 in 1 XP-D; and 1 in 1 XP-C. Of the 133 BCEs, 131 (99%) developed on sun-exposed areas. Clinically, most cases presented as a small, black, glossy nodule. Histopathologically, the solid type of BCE was most common (94%) . The age of onset of skin cancer is characteristically earlier in patients with XP. Although the mean age at first excision was 24 years among the 23 XP patients in this study, it differed considerably from one XP group to another. Ninety seven percent of the BCEs developed on the face in the patients with XP. This high incidence in comparison with 70% in patients without XP suggests that sunlight plays a significant role in the development of skin cancers in XP.