Inflammatory linear verrucous epidermal nevus
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https://doi.org/10.5070/D338j3g79cMain Content
Inflammatory linear verrucous epidermal nevus
Meredith K Kosann MD
Dermatology Online Journal 9(4): 15
From the Ronald O. Perelman Department of Dermatology, New York University
Abstract
Inflammatory verrucous epidermal nevus presents in early childhood as a pruritic, erythematous, linear plaque. The 23-year-old man described in this report demonstrates the adult onset of the condition. Clinical findings, differential diagnosis, disease associations, and treatment options are discussed.
Clincal summary
History.—A 23-year-old man presented to the Department of Veterans Affairs New York Harbor Health Care System Dermatology Clinic with a 4-and-a-half-year history a pruritic eruption under his right arm. He was treated with antifungal creams, topical glucocorticoids, and intralesional glucocorticoids without improvement. The patient denied a family history of this problem.
Physical examination.—There was an erythematous patch, approximately 5 cm in length, with a linear component in the inferior aspect of the right axilla.
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Laboratory data.—The white cell count was 6.5 x 109/L, hemoglobin 14 g/dL, and the platelet count 183 x 109/L. Basic metabolic profile, hepatic function tests, and thyroid-stimulating hormone levels were normal.
Histopathology.—There is a superficial perivascular infiltrate of lymphocytes and neutrophils that focally extends to the overlying irregularly hyperplastic epidermis, where there is slight spongiosis with a compact ortho- and parakeratotic cornified layer. The area of parakeratosis is slightly raised and lacks the underlying granular layer.
Diagnosis.—Inflammatory linear verrucous epidermal nevus.
Comment
Inflammatory linear verrucous epidermal nevus (ILVEN) presents as erythematous and verrucous papules, which are arranged in a linear array. The lesions are characteristically pruritic. ILVEN is usually unilateral and is frequently located on an extremity. Children are most commonly affected (girls more often than boys), often prior to the age 2. However, adult onset of the condition has been reported [1]. Most cases are sporadic, although familial cases have been described [2].
Clinically, ILVEN must be distinguished from other epidermal nevi, linear psoriasis, and lichen striatus. Unlike linear epidermal nevus, ILVEN is characteristically erythematous and pruritic. Unlike linear psoriasis, ILVEN is often difficult to treat and presents in early childhood. ILVEN is pruritic and does not spontaneously regress, which often occurs in lichen striatus. ILVEN has been reported to be associated with autoimmune thyroiditis [3], arthritis [4], and lichen amyloidosis [5].
Typically, ILVEN is resistant to treatment. Therapeutic options include topical and intralesional glucocorticoids, excision, cryotherapy, and laser therapy. The CO2 laser, at a low fluence, has been used to treat one patient with disfiguring ILVEN of the vulvar region [6]. Treatment with the flashlamp pumped pulsed-dye laser resulted in decreased pruritus as well as partial resolution of the lesion in one patient [7].
References
1. Kawaguchi H, et al. Adult onset of inflammatory linear verrucous epidermal nevus. J Dermatol 1999;26:599.2. Goldman K, Don PD. Adult onset of inflammatory linear verrucous epidermal nevus in a mother and her daughter. Dermatology 1994;189:170.
3. Dereure O, et al. Inflammatory linear verrucous epidermal naevus with auto-immune thyroiditis: Coexistence of two auto-immune epithelial inflammations? Acta Derm Venereol (Stockh) 1994;74:208.
4. Al-Enezi S, et al. Inflammatory linear verrucous epidermal nevus and arthritis: a new association. J Pediatr 2001;138:602.
5. Zhuang L, Wenyuan Z. Inflammatory linear verrucous epidermal nevus coexisting with lichen amyloidosis. J Dermatol 1996;23:415.
6. Molin L, Sarhammar G. Perivulvar inflammatory linear verrucous epidermal nevus (ILVEN) treated with CO2 laser. J Cut Las Ther 1999;1:53.
7. Alster TS. Inflammatory linear verrucous epidermal nevus: successful treatment with the 585 nm flashlamp-pulsed dye laser. J Am Acad Dermatol 1994;31:513.
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