A Case of Inflammatory Linear Verrucous Epidermal Nevus on the Upper Eyelid

Sir,

Inflammatory linear verrucous epidermal nevus (ILVEN) presents as a persistent, linear, intensely pruritic lesion composed of erythematous, slightly verrucous, scaly papules arranged in one or several lines. ILVEN usually appears on a lower extremity in early childhood.[1] Here, we report the first case of ILVEN on the upper eyelid.

A 19-year-old Japanese woman was referred to our department for diagnosis of a pruritic lesion on the right upper eyelid that had been present for 12 years without significant extension. She had been treated with cryotherapy and oral administration of the Chinese traditional medicine at other hospitals, but the treatments were ineffective. In our initial physical examination, erythematous and verrucous papules were seen on the right upper eyelid. The lesions were located at right upper eyelid extending from infero-medial to supero-lateral regions [Figure 1a]. She had no history of internal diseases or other skin diseases. A 3-mm punch biopsy specimen was taken from one of the papules. The histopathology showed hyperkeratosis with foci of parakeratosis, acanthosis, elongation, and thickening of the rete ridges, and moderate perivascular inflammatory infiltrate. A sharply demarcated alteration of orthokeratosis and parakeratosis in the cornified layer was observed [Figure 1b]. The parakeratotic areas were slightly raised and lacked a granular layer with slight spongiosis [Figure 1c]. The orthokeratotic areas showed a preserved granular layer [Figure 1d]. The superficial dermis showed moderate perivascular inflammatory infiltrate of lymphocytes and histiocytes [Figures 1c and d]. There were no intranuclear inclusion bodies in the granular layer. From these results, we diagnosed this case as ILVEN. CO₂ laser therapy as well as conventional topical corticosteroid therapy was recommended for treatment of the ILVEN, but she selected observation of the clinical course.

Figure 1.

Clinical photograph (a) and histopathological study (b-d). (a) Erythematous and verrucous papules were seen on the right upper eyelid. The lesions were located at the right upper eyelid extending from infero-medial to supero-lateral regions. (b) The histopathology showed hyperkeratosis with foci of parakeratosis, acanthosis, elongation, and thickening of the rete ridges, and moderate perivascular inflammatory infiltrate. A sharply demarcated alteration of orthokeratosis and parakeratosis in the cornified layer was observed (hematoxylin-eosin [H and E] staining, original magnification ×40). (c) The parakeratotic areas were slightly raised and lacked a granular layer. Slight spongiosis was present. The superficial dermis showed moderate perivascular inflammatory infiltrate of lymphocytes and histiocytes (H and E, ×100). (d) The orthokeratotic areas showed a preserved granular layer. The superficial dermis showed moderate perivascular inflammatory infiltrate of lymphocytes and histiocytes (H and E, ×100)

ILVEN was first reported by Altman and Mehregan in 1971.[2] They described the clinical and histopathologic characteristics: (1) early age of onset, (2) 4:1 predominance in females, (3) frequent involvement of the left lower extremity, (4) pruritus, (5) distinctive inflammatory and psoriasiform histologic appearance, and (6) persistent lesions showing marked refractoriness to treatment. Lee and Rogers[3] reviewed 23 cases of ILVEN. They concluded that ILVEN might occur in equal sex distribution and the lesions were seen in the lower half of the body in almost all patients. In our case, the eruption was located at an unusual site and showed unusual clinical findings. The differential of linear verurccous papules included epidermal nevus and koebnerized verruca. However, based on the histopathological findings, a diagnosis of ILVEN was made. To the best of our knowledge, this is the first report of ILVEN occurring 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]

Therefore, observation of the clinical course, examination of the medical history, and skin biopsy are crucial in the diagnosis of ILVEN, especially in cases with an atypical presentation. This case illustrates the importance of medical practitioners being aware of the fact that ILVEN may occur on areas of the face.