Xeroderma pigmentosum in black South Africans.

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 all 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.