Xeroderma pigmentosum in black south Africans
Xeroderma pigmentosum in black south Africans
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DOI：
10.1046/j.1365-4362.1999.00724.x
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发表时间：
1999-07-01
影响因子：
3.6
通讯作者：
Jacyk, WK
中科院分区：
文献类型：
--
作者：
Jacyk, WK
背景着色性干皮病发生在所有种族。有很少的信息，其临床表现，频率和类型的恶性病变在个人的非洲黑人extraction.Methods 15个黑人南非患者，年龄从10个月至21岁，着色性干皮病进行了前瞻性研究。对所有患者进行详细的皮肤科、眼科和神经系统检查。对皮肤、结膜和舌恶性病变进行组织学评估。结果12例皮肤恶性肿瘤，主要是鳞状细胞癌，通常在同一时间几个。舌尖癌发生在5例患者和结膜癌4。皮肤和粘膜癌的发病年龄早于世界其他地区。三名患者有广泛的皮肤受累的特点，着色性干皮病，但没有发展成恶性肿瘤。他们最有可能属于一个互补组（S）与高利率的计划外脱氧核糖核酸（DNA）synthesization.Conclusions着色性干皮病在南非黑人的特点是非常早期发展的皮肤，眼，舌鳞状细胞癌，通常有一个快速和毁灭性的过程。
Background Xeroderma pigmentosum occurs in all races. There is little information on its clinical picture, frequency, and types of malignant lesions in individuals of African negroid extraction.Methods Fifteen black South African patients, aged from 10 months to 21 years, with xeroderma pigmentosum were prospectively studied. Detailed dermatologic, ophthalmologic, and neurologic examinations were carried out in ail patients. Cutaneous, conjunctival, and lingual malignant lesions were histologically assessed. Complementation studies were not performed.Results Twelve patients had cutaneous malignancies, predominantly squamous carcinomas, usually several at one time. Carcinomas of the tip of the tongue occurred in five patients and conjunctival carcinomas in four. Cutaneous and mucosal carcinomas developed at an earlier age than in the series from other parts of the world. Three patients had extensive skin involvement characteristic of xeroderma pigmentosum, but did not develop malignancies. They most likely belong to a complementation group(s) with higher rates of unscheduled deoxyribonucleic acid (DNA) synthesis.Conclusions Xeroderma pigmentosum in South African black people is characterized by the very early development of cutaneous, ocular, and tongue squamous cell carcinomas and usually has a rapid and devastating course.