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Review
. 2003 Winter;11(4):205-8.
doi: 10.1177/229255030301100406.

Extramammary Paget's disease of the perineum: Avoiding pitfalls in diagnosis and management

Rajiv Y Chandawarkar  1 , Daniel Ricchuiti, Ibrahim Amjad, Robert E Marsico Jr, Mark D Wells
Affiliations
Review

Extramammary Paget's disease of the perineum: Avoiding pitfalls in diagnosis and management

Rajiv Y Chandawarkar et al. Can J Plast Surg. 2003 Winter.

Abstract
in English, French

Extramammary Paget's disease (EMPD) is a rare entity, especially in the perinoscrotal region, and typically presents in elderly white patients as a pruritic white or red patch in the area of distribution of apocrine glands. Typically, it affects a single site. Since its manifestations are insidious and easily misdiagnosed, the appropriate management is delayed. Management of this problem is complex and effective treatment can not only lower recurrence rates but also provide an optimal reconstructive result. The present report describes three patients with scrotal EMPD. Based on literature search, the etiopathology, diagnosis and management of these lesions is discussed. Reconstructive options, with special emphasis on scrotal lesions, are also discussed.

La maladie de Paget extramammaire (MPEM) est une maladie rare qui se manifeste surtout dans la région périnéoscrotale et qui se présente généralement chez des personnes âgées de race blanche sous forme de tache prurigineuse rougeâtre ou blanchâtre dans la zone de distribution des glandes apocrines. D’ordinaire, elle atteint un seul foyer. Puisque ses manifestations sont insidieuses et faciles à mal diagnostiquer, sa prise en charge convenable est retardée. D’ailleurs, cette prise en charge est complexe. Toutefois, un traitement efficace peut non seulement faire chuter le taux de récidive mais également assurer une reconstruction optimale. Le présent rapport décrit trois patients atteints d’une MPEM scrotale. Compte tenu d’une recherche dans la documentation scientifique, l’étiopathologie, le diagnostic et la prise en charge de ces lésions sont abordés. Les possibilités de reconstruction, surtout axées sur les lésions scrotales, sont également examinées.

Keywords: Cutaneous pruritic patch; Extramammary Paget’s disease; Scrotal lesion.

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Figures

Figure 1)
Figure 1)
Preoperative view of patient in Case 1 reveals an erythematous area along the medial aspect of the thigh and extending to the scrotum on the right side. Note the lack of ulceration and a seemingly well-defined macroscopic border
Figure 2)
Figure 2)
Postoperative view following complete resection of the lesion followed by reconstruction of the scrotal defect using a fasciocutaneous advancement flap from the scrotum itself and a rhomboid flap from the medial aspect of the thigh to close the defect in that area
Figure 3)
Figure 3)
Low power microscopic view of the specimen reveals tumour cells infiltrating the skin and the adnexa
Figure 4)
Figure 4)
High power microscopy confirm the presence of Paget cells – abundant and large, these cells are characteristic with pale cytoplasm.Distributed singly and in groups they are noted throughout the epidermis and the epithelia of the adnexa. Upper regions of the dermis show dense inflammatory cells
See this image and copyright information in PMC

References

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