Xeroderma pigmentosum. A clinical study of 24 Libyan cases.

Abstract:

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.

journal_name

J Am Acad Dermatol

authors

Khatri ML,Shafi M,Mashina A

doi

10.1016/0190-9622(92)70010-d

subject

Has Abstract

pub_date

1992-01-01 00:00:00

pages

75-8

issue

1

eissn

0190-9622

issn

1097-6787

pii

0190-9622(92)70010-D

journal_volume

26

pub_type

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