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

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.

What was known?

Inflammatory linear verrucous epidermal nevus (ILVEN) is a pruritic, erythematous scaly epidermal nevus which follows the Blaschko’s lines. Various treatment modalities including topical agents, dermabrasion, cryotherapy, laser therapy, and partial-thickness excision has been tried. Unfortunately, no one therapy has been successful consistently. Giant ILVEN is a relative contraindication to surgical excision and recurrence may be a problem.

Introduction

Inflammatory linear verrucous epidermal nevus (ILVEN) is an epidermal nevus presenting with pruritus, erythema, and scaling that occur as linear bands along the lines of Blaschko. Patients having giant ILVEN seek treatment due to cosmetic disfigurement. Reported therapeutic approaches include topical agents, dermabrasion, cryotherapy, laser therapy, and partial-thickness excision. Unfortunately, no one therapy has been successful consistently.[1] Giant ILVEN is a relative contraindication to surgical excision and recurrence may be a problem. We report the effectiveness of full thickness excision and skin grafting for the definitive treatment of a giant ILVEN.

Case Report

A 17 year old female presented with a linear pruritic lesion extending from lateral border of right foot to right gluteal region [Figure 1]. The parents noticed that lesion in the second month of life, which continued to grow up to puberty. Since last 2 years it was stable. Pruritus was intense leading to recurrent secondary infection. There were no other systemic abnormalities. None of the family members had similar type of lesion. She was prescribed oral, topical and intralesional steroid, isotretinoin and methotrexate in past, without any improvement. The routine investigations including tests for HIV were within normal limits.

Figure 1

Linear pruritic lesion extending from lateral border of right foot to right gluteal region at presentation

Histopathology showed hyperkeratosis with alternate parakeratosis and orthokeratosis and hypergranulosis beneath the orthokeratotic area. There was mild spongiosis with psoriasiform epidermal hyperplasia and mild to moderate perivascular lymphocytic infiltrate in superficial dermis [Figure 2].

Figure 2

Photomicrograph showing hyperkeratosis with alternate parakeratosis and orthokeratosis and hypergranulosis beneath the orthokeratotic area (H and E, ×10)

A diagnosis of ILVEN was made on the basis of intense pruritus, histopathological findings and lack of response to antipsoriatic treatments. Considering the marriageable age, posteriorly placed lesion and malignant potential, she had underwent full thickness excision followed by split thickness skin grafting. The postoperative period was uneventful. She was followed up for one year without any recurrence or significant scarring [Figure 3]. The cosmetic result was acceptable and the patient was satisfied.

Figure 3

Follow up photograph of the patient after 3 months

Discussion

ILVEN is probably due to unidentified lethal dominant mutation, rescued by mosaicism.[2] It is usually sporadic, but there have been reports of familial cases.[34] Absent involucrin expression in the parakeratotic epidermis has been reported, a finding that appears to distinguish ILVEN from psoriasis.[5] About 75% appear during the first five years of life, most often in the first six months, although later onset has been recorded. ILVEN can be of any length, occasionally extending the whole length of a limb. Usually it is unilateral but bilateral and widespread lesions have been described. Although generally persistent and resistant to treatment, some lesions have been reported to resolve spontaneously.[6] 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. Lichen striatus is distinguished by its rapid development, generally after the first year of life, by its relative lack of pruritus, by its more lichenoid clinical and histological features, and, eventually, by its spontaneous involution. The distinction between CHILD syndrome and ILVEN is uncertain.

Potent topical corticosteroids applied under occlusion, or intralesional steroid injections, provide little more than temporary symptomatic relief.[7] There are few reports of effective systemic treatments with acitretin and etanercept.[89] Laser ablation, electrofulguration, cryotherapy, and medium to full-depth chemical peels are other alternatives for smaller lesions. Earlier reports say, surgical excision tends to be followed by rapid recurrence unless a generous depth of underlying dermis is removed.[110] Lee et al. reported 4 patients with extensive ILVEN treated successfully with full-thickness surgical excision.

Our patient had an extensive lesion extending from right foot to gluteal region and didn’t respond to any form of medical treatment. The cosmetic results after full thickness excision and skin grafting in our case was excellent without any recurrence within one year of follow up. The patient was satisfied with the cosmetic outcome.

In conclusion, full thickness excision and skin grafting is a good therapeutic alternative for extensive ILVENs and those not responding to medical treatments.

What is new?

Our patient had an extensive lesion extending from right foot to gluteal region and didn’t respond to any form of medical treatment. The cosmetic results after full thickness excision and skin grafting in our case was excellent without any recurrence within one year of follow up. The patient was satisfied with the cosmetic outcome.