The current consensus for the diagnostic and treatment of extramammary Paget's disease; [Современный консенсус относительно диагностики и лечения экстрамаммарного рака Педжета]

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. © 2023 The Author(s).

Authors
Emelyanova O.O. , Zikirjahodzhaev A.D. , Saribekyan E.K. , Filonenko E.V.
Number of issue
3
Language
Russian
Pages
23-32
Status
Published
Volume
99
Year
2023
Organizations
  • 1 P. A. Hertsen Moscow Oncology Research Institute, Moscow, Russian Federation
  • 2 RUDN University, Moscow, Russian Federation
  • 3 Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russian Federation
Keywords
diagnosis of Paget's disease; extramammary Paget's disease; pathogenesis of Paget's disease; photodynamic therapy

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