Evidence from mucin core protein expression that some Paget's disease on areola can be of extramammary-like histogenesis and part of multisite disease

Abstract

Triple extramammary Paget's disease, which consists ordinarily of bilateral axillary and genital lesions, is uncommon. Triple extramammary Paget's disease involving other sites has never been reported, although solitary extramammary Paget's disease can occur at various sites around the body. Erythematous plaques on the areola, axilla and genitalia of a 91-year-old man were surgically removed under the clinical diagnosis of multiple extramammary Paget's disease. Histology revealed that all three lesions consisted of intraepidermal nests of Paget cells and other isolated Paget cells scattered in the epidermis. Although adnexal invasion was observed in the genital lesion, neither intraductal invasion nor underlying breast carcinoma was detected in the areolar lesion. Immunohistochemically, the Paget cells in all lesions expressed simple epithelial cytokeratins (CK8, 18 and 19), mucin (MUC)1 and MUC5AC, but neither CK20 nor MUC2. From the histological findings, the present case was interpreted as triple extramammary Paget's disease rather than synchronous mammary and extramammary Paget's disease. Furthermore, the mucin core protein expression pattern, which was identical to that observed in extramammary Paget's disease, supported the above interpretation.

Cutaneous metastases from triple primary extramammary Paget’s disease

“…Triple EMPD, first described by Kawatsu and Miki in 1971, is uncommon [3]. To date, there have been no more than 30 cases and most patients are Japanese [4]. This is the first case observed in a Chinese patient.…”

Role of mucins in the skin during benign and malignant conditions

“…This was seen in both the typical and atypical EMPD cases but in none of the control skin tissues, suggesting that Paget’ cells in EMPD possibly originate from these cells rather than Toker cells (MUC1-, MUC5AC-) as suggested by other studies [89]. In the case of triple-extramammary Paget’s disease (lesions on the areola and in the axilla and genitalia) in a 91-year old man, the Paget’s cells expressed MUC1 and MUC5AC but not MUC2 [90]. In the same study, expression of MUC1 but not MUC5AC was observed, albeit focally in two cases of MPD stained simultaneously.…”

Quadruple Extra-mammary Paget's disease

“…Since the first report by Kawatsu & Miki (1) in 1971. approximately 60 Japanese cases of triple extra-mammary Paget's disease have been reported, mostly in Ihe Japanese literature, and only 4 cases in the English literature (2)(3)(4). genital, perianal and a.xillary lesions (1).…”