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| Fig. 47.6 Primary extramammary Paget’s disease. A, Typical extramammary Paget’s disease arising in the groin. B, Typical large, pale, atypical epithelial cells that demonstrate a scattered growth pattern, both as single cells and as small aggregates throughout the epidermis (hematoxylin and eosin [H&E], 3400). |
EMPD is a malignant epithelial tumor most often found in the genital region or perineum, which also uncommonly occurs on the axillae, eyelid, ear, anterior chest, and accessory nipples. Nearly all cases of EMPD occur in areas that contain apocrine glands, and, although the histogenesis is not proven, current evidence suggests that they arise from Toker cells. The function of Toker cells is not entirely understood, but based on their distribution, they are likely involved in apocrine gland and breast development. Clinically, EMPD presents as a pruritic, erythematous patch or plaque with oozing and crusting (Fig. 47-6A), which can be confused with dermatitis. It occurs in older adults.
Wilman JH, Golitz LE, Fitzpatrick JE: Vulvar clear cells of Toker: precursors of extramammary Paget’s disease,
Am J Dermatopathol 27:185–188, 2005.
![Primary extramammary Paget’s disease. A, Typical extramammary Paget’s disease arising in the groin. B, Typical large, pale, atypical epithelial cells that demonstrate a scattered growth pattern, both as single cells and as small aggregates throughout the epidermis (hematoxylin and eosin [H&E], 3400).](images/dermatology_secrets/f47.6_large.jpg)
