Vulval and perianal inflammatory linear verrucous epidermal naevus

Abstract

Inflammatory linear verrucous epidermal naevus (ILVEN) is a rare form of epidermal naevus. It occurs as a linear dermatitic or psoriasiform plaque, with onset usually in the first 5 years of life. Lesions are characteristically intensely itchy. We present a case of ILVEN occurring on the vulva and perianal region of a 6-year-old girl. The lesion was initially thought to be an area of lichenified dermatitis; however, treatment with even super-potent topical corticosteroids did not significantly improve the inflammation. A biopsy was performed and histopathological examination showed characteristic features. ILVEN is frequently refractory to topical treatment and surgical excision of lesions may be an option for relief of symptoms. ILVEN occasionally presents in the inguinogenital region and in this area may, like many vulval naevi, be misdiagnosed as vulvitis, psoriasis, genital warts or sexual abuse.

Carbon dioxide laser: first-line therapy in vulvar inflammatory linear verrucous epidermal nevus

“…It presents as single or multiple erythematous, verrucous and characteristically pruritic plaques that follow Blaschko's lines and clearly stop at the median line (1). The lower extremities are typically affected (1), but a mucosal involvement has also been described (2).…”

Inflammatory linear verrucous epidermal nevus in perineum and vulva: A report of two rare cases

“…ILVEN most commonly occurs over the lower limb with a predilection for the left buttock. [12] Although literature search showed that inguino-genital presentation is very rare, both our cases presented with same. There have been further case reports of a 21-year-old male who had linear verrucous plaque over left side of body along with the involvement of penis and scrotum[3] and another male presenting with linear epidermolytic verrucous epidermal nevus over scrotum.…”

“…Clinically it presents as pruritic, verrucous, linear, plaque with an early age of onset and is resistant to treatment. [1] In half of the cases the onset is in the first 6 months of age[2] and almost all are present by the age of 4 years. [1] The ages of onset in our cases were at 5 years (Case 1) and at birth (Case 2).…”

“…[1] In half of the cases the onset is in the first 6 months of age[2] and almost all are present by the age of 4 years. [1] The ages of onset in our cases were at 5 years (Case 1) and at birth (Case 2). ILVEN most commonly occurs over the lower limb with a predilection for the left buttock.…”

Inflammatory linear verrucous epidermal nevus with psoriasiform histology

“…The patient had satisfactory response to surgical excision. We report this interesting case as only few cases[5678910] are reported in the literature and also because, despite of having histopathological features of psoriasis, there was no response to antipsoriatic treatment but to surgical excision.…”