Clear cell papulosis in Hispanic siblings

Clear cell papulosis is a rare condition that has been reported in 14 children, all but 1 of whom are of Asian descent. It was first described in 1987 and is thus named because of the presence of clear cells in the epidermis.

We describe the cases of 3 Hispanic children in the United States with clear cell papulosis. All 3 patients presented with multiple grouped, oval, hypopigmented macules and flat papules in the suprapubic area and trunk. Histopathologic examination revealed characteristic clear cells within the basal layer and Malpighian layer.

Clear cell papulosis is a unique entity with most cases reported in patients of Asian heritage. The characteristic cells have histologic and immunohistochemical similarities to Paget cells and Toker clear cells of the nipple, although the exact relationship to these cells is not clear. The natural history of clear cell papulosis is unknown; thus, continual surveillance for development of extramammary Paget disease is suggested.