Clear Cell Papulosis Associated With Multiple Developmental Abnormalities of the Skin

Clear cell papulosis of the skin is a rare condition in children characterized by multiple small hypopigmented macules or flat papules, predominantly distributed in the pubic area and over the abdomen, chest, and axillae, along the milk lines, and by mucin- and cytokeratin 7-positive large clear cells within the epidermis. Associations of clear cell papulosis with other diseases or disorders have been rarely described. We present a case of clear cell papulosis associated with a congenital sacral dermoid cyst, nevus of Ota, presumed accessory breast tissue, thin and lightly pigmented hair, congenital triangular alopecia, and a posterior helical ear pit.