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Ectopic extramammary Paget's disease arising on the cheek
Suneel Chilukuri1, Robert Page, Jon A Reed
1Department of Dermatology, Baylor College of Medicine, Houston, Texas 77030, USA.
Summary
This study reports the first case of extramammary Paget's disease (EMPD) occurring on the face. This rare skin cancer typically affects apocrine gland areas, making this facial presentation unusual.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Extramammary Paget's disease (EMPD) is a rare malignancy of apocrine gland-bearing skin.
- Typically presents as a chronic, unilateral, erythematous or whitish-gray plaque.
- Rarely occurs outside of the anogenital or axillary regions.
Observation:
- A 67-year-old male presented with a facial lesion.
- Histopathological examination of the excisional specimen was performed.
- Immunohistochemistry was utilized to characterize the atypical cells.
Findings:
- Histology showed hyperkeratosis, parakeratosis, acanthosis, and atypical epithelioid cells.
- Atypical cells infiltrated the epidermis and cutaneous adnexae.
- Positive staining for cytokeratins (AE1/AE), epithelial membrane antigen, and carcinoembryonic antigen.
- No apocrine glands were identified in the affected facial area.
Implications:
- This case represents the first documented instance of truly ectopic extramammary Paget's disease on the face.
- Highlights the importance of considering EMPD in facial lesions, even in the absence of typical locations.
- Expands the known clinical spectrum and anatomical distribution of extramammary Paget's disease.