vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v24i4.2946vsp-3808Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSДебют острого фебрильного нейтрофильного дерматоза Свита у ребенка после перенесенного COVID-19: клинический случайAcute Febrile Neutrophilic Dermatosis (Sweet Syndrome) Onset in a Child After COVID-19: Case Studyhttps://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; профессор кафедры дерматовенерологии и косметологии; профессор кафедры педиатрии и детской ревматологии

119991, Москва, Ломоносовский пр-т, д. 2, стр. 1

тел.: +7 (495) 967-14-20

Moscow

m_nn2001@mail.ruhttps://orcid.org/0000-0002-4107-4642ЕпишевР. В.EpishevRoman V.

Москва

Moscow

https://orcid.org/0009-0004-6841-6173БакуноваН. А.BakunovaNatalia A.

Москва

Moscow

https://orcid.org/0000-0001-9857-4456РодинаЮ. А.RodinaYulia A.

Москва

Moscow

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

Москва

Moscow

https://orcid.org/0000-0002-6034-8231МатерикинА. И.MaterikinAlexandr I.

Москва

Moscow

https://orcid.org/0000-0002-0858-8780ОпрятинЛ. А.OpryatinLeonid A.

Москва

Moscow

https://orcid.org/0000-0001-5739-0941ЛеоноваМ. А.LeonovaMariya A.

Москва

Moscow

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

Москва

Moscow

https://orcid.org/0000-0002-9399-6534АведоваА. Я.AvedovaAsmik Ya.

Москва

Moscow

Национальный медицинский исследовательский центр здоровья детей; Первый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет); Центральная государственная медицинская академияРоссияNational Medical Research Center of Children’s Health; Sechenov First Moscow State Medical University; Central State Medical Academy of Department of Presidential AffairsRussian FederationНациональный медицинский исследовательский центр здоровья детейРоссияNational Medical Research Center of Children’s HealthRussian FederationЦентральная государственная медицинская академияРоссияCentral State Medical Academy of Department of Presidential AffairsRussian FederationНациональный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Д. РогачеваРоссияDmitry Rogachev National Medical Research Center Of Pediatric Hematology, Oncology and ImmunologyRussian Federation202518092025244246254Copyright © Мурашкин Н.Н., Епишев Р.В., Бакунова Н.А., Родина Ю.А., Иванов Р.А., Материкин А.И., Опрятин Л.А., Леонова М.А., Павлова Е.С., Аведова А.Я., 20252025Мурашкин Н.Н., Епишев Р.В., Бакунова Н.А., Родина Ю.А., Иванов Р.А., Материкин А.И., Опрятин Л.А., Леонова М.А., Павлова Е.С., Аведова А.Я.Murashkin N.N., Epishev R.V., Bakunova N.A., Rodina Y.A., Ivanov R.A., Materikin A.I., Opryatin L.A., Leonova M.A., Pavlova E.S., Avedova A.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/3808

Обоснование. Синдром Свита — редкое воспалительное заболевание из группы нейтрофильных дерматозов, характеризующееся гиперпродукцией провоспалительных цитокинов. Описание клинического случая. У ребенка, возраст 1 год 3 мес, с направительным диагнозом «Гангренозная пиодермия» установлен клинический диагноз: «Острый фебрильный нейтрофильный дерматоз Свита». С целью уточнения диагноза проведено молекулярно-генетическое исследование — полноэкзомное секвенирование венозной крови пациента и его матери, в результате которого у обоих в экзоне 26 гена UBA1 был выявлен нуклеотидный вариант c.3061C>T. В связи с тяжестью патологического кожного процесса, высокой концентрацией интерлейкинов 1 и 6, а также в соответствии с опытом ведения таких пациентов, описанным в медицинской литературе, поэтапно проведена терапия препаратами метилпреднизолон, дапсон, тофацитиниб, анакинра, руксолитиниб. На фоне лечения отмечалась стойкая ремиссия кожного патологического процесса. Заключение. Представлен клинический случай острого фебрильного нейтрофильного дерматоза Свита. Продемонстрированы результаты успешной медикаментозной терапии.

Background. Sweet syndrome is a rare inflammatory disease from the group of neutrophilic dermatoses, characterized by proinflammatory cytokines hyperproduction. Case description. A child, 1 year 3 months old, with referral diagnosis of “Pyoderma gangrenosum” was diagnosed: «Acute febrile neutrophilic dermatosis. Sweet syndrome». Molecular genetic testing was carried out to clarify the diagnosis: whole-exome sequencing of venous blood from the patient and his mother. As a result both of them had nucleotide variant c.3061C>T in exon 26 of the UBA1 gene. Step-up therapy with methylprednisolone, dapsone, tofacitinib, anakinra, and ruxolitinib was administered due to pathological skin process severity, high interleukins 1 and 6 levels, and according to the experience in management of such patients described in medical literature. Sustained remission of the skin pathological process was noted on treatment. Conclusion. Case study of acute febrile neutrophilic dermatosis (Sweet syndrome) is presented. Results of successful drug therapy are shown.

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