Epithelioid blue naevus of the genital mucosa: report of four cases

Epithelioid blue naevi are an unusual cytological variant of blue naevus th at have been recently described mostly in patients with the Carney complex, although they may also occur in isolation. This variant of blue naevus is composed of melanin-laden polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive depth of dermal infiltration and, in contrast with the usual stromal changes in blue naevi, epithelioid blue naevi exhibit no dermal fibrosis. We describe four cases of epithelioid blue naevus located on the genital mucosa in four patients with no evidence of the Carney complex. Three male patients showe d an epithelioid blue naevus on the mucosa of the glans penis and a female patient had a lesion of the right labium minoris. Histopathologically, the lesions consisted of entirely intradermal melanocytic naevi composed mostly of heavily pigmented epithelioid melanocytes involving the dermis of the g enital mucosa. Immunohistochemically, in all cases, epithelioid melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A and MiTF ant ibodies.