Epidermal naevi - an overview

LAST UPDATED: Sept 18, 2022

Introduction

The word naevus indicates a non-neoplastic skin or mucosal lesion comprising an abnormal mixture of a tissue’s normal components, usually presenting at birth or at a young age - an epidermal naevus is a term used for naevi predominantly composed of keratinocytes.

Although there are many different forms of epidermal naevi this section focuses on verrucous epidermal naevi (both epidermolytic and non-epidermolytic types) and inflamed verrucous epidermal naevi (ILVEN).

Whilst the majority of epidermal naevi only affect the skin and are of limited significance, it is important to differentiate between epidermolytic and non-epidermolytic verrucous epidermal naevi as some patients with the former have an increased risk of having children born with epidermolytic hyperkeratosis (syn. bullous ichthyosiform erythroderma), a rare but serious condition. With regards to non-epidermolytic verrucous epidermal naevi, rarely, such lesions are associated with a wide range of non-cutaneous abnormalities, especially the CNS, eyes and skeleton, which together are known as the epidermal naevus syndrome.

Refer to the related chapters for more information.

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