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Vulvar and Perianal Condyloma Superimposed Inflammatory Linear Verrucous Epidermal Nevus: A Case Report and Review of the Literature

DOI: 10.1155/2013/261574

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Abstract:

Inflammatory linear verrucous epidermal nevus (ILVEN) is a benign cutaneous hamartoma characterized by intensely erythematous, pruritic, and inflammatory papules that occur as linear bands along the lines of Blaschko. There is a considerable clinical and histological resemblance between ILVEN and linear psoriasis, lichen striatus, linear lichen planus, and invasion of epidermal nevus by psoriasis. The pathogenesis of ILVEN is unknown. It is regarded as a genetic dyskeratotic disease reflecting genetic mosaicism. Here, a case of vulvar and perianal condyloma superimposed ILVEN is presented. 1. Introduction Inflammatory linear verrucous epidermal nevus (ILVEN) is a relatively uncommon benign cutaneous hamartoma described by Altman and Mehregan in 1971 [1]. It is characterized by intensely pruritic, erythematous, inflammatory papules coalescing into well-demarcated verrucous plaques in a linear distribution. Patients seek help for its symptoms and cosmetic appearance [2]. Medical management is extremely variable but unfortunately does not result in complete recovery. On the other hand, surgical excision of lesions is not preferable due to extensive scarring and relapse of disease [3]. Here,a case of vulvar and perianal condyloma superimposed ILVEN is presented. 2. Case Presentation A 21-year-old virgo patient, has presented with a huge amount of vulvar and perianal condylomas. Surgical excision was planned. But in physical examination of patient, erythematous scaly plaques were seen unilaterally on the right side of body. Detailed medical history revealed that, erythematous lesions, of linear or grouped distribution, involving only right side of body; upper trunk, axilla, and lower extremity unilaterally had existed since a few years after birth. Lesions were extremely pruriginous and aggravated with heat. There were no pathological antecedents or previous family history of the disease. Dermatological examination revealed papular lesions and erythematous plaques with areas of scaling and crusts, linearly distributed at the right side of the body. (Figures 1(a), 1(b), and 1(c)). Disease was diagnosed as ILVEN by histopathological examination. Besides, laboratory tests including C3, C4, immunoglobulin (Ig)A, IGM, IGE, IGG anti-HAV IGM, and anti-HAV IGG were performed. Laboratory investigations showed mild anemia (hemoglobin 10.5?g/dL), an elevated erythrocyte sedimentation rate (45?mm/h), and an elevated C-reactive protein level (36.3?mg/L, normal < 8.0?mg/L). Liver enzymes, renal function test results, and immunoglobulin levels were normal. Results of tests

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