A profound case of linear epidermal nevus in a patient with epidermal nevus syndrome

Abstract

Background: Epidermal nevus syndrome is a multi-system disease with a wide spectrum of clinical presentation. Numerous specialists may be required to address its extra cutaneous manifestations.Main observations: We report a severe case of epidermal nevus syndrome involving the oral cavity, pharynx, and central nervous system in addition to disfiguring skin lesions.Conclusions: Dermatologists are in a unique position to first render the diagnosis of epidermal nevus syndrome for young patients and ensure appropriate follow-up. (J Dermatol Case Rep. 2011; 5(2):

Linear Epidermal Nevus: A Case Report With Oral and Facial Manifestations

“…6,7,17 Neurologic involvement has been reported in more than 50% of ENS cases, with rates of ocular involvement ranging from 35 to 70% and with skeletal anomalies in 67% of cases. 5,7,8 Further workup is generally not warranted in children with isolated epidermal nevi and normal physical examination findings. 8 Oral LEN very closely resembles other benign diffuse papillary lesions including inflammatory papillary hyperplasia and spongiotic gingival hyperplasia.…”

“…27 Malodorous plaques are treated with benzoyl peroxide formulations. 5 These treatments at times are irritating, and therefore patient compliance is the primary concern. This is especially true in the pediatric patient population.…”

“…LEN represents a form of mosaicism, whereby an individual has cells with different genotypes derived from the same zygote. 5 LEN when present in conjunction with other systemic manifestations is known as epidermal nevus syndrome (ENS). First described in 1968 by Solomon, ENS is now classified into 9 distinct forms, based on specific histopathologic and genetic features.…”

“…It is often associated with Proteus syndrome, type 2 segmental Cowden disease, and congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD syndrome). [5][6][7][8] In 1960 Brown and Gorlin 9 were the first authors to describe the oral manifestations of LEN. Since then, only 15 cases have been reported.…”

Linear Epidermal Nevus of the Oral Cavity: A Rare Diagnosis

“…Bad smell plates can be managed with benzoyl peroxide solutions [1]. For minor obstructive lesions, surgical remove may be considered.…”