Perianal Paget's disease: a diagnostic dilemma

Abstract

Perianal Paget's disease (PPD) is part of the spectrum of pagetoid skin lesions occurring outside the region of the nipple/areolar complex that are collectively referred to as extramammary Paget's disease (EMPD). However, unlike Paget's disease of the breast, which in the vast majority of cases is invariably associated with an underlying ductal carcinoma, most cases of EMPD occur as either insitu or invasive adenocarcinomas with visceral malignancy being a rare entity. We present a case of a 50-year-old man who was referred to us with PPD. This case highlights the difficulties associated with making a clinical diagnosis of this condition. Furthermore, investigative workup to exclude possible malignancy with associated metastatic spread as well as the treatment options available are also discussed. In addition, this case highlights the need for a multidisciplinary team approach when dealing with this difficult problem.

Figure 1

Note the red, raised plaque-like lesion extending concentrically outwards from the perianal region.

Figure 2

Note infiltration of the epidermis by medium-sized to large-sized cells with round to ovoid nuclei and pale amphophilic cytoplasm; ×5 magnification (A); ×10 magnification (B) and ×20 magnification (C).

Figure 3

A small area of recurrence measuring approximately 2 cm in diameter within the natal cleft after 18 months postexcision.

Figure 4

Remnant of scar pattern left behind by the gluteal fold flap which was used to fill the defect immediately postexcision.