Sk India Quiz 26: Hypopigmented papules in a human immunodeficiency virus-positive patient.

A 40-year-old human immunodeficiency virus. (HIV)-positive male presented with one year history of multiple, asymptomatic, hypopigmented lesions on upper limb and trunk. The patient was on zidovudine, lamivudine, and nevirapine for 9. months. His CD4 count was 92. cells/mm3. Physical examination showed scaly, discrete to confluent papules of 1-3. mm size and distributed symmetrically on shoulder, arms and trunk [Figure 1a]. Koebnerization was evident at some places. Some of the confluent lesions had figurate morphology [Figure 1b]. None of the family members had similar lesions. Potassium hydroxide examination of scraped hypopigmented skin lesions did not show fragmented hyphae. Histopathology showed broad plate like epidermal hyperplasia with focal hypergranulosis and several vacuolated cells (koilocytes) with abundant pale cytoplasm in the upper spinous layer [Figure 2a–b].

Figure 1

(a) Discrete to confluent hypopigmented papules on arms and (b) figurate morphology

Figure 2

(a) Photomicrograph showing epidermal hyperplasia with focal hypergranulosis (H and E, ×40). (b) Several vacuolated cells with abundant pale cytoplasm in the upper spinous layers (H and E, ×100)

ANSWER

Epidermodysplasia verruciformis (EV).

DISCUSSION

Epidermodysplasia verruciformis, first described in 1922 by Lewandowksi and Lutz, is a very rare, chronic disease, characterized by a unique susceptibility to cutaneous infections by a group of phylogenetically related human papilloma virus (HPV) types, referred as EV types.[1] In most EV-affected families, inheritance follows an autosomal recessive pattern and recently, truncating mutations in two genes, TMC6 (EVER 1) and TMC8 (EVER 2) have been identified as a cause of EV. About 2 dozen EV-specific HPV types have been described, a subset of which (mainly 5 and 8) is detected in EV-associated skin cancers.[2] Squamous cell carcinomas usually arise in pityriasis versicolor like EV-lesions on sun exposed areas. It usually manifests in childhood with persistent and widespread warts. Individual lesions typically have either the appearance of flat warts or flat, scaly red-brown or hypopigmented macules that resemble lesions of pityriasis versicolor.[3] Histopathologically EV is characterized by hyperkeratosis, acanthosis and prominent vacuolation in the Malphigian layer. Biopsies of EV lesions from HIV-positive patients report higher rates of dysplasia (63%) compared to the patients of EV without immunosuppression (20%).[4] However, our case did not reveal features of dysplasia. Treatment is usually less successful in HIV-associated EV than classical EV. In various series[567] of HIV patients with EV, different treatment modalities including systemic retinoids, interferon, imiquimod, and combination of systemic and topical therapies have been attempted and found to be either unsuccessful, inconsistent or associated with rapid recurrence after treatment. The diagnosis of EV should be considered in patients with HIV who present with pityriasis versicolor like disseminated hypopigmented lesions resistant to treatment with antifungal medications.[8]