Genital/perigenital inflammatory linear verrucous epidermal nevus: A case series

Abstract

Background:Inflammatory linear verrucous epidermal nevus (ILVEN) is a distinct variety of keratinocytic epidermal naevus. In contrast to non-inflammatory epidermal naevi, ILVEN are far less common, usually erythematous and intractably pruritic. ILVEN usually appears at birth or early childhood and has a linear distribution following the Blaschko lines. Genital/perigenital involvement is relatively rare.Objectives:To describe the clinical features of 9 children with ILVEN localized to the genital and perigenital areas.Method:A retrospective study of 9 children with ILVEN presenting to a tertiary care Dermatology Clinic between 2007 and 2014 was undertaken. The clinical and histopathological features were reviewed.Results:Nine children (6 females, 3 males) were included in the study based on their characteristic clinicopathological features. The lesions were associated with severe itching in all cases. The mean age at presentation was 4 years (range 1-11 years). Onset of lesions was before 6 months of age in 8 patients. Left sided involvement was twice as common as the right sided one. Male patients had penoscrotal and groin involvement while all the female children had vulvar lesions. None of the children had any extracutaneous abnormalities. The children were treated with topical agents with variable relief or symptoms.Conclusions:The possibility of ILVEN should be considered in every linear genital lesion in children. We have presented the largest series of perigenital ILVEN reported in English literature.

Efficacy of Chemoradiotherapy on Health-Related Quality of Life in Esophageal Cancer Patients with Dysphagia

“…Epidermal nevi were encountered in 10% of the study patients. In literature, there are only few reports of epidermal nevi occurring over Anogenital area without documented exact prevalence (17)(18)(19). Epidermal nevi present as linear hyper pigmented verrucous grouped papules and plaques (22).…”

“…These lesions may resolve spontaneously, although treatment is better to prevent further spread of the virus (15). Epidermal nevi are benign cutaneous hamartoma characterized by hyper pigmented verrucous papules and plaques that follow linear arrangement along Blaschko's line over face, trunk, extremities, and rarely over genitalia (17)(18)(19). Epidermal nevi usually appear in the first year of life as flat lesions that get more verrucous with age (20)(21)(22).…”

A Case of “Inflammatory Linear Verrucous Epidermal Nevus” (ILVEN) Treated with CO2 Laser Ablation

“…More rarely, ILVEN has been described on the trunk with typical curvilinear transverse bands, which follow the Blaschko lines, sometimes stopping at the midline [ 8 ]. Different localisations, like genital or mucosal, are extremely rare [ 9 - 11 ].…”

“…At present, there are no effective medical therapies available. Topical corticosteroids, dithranol and retinoids are beneficial in a small percentage of patients [ 9 , 19 , 20 ]. Topical vitamin D analogues, 5-FU and calcineurin inhibitors may be considered as therapeutic options [ 21 , 22 ].…”

Vulvar Inflammatory Linear Verrucous Epidermal Nevus: Remission with Nonsteroidal Antiinflammatory Cream: Case Report

“…Diğer epidermal nevüslerle birliktelik ve tırnak distrofisi görülebilmektedir. 3,7 Bununla birlikte, İLVEN'in çeşitli ekstremite defektleri ile de birliktelik göste-rebileceği bildirilmiştir. 2 İLVEN ayırıcı tanısında başta lineer psöriyazis olmak üzere liken striatus, verrüköz epidermal nevüs, liken simpleks kronikus, lineer liken planus ve lineer Darier hastalığı gibi birçok hastalık yer almaktadır.…”