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Xeroderma pigmentosum in Yemen.

Mishri Lal Khatri1

  • 1Department of Dermatology, Saudi Hospital at Hajjah, Sana'a, Republic of Yemen.

International Journal of Dermatology
|February 4, 2021
PubMed
Summary

Xeroderma pigmentosum (XP) in Yemen shows a high incidence with early onset of skin issues and frequent consanguinity. Treatment with acitretin yielded positive outcomes for affected individuals.

Area of Science:

  • Dermatology
  • Genetics
  • Epidemiology

Background:

  • Xeroderma pigmentosum (XP) incidence in Yemen is notably high, yet undocumented.
  • This study addresses the lack of previous reports on XP in the Yemeni population.

Purpose of the Study:

  • To investigate the clinical and epidemiological characteristics of Xeroderma pigmentosum (XP) in Yemen.
  • To document the presentation, progression, and treatment outcomes of XP patients in this region.

Main Methods:

  • A retrospective analysis of 40 XP patients (24 male, 16 female) from 32 families treated between 1997 and 2014.
  • Diagnosis confirmed via clinical features and histopathology; tumor confirmation by histopathology.
  • Standardized protocol used for data collection on patient demographics, clinical manifestations, and treatment.

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Main Results:

  • Median onset of initial manifestations at 9.5 months, malignant lesions at 7 years.
  • High consanguinity rate (38/40 patients); initial symptoms include skin dryness and facial freckling.
  • Significant rates of premalignant and malignant lesions: actinic keratosis (15), squamous cell carcinoma (10), ocular SCC (7), and malignant melanoma (2).

Conclusions:

  • XP in Yemen presents with high incidence, early onset, significant consanguinity, and severe ocular/oral manifestations.
  • Acitretin demonstrated positive therapeutic results in 12 treated patients.
  • Findings highlight the need for increased awareness and management strategies for XP in Yemen.