A novel approach to the classification of epidermodysplasia verruciformis. (29th August 2018)

• Record Type: Journal Article
• Authors: Huang, Simo; Wu, Julie H.; Lewis, Daniel J.; Rady, Peter L.; Tyring, Stephen K.
• Is Part Of: International journal of dermatology. Volume 57:Number 11(2018)
• Page Start: 1344
• Page End: 1350
• Publication Date: 2018-08-29
• Subjects: Dermatology -- Periodicals; 616.5
• DOI: 10.1111/ijd.14196
• Languages: English
• ISSNs: 0011-9059

Abstract

Abstract: 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 .