Article

Clear cell papulosis.

Department of Dermatology, New York University, USA.
Dermatology online journal 02/2008; 14(10):19.
Source: PubMed

ABSTRACT A 2-year-old boy presented with multiple, hypopigmented, flat-topped papules in the pubic region and on the abdomen in a distribution pattern that followed the milk lines. The lesions had first appeared at age three months and increased in number over time. Histopathologic examination showed large clear cells within the lower epidermis, which stained positively with periodic acid-Schiff. These findings were diagnostic of clear cell papulosis, a rare condition that primarily affects young children. We review the histopathologic and immunohistochemical findings that link clear cell papulosis to clear cells of Toker and extramammary Paget disease.

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    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.
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