Extramammary Paget disease
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Extramammary Paget disease, abbreviated EMPD, is a skin disease. As the name suggests, there is also a Paget disease of the breast.
| Extramammary Paget disease | |
|---|---|
| Diagnosis in short | |
 Extramammary Paget's disease. H&E stain. | |
|
| |
| LM | large epithelioid cells - nested or single - in the epidermis, clear/pale cytoplasm (occasionally eosinophilic), large nucleoli |
| LM DDx | benign Toker cell hyperplasia, malignant melanoma, Bowen's disease, apocrine carcinoma of the skin |
| IHC | CK7 +ve, CEA +ve, S-100 -ve, CK5/6 -ve, HER2 +ve |
| Gross | erythema, +/-weeping, +/-crusted |
| Site | vulva |
|
| |
| Symptoms | pruritis (itchy) |
| Prognosis | typically benign - usually not associated with an underlying malignancy (unlike Paget's disease of the breast) |
| Clin. DDx | contact dermatitis, lichen sclerosus |
There is also a Paget disease of the bone - just to make things confusing. This is dealt with in the bone article and has nothing (from a pathologic perspective) to do with the Paget disease discussed in this article
General
- Usually not associated with malignancy, unlike the Paget disease of the breast - important difference.
- Classically seen in the vulva.
- May afflict penis.[1]
Clinical:
- Pruritis.
Gross
- Erythema.
Microscopic
Features:
- Epitheliod morphology (round/ovoid).
- Cells nested or single.
- Clear/pale cytoplasm key feature - may also be eosinophilic.
- Large nucleoli.
Images
DDx
- Benign Toker cell hyperplasia.
- Malignant melanoma.
- Bowen disease.
- Apocrine carcinoma of the skin.[2]
- Vulvar squamous cell carcinoma.
Stains
- Mucicarmine stain +ve.
IHC
Panel:
- CEA +ve (-ve in Bowen's disease, -ve in Toker cells).
- CK7 +ve.
- Toker cells CK7 +ve.[3]
- S100 -ve, HMB-45 -ve (both typically +ve in melanoma).
Additional:
- HER2/neu - usually +ve.
- CK5/6 -ve.[4]
- Usu. +ve in squamous cell carcinoma.
- CAM 5.2 +ve.
See also
References
- ↑ Ekwueme, KC.; Zakhour, HD.; Parr, NJ. (2009). "Extramammary Paget's disease of the penis: a case report and review of the literature.". J Med Case Reports 3: 4. doi:10.1186/1752-1947-3-4. PMID 19126202.
- ↑ URL: http://derm101.com/searchResults.aspx?searchStr=apocrine+carcinoma&rootTerm=apocrine+carcinoma&searchType=2&rootID=12687. Accessed on: 9 September 2011.
- ↑ Nofech-Mozes, S.; Hanna, W.. "Toker cells revisited.". Breast J 15 (4): 394-8. doi:10.1111/j.1524-4741.2009.00743.x. PMID 19601945.
- ↑ RS. May 2010.