Primary Extramammary Paget's Disease Combined with Bowen's Disease in Vulva

Abstract

Extramammary Paget's disease (EMPD) is a uncommon neoplastic condition of apocrine gland-bearing skin and its occurrence in combination with Bowen's disease is very rare. The most common site of involvement is the vulva, although perineal, perianal, scrotal and penile skin may also be affected. EMPD is usually not combined with Bowen's disease. We report an interesting case of EMPD combined with Bowen's disease, which was confirmed by immunohistochemical stain.

Keywords: Bowen's disease; Paget disease; Vulva; extramammary.

Fig. 1

Clinical appearance and histological findings of the biopsy specimen. Eczematoid nodules on the vulva was observed (A). Typical full thickness atypia (B), cellular atypia combined with basal Paget cells (C), and pagetoid distribution of Paget cells were observed (D) (H&E: ×100 and ×400).

Fig. 2

Immunohistochemistry examinations of the biopsy specimen. (A) In the middle lesion of tumor, immunohistochemical staining of cytokeratin 20 (CK20), CK7, carcinoembryonic antigen (CEA), diastase-resistant periodic acid-Schiff (PAS), mucin core protein (MUC) 5-AC, and MUC2. CK20, CK7, and diastase-resistant PAS were stained positively in Pagetoid cells, but CEA was stained with faint. (B) In the left lesion of tumor, MUC 5-AC was clearly observed in Paget cells, but not by MUC-2. Original magnification ×100.