title: Mammary and extramammary Paget's disease.
abstract: Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.
authors.affiliation: Universidade Federal do Piauí, Teresina, PI, Brazil.
authors.forename: Lauro Lourival
authors.initials: LL
authors.lastname: Lopes Filho
authors.affiliation: Universidade Federal do Piauí, Teresina, PI, Brazil.
authors.forename: Ione Maria Ribeiro Soares
authors.initials: IM
authors.lastname: Lopes
authors.affiliation: Universidade Federal do Piauí, Teresina, PI, Brazil.
authors.forename: Lauro Rodolpho Soares
authors.initials: LR
authors.lastname: Lopes
authors.affiliation: Universidade Federal de São Paulo, São Paulo, SP, Brazil.
authors.forename: Milvia M S S
authors.initials: MM
authors.lastname: Enokihara
authors.affiliation: Universidade de Santo Amaro, Santo Amaro, SP, Brazil.
authors.forename: Alexandre Osores
authors.initials: AO
authors.lastname: Michalany
authors.affiliation: Faculdade de Medicina do ABC (FMABC), Santo André, SP, Brazil.
authors.forename: Nobuo
authors.initials: N
authors.lastname: Matsunaga
mesh_terms: D001943:Breast Neoplasms / Q000473:pathology* / Q000628:therapy; D002277:Carcinoma / Q000473:pathology / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D005834:Genital Neoplasms, Male / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male; D009558:Nipples / Q000473:pathology; D010145:Paget Disease, Extramammary / Q000473:pathology* / Q000628:therapy; D010144:Paget's Disease, Mammary / Q000473:pathology* / Q000628:therapy; D012878:Skin Neoplasms / Q000473:pathology* / Q000628:therapy; D014846:Vulvar Neoplasms / Q000473:pathology / Q000628:therapy
references.citation: J Am Coll Surg. 2008 Feb;206(2):316-21
references.pmid: 18222386
references.citation: Histopathology. 2007 Mar;50(4):439-47
references.pmid: 17448019
references.citation: J Cutan Pathol. 1986 Feb;13(1):59-66
references.pmid: 3009569
references.citation: Gynecol Oncol. 2012 May;125(2):346-51
references.pmid: 22293043
references.citation: Am Surg. 1996 Dec;62(12):1068-72
references.pmid: 8955252
references.citation: Dermatologica. 1985;170(4):170-9
references.pmid: 2987049
references.citation: J Eur Acad Dermatol Venereol. 2013 Jan;27(1):e24-9
references.pmid: 22211938
references.citation: J Natl Cancer Inst. 2000 Apr 19;92(8):622-8
references.pmid: 10772679
references.citation: Lancet. 1989 Sep 16;2(8664):664-6
references.pmid: 2570909
references.citation: APMIS. 1995 Sep;103(9):628-34
references.pmid: 7488383
references.citation: J Cutan Pathol. 1994 Jun;21(3):283-6
references.pmid: 7962832
references.citation: J Eur Acad Dermatol Venereol. 2007 May;21(5):581-90
references.pmid: 17447970
references.citation: Anticancer Res. 1993 Nov-Dec;13(6B):2429-33
references.pmid: 8135479
references.citation: Arch Pathol Lab Med. 1990 Aug;114(8):858-61
references.pmid: 1695839
references.citation: Arch Pathol Lab Med. 1993 Mar;117(3):291-4
references.pmid: 7680194
references.citation: Gynecol Oncol. 2002 Aug;86(2):150-6
references.pmid: 12144821
references.citation: BMC Dermatol. 2011;11:13
references.pmid: 21676258
references.citation: Histopathology. 2002 Mar;40(3):230-6
references.pmid: 11895488
references.citation: Invest Radiol. 2005 Jun;40(6):363-7
references.pmid: 15905723
references.citation: Br J Dermatol. 1980 Oct;103(4):443-8
references.pmid: 6254556
references.citation: Mod Pathol. 1997 Nov;10(11):1069-74
references.pmid: 9388055
references.citation: Gynecol Oncol. 1998 Oct;71(1):137-40
references.pmid: 9784335
references.citation: South Med J. 2006 Apr;99(4):396-402
references.pmid: 16634252
references.citation: Hum Pathol. 2002 May;33(5):545-8
references.pmid: 12094381
references.citation: Am J Gastroenterol. 1995 Mar;90(3):507-8
references.pmid: 7872301
references.citation: Br J Surg. 1991 Jun;78(6):722-3
references.pmid: 1648987
references.citation: Mod Pathol. 2005 Oct;18(10):1283-8
references.pmid: 15920545
references.citation: Hum Pathol. 2002 May;33(5):549-54
references.pmid: 12094382
references.citation: J Carcinog. 2011;10:31
references.pmid: 22279416
references.citation: Cancer. 2002 Jul 1;95(1):1-7
references.pmid: 12115309
references.citation: An Bras Dermatol. 2013 Mar-Apr;88(2):290-2
references.pmid: 23739693
references.citation: J Am Acad Dermatol. 1990 Aug;23(2 Pt 2):338-41
references.pmid: 2168446
references.citation: Ann Surg Oncol. 1997 Jun;4(4):287-92
references.pmid: 9181226
references.citation: Breast J. 2005 Sep-Oct;11(5):355-6
references.pmid: 16174159
references.citation: Histopathology. 2010 Oct;57(4):564-71
references.pmid: 20955381
references.citation: J Surg Oncol. 2001 Jul;77(3):171-8
references.pmid: 11455553
references.citation: Radiother Oncol. 1990 Apr;17(4):305-9
references.pmid: 2160679
references.citation: Breast J. 2005 Jul-Aug;11(4):281-2
references.pmid: 15982397
references.citation: Ann Surg Oncol. 2005 May;12(5):391-7
references.pmid: 15915373
references.citation: J Clin Pathol. 2000 Oct;53(10):742-9
references.pmid: 11064666
references.citation: Pathol Int. 2010 Nov;60(11):726-34
references.pmid: 20946522
references.citation: Cancer. 1984 Aug 1;54(3):545-51
references.pmid: 6329506
references.citation: Histopathology. 1990 Sep;17(3):243-7
references.pmid: 2173676
references.citation: Am J Obstet Gynecol. 1999 Jan;180(1 Pt 1):24-7
references.pmid: 9914572
references.citation: Eur J Radiol. 2006 Nov;60(2):256-63
references.pmid: 16887314