Back داء باجيت خارج الثدي Arabic Enfermedad de Paget extramamaria Spanish Maladie de Paget extramammaire French 乳房外ページェット病 Japanese Экстрамаммарная болезнь Педжета Russian 乳房外柏哲德氏病 Chinese
 | 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 malignancy which occurs within the epithelium[1] and accounts for 6.5% of all Paget's disease.[2] The clinical presentation of this disease is similar to the characteristics of mammary Paget's disease (MPD).[3] However, unlike MPD, which occurs in large lactiferous ducts and then extends into the epidermis,[3] EMPD originates 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.
- ^ Isrow D, Oregel KZ, Cortes J, Gomez H, Milikowski C, Feun L, Silva O (2014-07-06). "Advanced Extramammary Paget's Disease of the Groin, Penis, and Scrotum". Clinical Medicine Insights: Oncology. 8: 87–90. doi:10.4137/CMO.S13107. PMC 4116380. PMID 25089091.
- ^ Wagner G, Sachse MM (June 2011). "Extramammary Paget disease - clinical appearance, pathogenesis, management". Journal der Deutschen Dermatologischen Gesellschaft. 9 (6): 448–54. doi:10.1111/j.1610-0387.2010.07581.x. PMID 21205169.
- ^ a b Lloyd J, Flanagan AM (October 2000). "Mammary and extramammary Paget's disease". Journal of Clinical Pathology. 53 (10): 742–9. doi:10.1136/jcp.53.10.742. PMC 1731095. PMID 11064666.
- ^ Fukuda K, Funakoshi T (2018-02-16). "Metastatic Extramammary Paget's Disease: Pathogenesis and Novel Therapeutic Approach". Frontiers in Oncology. 8: 38. doi:10.3389/fonc.2018.00038. PMC 5820294. PMID 29503810.
- ^ a b Zhou S, Zhong W, Mai R, Zhang G (2017). "Mammary and Extramammary Paget's Disease Presented Different Expression Pattern of Steroid Hormone Receptors". BioMed Research International. 2017: 3768247. doi:10.1155/2017/3768247. PMC 5610822. PMID 29082243.
- ^ a b Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N (April 2015). "Mammary and extramammary Paget's disease". Anais Brasileiros de Dermatologia. 90 (2): 225–31. doi:10.1590/abd1806-4841.20153189. PMC 4371672. PMID 25830993.
- ^ Konda S, Chen W, Minus HR (March 2015). "What is your diagnosis? Extramammary Paget disease". Cutis. 95 (3): 132, 135–6, 142. PMID 25844784.
- ^ Hsu LN, Shen YC, Chen CH, Sung MT, Chiang PH (March 2013). "Extramammary Paget's disease with invasive adenocarcinoma of the penoscrotum: Case report and systematic review". Urological Science. 24 (1): 30–33. doi:10.1016/j.urols.2013.01.003. ISSN 1879-5226.