vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v24i4.2929vsp-3811Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSГенерализованный линейный порокератоз у ребенка: клинический случайGeneralized Linear Porokeratosis in a Child: Case Studyhttps://orcid.org/0000-0003-2252-8570МурашкинН. Н.MurashkinNikolay N.

Мурашкин Николай Николаевич, доктор медицинских наук, профессор, руководитель НИИ детской дерматологии; профессор кафедры дерматовенерологии и косметологии; профессор кафедры педиатрии и детской ревматологии

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

тел.: +7 (499) 134-08-89

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

Москва

Moscow

https://orcid.org/0009-0002-4991-4279ЕремееваА. Д.EremeevaAnastasiya D.

Москва

Moscow

https://orcid.org/0000-0002-5245-2987СычеваН. Л.SychevaNatalya L.

Краснодар

Krasnodar

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

Москва

Moscow

https://orcid.org/0000-0002-4107-4642ЕпишевР. В.EpishevRoman V.

Москва

Moscow

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

Москва

Moscow

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

Москва

Moscow

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

Москва

Moscow

https://orcid.org/0009-0001-0142-9090БорискинаС. А.BoriskinaSofya A.

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 FederationРоссийская медицинская академия непрерывного профессионального образованияРоссияRussian Medical Academy of Continuing Professional EducationRussian FederationКубанский государственный медицинский университетРоссияKuban State Medical UniversityRussian FederationПервый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет)РоссияSechenov First Moscow State Medical UniversityRussian Federation202518092025244267272Copyright © Мурашкин Н.Н., Опрятин Л.А., Еремеева А.Д., Сычева Н.Л., Павлова Е.С., Епишев Р.В., Материкин А.И., Леонова М.А., Иванов Р.А., Борискина С.А., 20252025Мурашкин Н.Н., Опрятин Л.А., Еремеева А.Д., Сычева Н.Л., Павлова Е.С., Епишев Р.В., Материкин А.И., Леонова М.А., Иванов Р.А., Борискина С.А.Murashkin N.N., Opryatin L.A., Eremeeva A.D., Sycheva N.L., Pavlova E.S., Epishev R.V., Materikin A.I., Leonov M.A., Ivanov R.A., Boriskina S.A.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/3811

Обоснование. Линейный порокератоз — редкий тип порокератоза, генетически детерминированное нарушение мевалонатного пути в кератиноцитах. Характеризуется высоким риском развития злокачественных новообразований (плоскоклеточный рак, базальноклеточный рак, болезнь Боуэна, меланома). Патогенетическое лечение не разработано. Описание клинического случая. Пациент К., возраст 8 лет, высыпания с первых дней жизни. С возрастом высыпания распространились по всему телу. На фоне приема ацитретина в дозировке 10 мг/сут спустя 3 мес отмечен положительный эффект в виде регресса части высыпаний. Заключение. Показан положительный эффект лечения линейного порокератоза ацитретином. Однако по-прежнему актуальным остается определение стандартных схем лечения порокератоза.

Background. Linear porokeratosis is a rare type of poroceratosis, genetically determined disorder of mevalonate pathway in keratinocytes. It is characterized by high risk of malignant neoplasms (squamous cell carcinoma, basal cell carcinoma, Bowen's disease, melanoma). There is no pathogenetic treatment. Case description. Patient K., 8 years old, had rashes from the first days of life. The rashes have spread all over the body with age. Positive effect in partial regression of rashes was noted after 3 months on acitretin administration at dosage of 10 mg/day. Conclusion. The positive effect of linear porokeratosis management via acitretin has been shown. However, the definition of standard treatment regimens for porokeratosis remains relevant.

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