Let's Talk Medicine

What is Paget’s Disease?

Via BMC Dermatology

Paget’s disease is a rare skin disorder occurring in the breast (mammary) or in the groin, genital, peri-anal and axillary regions (extra-mammary). Typical treatment involves surgical excision, which in the case of extra-mammary Paget’s disease, can lead to significant morbidity. Photodynamic therapy (PDT) which uses a topical or intravenous photosensitizing agent that is activated by a light source to ablate abnormal tissue, offers a minimally invasive alternative. The purpose of this study was to assess the effectiveness of photodynamic therapy in the treatment of Paget’s disease.

Paget’s disease is an extremely rare form of intraepithelial adenocarcinoma that may have an underlying tumour component. It primarily affects Caucasian women over the age of 50. Since in its early stages symptoms are often minor, individuals may not seek medical attention for several years, delaying diagnosis and treatment. In addition, the condition can be misdiagnosed as eczema or dermatitis, with a benign dermatologic diagnosis creating further delays.

Paget’s disease is categorized as either mammary or extramammary. While the two types are histologically the same (the epidermis shows diffuse infiltration of large vacuolated cells with a bluish cytoplasm (Paget cells)), the location differs. Mammary Paget’s disease involves the skin of the breast, in or around the nipple. Most individuals with the condition (> 92%) have underlying breast cancer (either ductal carcinoma in situ or invasive breast cancer).

Extra-mammary Paget’s disease mainly affects the perianal and genital areas, or elsewhere on the skin near apocrine glands, and is extremely rare (only a few hundred reports have been documented in peer-reviewed literature). In many patients, the disease may exist for 10 to 15 years without progressing. Unlike mammary Paget’s, a smaller proportion of cases exhibit an underlying neoplastic component (approximately 25% have carcinoma of the Bartholin glands, urethra, bladder, vagina, cervix, endometrium, prostate, rectum, or colon).

Corresponding author: Alexa A Nardelli
Department of Public Health Sciences, University of Alberta, Room 3021, Research Transition Facility, 8308 114 Street, Edmonton, Alberta, T6G 2V2, Canada

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This entry was posted on March 6, 2012 by in Dermatology.
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