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Clear cell papulosis
Rachel Farley-Loftus1, Nicole M Bossenbroek, Karla Rosenman
1Department of Dermatology, New York University, USA.
Insights
Clear cell papulosis is a rare pediatric skin condition presenting as hypopigmented papules along milk lines. Histopathology reveals diagnostic clear cells in the epidermis, linking it to Toker cell and Paget disease origins.
Area of Science:
- Dermatology
- Pediatric Dermatology
- Histopathology
Background:
- Clear cell papulosis is a rare genodermatosis.
- Its pathogenesis and relationship to other clear cell lesions remain under investigation.
Observation:
- A 2-year-old boy presented with multiple hypopigmented, flat-topped papules on the abdomen and pubic region, following milk lines.
- Lesions first appeared at three months and progressively increased in number.
Findings:
- Histopathologic examination revealed large clear cells in the lower epidermis.
- These cells stained positive with periodic acid-Schiff (PAS).
- The findings confirmed the diagnosis of clear cell papulosis.
Implications:
- This case highlights the characteristic presentation of clear cell papulosis in children.
- The histopathologic findings suggest a potential link between clear cell papulosis, clear cells of Toker, and extramammary Paget disease.
- Further research into immunohistochemical markers may elucidate the precise cellular origin and relationship between these conditions.
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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