Giant inflammatory linear verrucous epidermal nevus: Successfully treated with full thickness excision and skin grafting

Abstract

Inflammatory linear verrucous epidermal nevus (ILVEN) is a pruritic, erythematous scaly epidermal nevus which follows a Blaschko's lines. Lichen striatus, linear Darier disease, linear porokeratosis, linear lichen planus, linear psoriasis, and the verrucous stage of incontinentia pigmenti may all have similar clinical presentations as the linear verrucous epidermal nevus. ILVEN can be distinguished from true nevoid psoriasis by pruritus and lack of response to antipsoriatic treatments. Various therapeutic modalities have been described, but no one therapy has been successful consistently. Though giant ILVEN is a relative contraindication to surgical excision, here we report a case showing effectiveness of full thickness excision and skin grafting for this condition.

Inflammatory Linear Verrucous Epidermal Nevus – Rare Presentation in a Young Female

“…It is usually sporadic; however familial cases have also been reported [1,2] .Although its exact etiology is still debatable, ILVEN is thought to be caused by somatic heterozygous mutations of GJA1, a gene encoding a gap junction protein and also known as connexin 43. The GJA1 gene mutations result in genetic mosaicism and upregulation of interleukin 1, interleukin 6, tumor necrosis factor α, and intercellular adhesion molecules [3][4][5] .…”

“…Clinically, ILVEN can mimic linear Darier disease, linear porokeratosis, linear lichen planus, linear psoriasis, and the verrucous stage of incontinentia pigmenti, other epidermal nevi, nevoid psoriasis, and lichen striatus [1,2] .In contrast to the linear epidermal nevus, lesions are usually erythematous and pruriginous. Spontaneous regression and absence of pruritus distinguish ILVEN from lichen striatus.…”

“…Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare variant of epidermal nevus characterized by psoriasiform epidermal hyperplasia, pruritic, warty lesions, in a linear configuration, following the lines of Blaschko i.e. embryonic lines situated along the planes of ectodermal cleavage [1] . About 75% of these cases present during the first five years of life, most often in the first 6 months [1] .…”

“…embryonic lines situated along the planes of ectodermal cleavage [1] . About 75% of these cases present during the first five years of life, most often in the first 6 months [1] . Although uncommon, cases with adult onset have also been reported.…”

A case of inflammatory linear verrucous epidermal nevus on the upper eyelid

“…Recently, the effectiveness of laser therapy, surgical excision and skin grafting, and trichloroacetic acid peeling for treatment of ILVEN has been reported. [ 4 5 ]…”