Lewandowsky–Lutz verruciform epidermodysplasia: a brief review and case report

Verruciform (warty) epidermodysplasia (Lewandowsky–Lutz syndrome) is a rare autosomal recessive disease manifested by multiple cutaneous lesions associated with human papillomavirus infection. To date, no more than 500 patients with this form of genodermatosis have been reported worldwide. Hereditary forms of verruciform (warty) epidermodysplasia are caused by inactivating mutations in the TMC6, TMC8, CIB1, RHOH, and IL7 genes in a homozygous or compound heterozygous state. A non-classical variant of hereditary verruciform (warty) epidermodysplasia has also been described; it is associated with primary T-cell immunodeficiency resulting from mutations in the CORO1A, CARMIL2, DCLRE1C, DOCK8, ECM1, GATA2, LCK, MST1, RASGRP1, and TPP2 genes. Acquired verruciform (warty) epidermodysplasia usually develops in patients with secondary immunodeficiency of various origins. The low prevalence and insufficient clinical diagnostic experience explain the lack of standardized therapy protocols and clinical guidelines for verruciform (warty) epidermodysplasia. Several researchers and clinicians have reported treatment of this condition using chemotherapeutic agents, immunomodulators, interferons, retinoids, and other drugs in combination with surgical excision or physical methods of lesion destruction. This article presents a case report of aggressive verruciform (warty) epidermodysplasia without preceding immunodeficiency or alterations in key genes, describing the experience of treating the patient with a retinoid in combination with CO2 laser destruction of cutaneous lesions.