Clinicopathologic features of an infant with generalized congenital epithelioid blue nevi

Since epithelioid blue nevus (EBN) was 1st described in patients with Carney complex, 49 sporadic EBN cases, including 4 congenital EBN, have been reported. We present a 2-month-old healthy female with more than 1000 congenital EBN on the entire body. Skin biopsy revealed many nevus nests located in the upper dermis and a few nests extended around the sweat ducts and hair follicles in the middle and lower dermis. The heavily pigmented melanocytes were substantially epithelioid and occasionally spindle cells, admixed with melanophages. Immunohistochemistry revealed strong staining for S-100 and HMB-45 but weak or moderate staining for Melan-A in dermal melanocytes after melanin bleaching with potassium permanganate and oxalic acid prior to incubation with the primary antibody. A diagnosis of congenital EBN was made based on clinicopathologic and immunopathologic findings.