Xeroderma pigmentosum. A clinical study of 24 Libyan cases.

Abstract

Despite a high incidence of xeroderma pigmentosum, there is no previous publication from Libya. The purpose was to study the clinical profile of Libyan cases of xeroderma pigmentosum. With the help of a special protocol, 24 cases of xeroderma pigmentosum treated between 1981 and 1990 were subjected to detailed analysis. The age of onset of initial manifestations ranged between 6 and 18 months whereas that of malignant lesions ranged from 2 to 10 years. Malignant lesions observed were squamous cell carcinoma in 15 patients, basal cell carcinoma in 12, and basosquamous carcinoma in 2 patients; squamous cell carcinoma of the tongue, carcinoma of the thyroid, and lymphatic leukemia affected individual cases. None of our patients developed malignant melanoma. Six patients have died; the age at death ranged between 9 and 18 years. A history of consanguinity in the parents of patients was recorded in all but two patients. We observed early onset of severe ophthalmic lesions affecting a higher percentage of the patients.