vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v22i5.2621vsp-3305Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSРезультаты комбинированной патогенетической терапии при синдроме Нетертона: клинический случайResults of Combined Pathogenetic Therapy in Netherton Syndrome: Clinical Casehttps://orcid.org/0000-0003-2252-8570МурашкинН. Н.MurashkinNikolay N.

Мурашкин Николай Николаевич - доктор медицинских наук, профессор, руководитель НИИ детской дерматологии, заведующий отделением дерматологии и аллергологии, заведующий лабораторией патологии кожи у детей отдела научных исследований в педиатрии ФГАУ «НМИЦ здоровья детей» Минздрава России, профессор кафедры дерматовенерологии и косметологии ФГБУ ДПО «ЦГМУ» УДП РФ, профессор кафедры педиатрии и детской ревматологии ФГАОУ ВО Первый МГМУ им. И.М. Сеченова Минздрава России (Сеченовский Университет).

119991, Москва, Ломоносовский пр-т, д. 2, стр. 1, тел.: +7 (495) 967-14-20

Moscow

m_nn2001@mail.ruhttps://orcid.org/0000-0002-0858-8780ОпрятинЛ. А.OpryatinLeonid A.

Москва

Moscow

https://orcid.org/0009-0004-3764-4034Бридан-РостовскаяА. С.Bridan-RostovskayaAnna S.

Москва

Moscow

https://orcid.org/0009-0003-5367-3268ПавловаЕ. С.PavlovaEkaterina S.

Москва

Moscow

https://orcid.org/0000-0002-0081-0981ИвановР. А.IvanovRoman A.

Москва

Moscow

https://orcid.org/0000-0002-7335-6329АветисянК. О.AvetisyanKarine O.

Москва

Moscow

https://orcid.org/0000-0002-1107-8693КуликовК. А.KulikovKirill A.

Москва

Moscow

НМИЦ здоровья детей; Первый МГМУ им. И.М. Сеченова (Сеченовский Университет); ЦГМАРоссияNational Medical Research Center of Children’s Health; Sechenov First Moscow State Medical University; Central State Medical Academy of Department of Presidential AffairsRussian FederationНМИЦ здоровья детейРоссияNational Medical Research Center of Children’s HealthRussian Federation202306112023225433442Copyright © Мурашкин Н.Н., Опрятин Л.А., Бридан-Ростовская А.С., Павлова Е.С., Иванов Р.А., Аветисян К.О., Куликов К.А., 20232023Мурашкин Н.Н., Опрятин Л.А., Бридан-Ростовская А.С., Павлова Е.С., Иванов Р.А., Аветисян К.О., Куликов К.А.Murashkin N.N., Opryatin L.A., Bridan-Rostovskaya A.S., Pavlova E.S., Ivanov R.A., Avetisyan K.O., Kulikov K.A.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/3305Обоснование

Обоснование. Синдром Нетертона — тяжелое аутосомно-рецессивное заболевание, в основе которого лежат не только генетически детерминированное нарушение кератинизации, но и дисрегуляция иммунной системы.

Описание клинического случая. Мальчик К., 9 лет, с диагнозом «атопический дерматит тяжелого течения», у которого в ходе обследований был выявлен синдром Нетертона. В связи с наличием выраженных явлений поливалентной сенсибилизации и клинических признаков аллергического процесса назначен дупилумаб. На фоне данной терапии отмечалось волнообразное течение заболевания. На 7-м мес лечения у пациента развилось выраженное обострение в виде генерализованной пустулезной сыпи. Опираясь на данные зарубежной литературы патогенетического лечения синдрома Нетертона ингибитором интерлейкина (IL) 17, пациенту инициировали терапию препаратом секукинумаб, на фоне которой отмечалась положительная динамика. В настоящее время пациент получает комбинированную терапию ингибиторами IL-17 и IL-4/13 с выраженным положительным эффектом.

Заключение

Заключение. Генно-инженерные биологические препараты, направленные как на ось Th17, так и на Th2, показывают свою эффективность при синдроме Нетертона.

Background

Background. Netherton syndrome is a severe autosomal recessive disease based not only on genetically determined keratinization disorders, but also on immune system dysregulation.

Clinical case description. Boy K., 9 years old, diagnosed with severe atopic dermatitis and revealed Netherton syndrome during examinations. The dupilumab was administered due to the presence of severe polysensitization and clinical signs of allergic process. Wave-like course of the disease was observed during this therapy. Patient had acute exacerbation — generalized pustular rash — at the 7th month of treatment. The therapy with secukinumab was initiated according to the foreign literature on pathogenetic treatment of Netherton syndrome with interleukin (IL) 17 inhibitor. It has shown positive dynamics. Currently patient is administered with the combined therapy of IL-17 and IL-4/13 inhibitors with significant positive effect.

Conclusion

Conclusion. Genetically engineered biologic drugs targeting both the Th17 and Th2 have shown their efficacy in Netherton syndrome management.

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The authors declare that there are no conflicts of interest present.