Sclerotic Epithelioid Dermatofibroma With Cytokeratin Expression
Zita Álvarez Bobillo1, Agustín Viera Ramírez2, Ángel Fernández Flores3
1Department of Dermatology, Miguel Servet University Hospital, IIS Aragón, Zaragoza, Spain.
The American Journal of Dermatopathology
|April 6, 2026
Summary
This case report details a rare sclerosing epithelioid dermatofibroma with unusual keratin and p40/p63 marker expression. This finding is crucial for accurate diagnosis, differentiating it from malignant tumors.
Area of Science:
- Dermatopathology
- Oncology
- Histology
Background:
- Dermatofibromas are common benign skin lesions with diverse histological presentations.
- The sclerotic and epithelioid variant is exceptionally rare, with limited documented cases.
Purpose of the Study:
- To report the first case of sclerosing epithelioid dermatofibroma with diffuse cytokeratin and p40/p63 expression.
- To highlight the importance of recognizing this atypical immunophenotype to prevent misdiagnosis.
Main Methods:
- Histopathological examination of a 1-cm erythematous plaque on the chest.
- Immunohistochemical analysis of neoplastic cells for various differentiation markers.
Main Results:
- The lesion showed epithelioid cells amidst hyalinized collagen bundles.
- Immunohistochemistry revealed positivity for factor XIIIa, CD68, vimentin, cytokeratins (AE1/AE3, CK8/18), p40, and p63.
- Negative markers for muscle, vascular, melanocytic, and neural differentiation were noted.
Conclusions:
- This is the first reported sclerosing epithelioid dermatofibroma with diffuse cytokeratin and p40/p63 expression.
- The aberrant immunophenotype may mimic malignant neoplasms like sarcomatoid squamous cell carcinoma or epithelioid sarcoma.
- The observed expression pattern likely represents phenotypic plasticity, not true epithelial malignancy.
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