Inflammatory Linear Verrucous Epidermal Nevus

The lesion on the left forearm of this 20-month-old girl developed shortly after birth and gradually worsened. The toddler scratched the lesion daily. It bled on occasion. The mother vigilantly kept the baby’s nails short and administered an oral antihistamine regularly. There was a family history of chronic eczema.

Consultation with a dermatologist led to a diagnosis of inflammatory linear verrucous epidermal nevus (ILVEN). Altman and Mehregan1 first described this epidermal nevus syndrome in 1971 as a chronic benign cutaneous hamartoma associated with pruritic, erythematous papules that commonly appear during infancy and childhood. The inflamed, psoriasiform papules form linear verrucous plaques and may present along the lines of Blaschko.^1,2 The natural history of these lesions suggests that they generally do not improve over time like most cases of pediatric eczema.³ Although the cause and pathogenesis are unknown, an up-regulation of interleukin (IL)-1, IL-6, tumor necrosis factor-, and intercellular adhesion molecule 1 may play a role.² 

ILVEN has 6 characteristic features^1,4:
•Early age of onset.
•Female predominance (4:1 female-to-male ratio).
•Frequent involvement of the left leg.
•Pruritus.
•Marked refractoriness to therapy.
•Distinctive inflammatory histological appearance: findings include psoriasiform hyperplasia of the epidermis, alternating parakeratosis without a granular layer, and orthokeratosis with a thickened granular layer.⁴

Diagnosis of epidermal nevus syndromes such as ILVEN requires consultation with a dermatologist. ILVEN resembles linear psoriasis and linear lichen simplex chronicus. The marked similarities between ILVEN and linear psoriasis have led researchers to examine whether T lymphocytes and epidermal growth markers can provide a more accurate diagnosis.⁵

Because of the possibility of resistance to treatment and the impact the condition has on quality of life, therapy for ILVEN can pose a challenge. For patients with chronic symptoms, initial treatment usually focuses on topical agents, such as corticosteroids and immunomodulators, and on oral antihistamines. Dermabrasion, cryotherapy, laser therapy, and partial-thickness excision can be considered for lesions that do not respond to conservative therapy. In a review of 4 patients with ILVEN who were treated with full-thickness excision, although the procedures varied, the outcomes were excellent with no evidence of recurrence. ² Studies of the use of biologics in patients with psoriasis have shown encouraging results and suggest a potential benefit for patients with ILVEN.^6,7

This patient’s symptoms are currently controlled with moderate potency corticosteroid creams and oral antihistamines. 

References:

REFERENCES:
1. Altman J, Mehregan AH. Inflammatory linear verrucose epidermal nevus. Arch Dermatol. 1971;104:385-389.
2. Lee BJ, Mancini AJ, Renucci J, et al. Full-thickness surgical excision for the treatment of inflammatory linear verrucous epidermal nevus. Ann Plast Surg. 2001;47:285-292.
3. Rogers M. Epidermal nevi and the epidermal nevus syndromes: a review of 233 cases. Pediatr Dermatol. 1992;26:599-602.
4. Miteva LG, Dourmishev AL, Schwartz RA. Inflammatory linear verrucous epidermal nevus. Cutis. 2001;68:327-330.
5. Sotiriadis D, Patsatsi A, Lazaridou E, et al. Is inflammatory linear verrucous epidermal naevus a form of linear naevoid psoriasis? J Eur Acad Dermatol Venereol. 2006;20:483-484.
6. Weinberg JM, Bottino CJ, Lindholm J, Buchholz R. Biologic therapy for psoriasis: an update on tumor necrosis factor inhibitors infliximab, etanercept, and adalimumab, and the T-cell-targeted therapies efalizumab and alefacept. J Drugs
Dermatol. 2005;4:544-555.
7. Hawrot AC, Metry DW, Theos AJ, Levy ML. Etanercept for psoriasis in the pediatric population: experience in nine patients. Pediatr Dermatol. 2006;23:67-71.