Management challenges of epidermodysplasia verruciformis in a resource-limited setting: A retrospective review

Abstract

Background: Epidermodysplasia verruciformis (EV) is a rare genodermatosis with autosomal recessive inheritance linked to human papilloma virus 5 and 8. It presents with flesh-colored or hypopigmented macules and plaques on the face, neck, hands, and body. They are asymptomatic but cause great cosmetic concern to affected individuals. An immune deficient state renders patients more susceptible to it; although some have no demonstrable ill health. Diagnosis is mostly clinical; nevertheless, skin biopsy for histopathology may be performed in doubtful cases. Treatment is aimed at cosmetic removal with retinoids, Imiquimod, and keratolytics among others. The aim of this report is to document the clinical profile of patients with EV, and highlight the challenges faced in managing patients. Materials and Methods: The clinical records from January 2006 to December 2013 were reviewed. Demographic data, HIV status, and biopsy results were extracted and entered onto Microsoft Excel spreadsheet. The results were analyzed with SPSS version 18. Results: A total of 33, 326 patients were seen during the review period; 96 (0.3%) had a clinical diagnosis of EV with 45 (46.9%) males and 51 (53.1%) females. The mean age was 20.8 ± 13.4, with a range of 2–52 years. A quarter of the patients were tested for HIV and 12 (13%) were positive. Conclusions: This study documents the frequency of EV in our environment and provides a baseline for more studies. The results concur with its rare occurrence worldwide and fail to show the exact relationship between HIV infection and EV. The challenges to managing these patients are mostly due to financial constraints; hence, basic investigations and optimal treatment could not be done in many of the patients.

Keywords

• Epidermodysplasia verruciformis
• genodermatoses
• human papiloma virus
• resource limited