Xeroderma pigmentosum. A clinical study of 24 Libyan cases.

BACKGROUND

Despite a high incidence of xeroderma pigmentosum, there is no previous publication from Libya.

OBJECTIVE

The purpose was to study the clinical profile of Libyan cases of xeroderma pigmentosum.

METHODS

With the help of a special protocol, 24 cases of xeroderma pigmentosum treated between 1981 and 1990 were subjected to detailed analysis.

RESULTS

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.

CONCLUSION

We observed early onset of severe ophthalmic lesions affecting a higher percentage of the patients.