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Xeroderma pigmentosum in Libya.

M L Khatri1, M Bemghazil, M Shafi

  • 1Department of Dermatology, Faculty of Medicine, Al-Fateh University of Medical Sciences and Central Hospital, Tripoli, Libya.

International Journal of Dermatology
|August 10, 1999
PubMed
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Xeroderma pigmentosum (XP) in Libya presents with early onset, severe skin and eye lesions, and high cancer rates. Etretinate shows promise in preventing cancer but does not cure XP.

Area of Science:

  • Genetics and Human Diseases
  • Dermatology
  • Oncology

Background:

  • A preliminary study of 24 xeroderma pigmentosum (XP) cases was published in 1992.
  • An additional 18 XP cases have been identified since the initial study.

Purpose of the Study:

  • To investigate the clinical characteristics of xeroderma pigmentosum (XP) in the Libyan population.
  • To analyze the incidence, onset, manifestations, and outcomes of XP in Libyan patients.

Main Methods:

  • A detailed analysis of 42 XP cases (23 female, 19 male) from 29 families was conducted.
  • Patients were treated and followed between 1981 and 1994 using a specialized protocol.

Main Results:

  • XP in Libya has a high incidence (15-20 per million), with early onset of symptoms (median 12 months) and cancers (median 8 years).

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  • Consanguinity was noted in 39% of parents. Severe ocular and oral lesions were common, leading to early mortality (median 15.5 years).
  • Squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) were prevalent; malignant melanoma was absent. Etretinate therapy showed preventive effects against carcinogenesis.

    • Conclusions:

    • XP patients in Libya frequently exhibit severe ophthalmic issues at a young age.
    • Malignant melanoma was not observed, with only one case of lentigo maligna identified.