Ann Dermatol.  2011 Oct;23(Suppl 2):S226-S230. 10.5021/ad.2011.23.S2.S226.

Acantholytic Anaplastic Extramammary Paget's Disease: A Case Report and Review of the Literature

• Oh YJ
• Lew BL
• Sim WY

• KMID: 2156796
• DOI: http://doi.org/10.5021/ad.2011.23.S2.S226

Abstract

Extramammary Paget's disease (EMPD) is an uncommon intraepithelial neoplasm that most commonly arises on the vulva and perianal region. Very few cases of EMPD revealing a histological Bowenoid appearance have been reported. This study describes scrotal EMPD presenting with histological features of Bowen's disease in a 79-year-old man. He presented with a 5-year history of a pruritic erythematous plaque and a verrucous papule on the scrotum. The verrucous papule histopathologically showed Bowenoid features, and the erythematous plaque demonstrated acantholytic EMPD. Immunohistochemical findings revealed strong expression for carcinoembryonic antigen, Cam 5.2, epithelial membrane antigen, cytokeratin (CK) 7, and pancytokeratin (AE1/AE3) in both areas, but negative CK20 staining, supporting the overall diagnosis of primary acantholytic anaplastic EMPD. This is the first reported case of acantholytic anaplastic EMPD in the Korean literature.

Keyword

Acantholytic; Anaplastic; Bowenoid; Extramammary Paget disease; Immunohistochemistry

MeSH Terms

Aged
Bowen's Disease
Carcinoembryonic Antigen
Carcinoma in Situ
Humans
Immunohistochemistry
Keratins
Mucin-1
Paget Disease, Extramammary
Scrotum
Vulva
Carcinoembryonic Antigen
Keratins
Mucin-1

Figure

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  Fig. 1 (A) A solitary verrucous surfaced, sessile papule was observed on the scrotum. (B) A localized, well defined, erythematous plaque with yellow scales was observed on the scrotum.

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  Fig. 2 Specimen obtained from the papular lesion. (A) The epidermis revealed marked acanthosis with papillomatosis (H&E, ×100). (B) Full thickness cellular atypia with mitotic figures was observed (H&E, ×200). (C) Atypical pleomorphic cells found in the spinous zone, a few of which had abundant, pale cytoplasm, were separated by clefts from the surrounding cells (H&E, ×200).

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  Fig. 3 Specimen obtained from the plaque lesion. (A) The epidermis showed prominent acantholysis with nests of Paget cells in the cavity (H&E, ×100). (B) The Paget cells had pale-staining cytoplasm with vesicular nuclei containing occasional prominent nucleoli and were separated from the underlying dermis by flattened basal cells (H&E, ×200).

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  Fig. 4 Immunohistochemical stains of the papular lesion. (A) Mucicarmine, (B) Carcinoembryonic antigen, (C) Cytokeratin 7 (magnification, ×100).

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  Fig. 5 Immunohistochemical stains of the plaque lesion. (A) Carcinoembryonic antigen, (B) Cytokeratin 7, (C) CK20, (D) Gross cystic disease fluid protein (magnification, ×100).

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