Clear-Cell Papulosis: A Rare Entity That May Be Misconstrued Pathologically as Normal Skin


Address correspondence to Latanya T. Benjamin, M.D., Stanford University Department of Dermatology, 269 Campus Drive, Room 2115a, MC 5168, Sanford, CA 94305, or e-mail:


Abstract:  We describe a case of multiple, discrete, hypopigmented macules in the suprapubic and axillary region in a healthy 3-year-old girl. The lesions first appeared at approximately 9 months of age and increased in number over time. Initial histopathologic examination by an outside dermatopathologist at 1 year of age was reported as showing nonspecific histologic changes. A repeat biopsy at 3 years of age showed large intraepidermal clear cells that expressed CKAE1/CAM5.2, CK7, and BRST2. These findings are diagnostic for clear-cell papulosis, a rare condition that primarily affects children. Without great clinical and pathologic suspicion, this is a diagnosis that can often be overlooked because the histologic findings are virtually identical to those of normal skin.