| Non-organic epidermal nevus (NEN), also called verrucous nevus, are benign skin hamartomas that arise from the overproduction of keratinocytes. NEN is characterized by localized or diffuse, closely set, skin-colored,brown,or gray-brown verrucous papules, which may coalesce to form well-demarcated papillomatous plaques. Linear configurations are common on the limbs as is distribution in Blaschko’s lines. Based on clinical manifestations and the scope of lesions. NEN can be divided into three clinical variants: localized, systematized, and inflammatory linear verrucous epidermal nevus (ILVEN). Sporadical NEN are common, but familial NEN are pretty rare. We report here two cases with family history and review the literature. A22-year-old man presented with erythematous, linear, verrucous and keratotic lesions following Blaschko’s line since early infancy on the buttock and perianal areas, the right upper and lower extremities, as well as submaxilla. The lesions were pruritus all the time. No abnomality was detected on general physical and systemic examinations.Laboratory examinations including complete blood count, rutine urine analysis, serum electrolytes, liver and kidney function tests were all within normal range. Histological examination showed hyperparakeratosis and acanthosis of the epidermis, the upper dermis showed a mild perivascular lymphohistocytic infiltrate with dilated blood vessels. His nephew, a2-year-old boy,had similar clinical and histopathological features. The third case in this family, was the first patient’s mother, who had a localized clustered keratotic papule since an early age. From her clinical characteristics and family history, the primary diagnosis was localized NEN. Overall, NEN occurred in5members in three generations (mother, uncle, cousins, nephew) of a family that had no consanguineous marriage. In this composition, we reviewed the epidemiology, molecule pathogenesis, clinical classification, pathology, differential diagnosis and treatment. |
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