This article may be too technical for most readers to understand.(June 2018) |
Extramammary Paget's disease | |
---|---|
Micrograph of extramammary Paget's disease, H&E stain | |
Specialty | Dermatology, Oncology |
Symptoms | Rash, Itchiness, Eczematous lesions, Pain |
Causes | Unknown |
Diagnostic method | Excisional biopsy, histological pattern |
Named after | James Paget |
Extramammary Paget's disease (EMPD) is a rare and slow-growing cancer, which occurs within the epithelial tissues[1] and accounts for 6.5% of all Paget's disease.[2] This disease presents similarly to the more conventional form of mammary Paget's disease (MPD).[3] However, unlike MPD, which occurs in large milk-carrying ducts in the breast and then extends into the epidermis,[3] EMPD begins in glandular regions rich in apocrine secretions outside the mammary glands.[4] EMPD incidence is increasing by 3.2% every year, affecting hormonally-targeted tissues such as the vulva and scrotum.[5] In women, 81.3% of EMPD cases are related to the vulva, while for men, 43.2% of the manifestations present at the scrotum.[5]
The disease can be classified as being either primary or secondary depending on the presence or absence of associated malignancies.[6] EMPD presents with typical symptoms such as scaly, erythematous, eczematous lesions accompanied by itchiness.[6] In addition to this, 10% of patients are often asymptomatic.[7] As a consequence, EMPD has high rates of misdiagnoses and delayed diagnoses.[8] There are a variety of treatment options available, but most are unsuccessful. If caught early and treated, prognosis is generally good.