A novel approach to the classification of epidermodysplasia verruciformis

BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare genodermatosis that causes disseminated eruptions of hypo- or hyperpigmented macules and wart-like papules that can coalesce and scale. It is uniquely characterized by an increased susceptibility to specific human papillomavirus (HPV) genotypes. Classically, EV is associated with mutations of the EVER1/TMC6 and EVER2/TMC8 genes. The term "acquired" epidermodysplasia verruciformis was coined to describe an EV-like syndrome that can develop in patients with a compromised immune system. Recent discoveries of other genes implicated in EV, including RHOH, MST-1, and CORO1A, have complicated the classification of EV and EV-like syndromes. METHODS: We review the available data on epidermodysplasia verruciformis in the literature in order to propose a new classification system to encompass current and future developments on EV and EV-like syndromes. RESULTS: We propose classifying EV into: (1) classic genetic EV, (2) non-classic genetic EV, and (3) acquired EV. CONCLUSION: The proposed categorization scheme provides a simple and logical way to organize the different cases of EV that have been described in the literature. This system organizes EV by its cause, allowing for a better understanding of the disease and helps differentiate EV from other causes of generalized verrucosis.