Наследственные кожно-легочные синдромы

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

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