Acquired epidermodysplasia verruciformis presenting as isolated genital hypopigmentation in a renal transplant recipient: a rare case report.

Source: PubMed, NCBI / U.S. National Library of Medicine

Epidermodysplasia verruciformis (EV) is a rare genodermatosis resulting from mutations in the EVER1/EVER2 genes, that increase vulnerability to beta-human papillomavirus (β-HPV) infection. The acquired form (AEV) develops in immunocompromised individuals, particularly organ transplant recipients, and typically manifests as pityriasis versicolor-like macules on sun-exposed areas. However, lesions confined to non-sun-exposed regions, especially the genitalia, are exceedingly uncommon and may be misdiagnosed. We report the case of a 38-year-old woman, nine years post-renal transplantation and on long-term triple immunosuppressive therapy (cyclosporine, mycophenolate mofetil, and prednisolone), who presented with progressive, non-pruritic, well-demarcated hypopigmented macules localized exclusively to her labia majora and mons pubis. The lesions were initially misdiagnosed and unsuccessfully treated as tinea versicolor. A skin biopsy revealed histopathologic features characteristic of EV, including enlarged keratinocytes with blue-gray cytoplasm and perinuclear halos and koilocytotic changes. This case emphasizes that AEV should be considered in the differential diagnosis of genital hypopigmented lesions in immunosuppressed patients. Accurate histopathologic evaluation is crucial for timely recognition, appropriate management, and ongoing surveillance to prevent missed malignant transformation.