Epithelioid Blue Nevus with No Evidence of Carney Complex

Epithelioid blue nevus (EBN) is a rare variant of blue nevus characterized by large polygonal pigmented melanocytes. As EBN was first described in patients with Carney complex, familial lentiginosis, and low-grade multi-organ neoplasia syndrome, sporadic cases without such clinical features have been reported and debated as having considerable histopathological overlap with animal-type melanoma. A 34-year-old male presented with a well-defined, 2.0-x 1.8-cm-sized, dome-shaped, grayish mass on the anterior aspect of the left ankle. A complete surgical resection of the lesion was performed. Histopathological findings revealed a well-circumscribed round tumor in the reticular dermis. Heavily pigmented epithelioid melanocytes were admixed with the collagen bundles. The lesion was diagnosed as EBN. A follow-up period of 9 months showed no evidence of recurrence or metastasis. Here, we report a sporadic case of EBN, which is rare in the Korean population.