pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2025-35-6-854-865pulmo-4721Research ArticleОБЗОРЫREVIEWНаследственные кожно-легочные синдромыHereditary skin-pulmonary syndromeshttps://orcid.org/0000-0003-0059-9159ГостеваТ. А.GostevaT. A.

Гостева Татьяна Александровна – врач-терапевт, пульмонолог, заместитель главного врача по клинико-экспертной работе; член Курской региональной общественной организации «Общество интегративной дерматологии»

307250, Курская обл., Курчатов, ул. Энергетиков,10, тел.: (908) 120-40-45 

Tatyana A. Gosteva, Physician-Therapist, Pulmonologist, Deputy Chief Physician for Clinical and Expert Work; Member of the Kursk regional public organization “Society of Integrative Dermatology”

ul. Energetikov 10, Kursk region, Kurchatov, 307250, tel.: (908) 120-40-45 

gosteva.t.tatyana@yandex.ruhttps://orcid.org/0000-0001-9676-1581СаранюкР. В.SaranyukR. V.

Саранюк Роман Владимирович – врач-дерматовенеролог, руководитель; президент Курской региональной общественной организации «Общество интегративной дерматологии»

305006, Курск, просп. Анатолия Дериглазова, 1, офис 3, тел.: (910) 214-95-17 

Roman V. Saranyuk, Dermatovenerologist, Head; President, Kursk Regional Public Organization “Society of Integrative Dermatology”

prosp. Anatoliya Deriglazova 1, office 3, Kursk, 305006, tel.: (910) 214-95-17 

roman.saranuk@gmail.comОбщество с ограниченной ответственностью «Курчатовский центр современной медицины»Россия“Kurchatov Center for Modern Medicine” Limited Liability CompanyRussian FederationКабинет дерматологии и венерологии “Derma Эксперт”РоссияCabinet of Dermatology and Venereology “Derma Expert”Russian Federation202514122025356854865Copyright © Гостева Т.А., Саранюк Р.В., 20252025Гостева Т.А., Саранюк Р.В.Gosteva T.A., Saranyuk R.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/4721

Кожа как орган часто вовлекается в патологические процессы, происходящие внутри различных систем организма. Это может наблюдаться в случаях патологии дыхательной системы, при которой на коже и ее придатках отмечается множество патологических изменений. Поражение той или иной системы может привести к вовлечению в патологический процесс другой системы, создавая порой сложную междисциплинарную проблему. Особенно ярко сочетанные поражения органов дыхания и кожи наблюдаются в качестве составляющих ряда наследственных синдромов, при которых патология кожи и органов дыхания зачастую являются ведущими симптомами заболевания. В случае наследственной патологии поражение кожи и органов дыхания происходит вследствие генетически обусловленного нарушения работы и созревания определенного физиологического звена. Генетически обусловленные нарушения со стороны сосудов (синдром Рандю–Ослера–Вебера, Клиппеля–Треноне), соединительной ткани (нейрофиброматоз I типа, синдром Элерса–Данлоса, Сutis laxa, туберозный склероз), меланоцитарной системы (синдром Германски–Пудлака) вовлекают в патологический процесс целый ряд органов и систем организма, включая кожу и органы дыхания. Целью данной работы явилось освещение имеющихся данных о наследственных заболеваниях и синдромах с вовлечением кожи и органов дыхания. Заключение. Знание о клинических взаимодействиях в рамках наследственных кожно-легочных синдромов, их своевременная диагностика и рациональное лечение могут помочь увеличить выживаемость пациентов и сохранить качество жизни. При рассмотрении данного междисциплинарного вопроса на стыке пульмонологии и дерматологии требуется дальнейшее изучение с целью совершенствования методов медицинской помощи пациентам данной группы

The skin, as an organ, is often involved in pathological processes occurring within various body systems. This can be observed in respiratory conditions associated with many pathological changes on the skin and its appendages. Damage to one system can lead to the involvement of another in the pathological process, sometimes creating a complex interdisciplinary problem. Especially vivid combined lesions of the respiratory organs and skin are observed as components of a number of hereditary syndromes, in which the skin and respiratory conditions are often the leading symptoms of the disease. In the case of hereditary pathology, lesions of the skin and respiratory organs occur due to a genetically determined disruption of the work and maturation of a certain physiological link. Genetically determined disorders of the vessels (Rendu – Osler – Weber syndrome, Klippel – Trenaunay syndrome), connective tissue (neurofibromatosis type I, Ehlers – Danlos syndrome, cutis laxa, tuberous sclerosis), melanocytic system (Hermansky – Pudlak syndrome) – all involve a number of organs and body systems in the pathological process, including the skin and respiratory organs. 

The aim of this work is to provide an overview of the available data on hereditary diseases and syndromes involving the skin and respiratory system.

Conclusion. Knowledge of clinical interactions within hereditary skin and lung syndromes, their modern diagnostics and rational treatment can help to increase patient survival and maintain their quality of life. This interdisciplinary issue at the intersection of pulmonology and dermatology requires further study in order to improve the methods of medical care for this group of patients.

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