Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children45010.24287/1726-1708-2020-19-4suppl-85-93MATERIALS OF THE 3rd WORKING MEETING OF ALLERGISTS-IMMUNOLOGISTSМАТЕРИАЛЫ III РАБОЧЕГО СОВЕЩАНИЯ АЛЛЕРГОЛОГОВ-ИММУНОЛОГОВGranulomatous skin lesion as a manifestation of primary immunodeficiency in childrenГранулематозное поражение кожи как проявление первичного иммунодефицитного состояния у детейhttps://orcid.org/0000-0003-1907-4168SeleznevaO. S.СелезневаО. С.
Russian Federation

allergist-immunologist of the department of pediatric hematology and oncology with chemotherapy339th Strelkovoy Divisii St. 14, Rostov-on-Don 344014, Russia

врач-аллерголог-иммунолог отделения детской гематологии и онкологии с химиотерапией 

344014, Ростов-на-Дону, ул. 339-й Стрелковой Дивизии, 14 

Selezneva-doc@mail.ruhttps://orcid.org/0000-0002-3113-4939ShcherbinaA. Yu.ЩербинаА. Ю.
Russian Federation

Moscow

Москва

Regional children's clinical hospitalГБУ РО «Областная детская клиническая больница»Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России11012021194supplementприложение85931001202110012021Copyright ©; 2021, «D. Rogachev NMRCPHOI»Copyright ©; 2021, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2021«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/450

Primary immunodeficiencies (PID) compile a genetically heterogeneous group of more than 400 disorders. Most patients with PID are shown to be highly susceptible to various types of infectious diseases. However, in the past decade, non-infectious complications associated with immune dysregulation and autoimmunity have been increasingly recognized in PID. Patients with PID often have skin manifestations, that allow to suspect the diagnosis of immunodeficiency in early childhood. One of the least studied skin manifestations of PID is granulomatous dermatitis. This manuscript current research on the pathogenesis, methods of diagnosis and therapy of granulomatous dermatitis in patients with various PID.

Первичные иммунодефицитные состояния (ПИДС) представляют собой генетически гетерогенную группу заболеваний из более 400 нозологий. Традиционно ПИДС проявляются повышенной восприимчивостью к различного рода инфекционным заболеваниям. Тем не менее в последнее десятилетие все большее значение приобретают неинфекционные осложнения, связанные с дисрегуляцией и аутоиммунными расстройствами. У пациентов с ПИДС часто встречаются кожные проявления, они являются одним из признаков, позволяющих заподозрить диагноз иммунодефицита в раннем детстве. При этом одним из наименее изученных кожных проявлений ПИДС является гранулематозный дерматит. Данный обзор посвящен обобщению данных исследований патогенеза, методов диагностики и терапии гранулематозного дерматита у пациентов с различными ПИДС.

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