Vestnik dermatologii i venerologiiVestnik dermatologii i venerologiiВестник дерматологии и венерологии0042-46092313-6294Rossijskoe Obschestvo Dermatovenerologov i Kosmetologov140010.25208/vdv1400REVIEWSОБЗОР ЛИТЕРАТУРЫReview ArticleThe current consensus for the diagnostic and treatment of extramammary Paget’s diseaseСовременный консенсус относительно диагностики и лечения экстрамаммарного рака Педжетаhttps://orcid.org/0000-0002-1276-4513EmelyanovaOlga O.ЕмельяноваОльга Олеговна
Russian Federation
yemelyanova-olya@list.ruhttps://orcid.org/0000-0001-7141-25028421-0364ZikirjahodzhaevAziz D.ЗикиряходжаевАзиз Дильшодович
Russian Federation

MD, Dr. Sci. (Med.)

д.м.н.

azizz@mail.ruhttps://orcid.org/0000-0003-0827-79983491-0586SaribekyanErik K.СарибекянЭрик Карлович
Russian Federation

MD, Dr. Sci. (Med.)

д.м.н.

mammolog3@yandex.ruhttps://orcid.org/0000-0001-8506-74556868-9605FilonenkoElena V.ФилоненкоЕлена Вячеславовна
Russian Federation

MD, Dr. Sci. (Med.), Professor

д.м.н., профессор

derkul23@yandex.ruP. A. Hertsen Moscow Oncology Research InstituteМосковский нaучнo-иccлeдoвaтeльcкий oнкoлoгичecкий инcтитут имeни П. A. Гepцeнa — филиaл Нaциoнaльного медицинского иccлeдoвaтeльcкого цeнтpа paдиoлoгииRUDN UniversityPoccийcкий унивepcитeт дpужбы нapoдoвSechenov First Moscow State Medical University (Sechenov University)Первый МГМУ им. И. М. Сеченова (Сеченовский университет)110520232507202399323323110202214042023Copyright ©; 2023, Emelyanova O.O., Zikirjahodzhaev A.D., Saribekyan E.K., Filonenko E.V.Copyright ©; 2023, Емельянова О.О., Зикиряходжаев А.Д., Сарибекян Э.К., Филоненко Е.В.2023Emelyanova O.O., Zikirjahodzhaev A.D., Saribekyan E.K., Filonenko E.V.Емельянова О.О., Зикиряходжаев А.Д., Сарибекян Э.К., Филоненко Е.В.https://creativecommons.org/licenses/by-nc/4.0https://vestnikdv.ru/jour/article/view/1400

Extramammary Paget's disease is a slow-growing cutaneous intraepithelial adenocarcinoma of the apocrine glands in the anogenital and axillary regions. The disease is extremely rare, affects predominantly postmenopausal women, and has nonspecific clinical caracteristics, so that it can take 2–10 years from the first clinical signs to diagnosis.

The vulva and the perianal region are the most affected zones. Extramammary Paget’s disease is clinically manifested as well-defined erythematous plaques with secondary changes, such as scaling, ulceration and even bleeding.

Differential diagnosis is made between primary and secondary forms of Extramammary Paget’s disease, candidiasis, contact dermatitis, Crohn's disease, eczema, erosive lichen planus, hydradenitis suppurativa, Langerhans cell histiocytosis, sclerosing lichen, psoriasis, squamous cell carcinoma in situ, amelanotic melanoma and mycosis fungoide.

Treatment mainly includes the surgical component, as well as the use of photodynamic therapy, cytostatic agents, and, depending on the stage, systemic chemotherapy.

Экстрамаммарный рак Педжета — медленно растущая кожная внутриэпителиальная аденокарцинома апокринных желез в аногенитальной и подмышечных областях. Заболевание встречается крайне редко, преимущественно у женщин постменопаузального возраста и характеризуется неспецифической клинической картиной, в связи с чем от момента первых клинических признаков до постановки диагноза может пройти 2–10 лет.

Чаще всего поражаются вульва и перианальная зона, при этом клинически заболевание проявляется в виде четко очерченных эритематозных бляшек со вторичными изменениями, такими как шелушение, изъязвление и даже кровотечение.

Дифференциальная диагностика проводится между первичными и вторичными формами экстрамаммарного рака Педжета, кандидозом, контактным дерматитом, болезнью Крона, экземой, эрозивным красным плоским лишаем, гнойным гидраденитом, гистиоцитозом из клеток Лангерханса, склероатрофическим лихеном, псориазом, плоскоклеточной карциномой in situ, амеланотической меланомой и грибовидным микозом.

Лечение в основном включает хирургический компонент, также возможно применение фотодинамической терапии, цитостатических агентов, в зависимости от стадии — системной химиотерапии.

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