Abstract
Porokeratosis is a rare disease with an autosomal dominant type of inheritance. It is customary to distinguish classic Mibelli porokeratosis, as well as disseminated and localized variations of the course of dermatosis, which differ in genetic predisposition, trigger factors, and treatment approaches. The article describes the variants of the disease – eruptive itchy papular malformation that occurs on the extremities against the background of microcirculation disorders and giant, developed on the skin of the trunk. The main methods of treatment of porokeratosis are also described.
Highlights
Summary Porokeratosis is a rare disease with an autosomal dominant type of inheritance
It is customary to distinguish classic Mibelli porokeratosis, as well as disseminated and localized variations of the course of dermatosis, which differ in genetic predisposition, trigger factors, and treatment approaches
The article describes the variants of the disease – eruptive itchy papular malformation that occurs on the extremities against the background of microcirculation disorders and giant, developed on the skin of the trunk
Summary
Принято выделять классический порокератоз Мибелли, а также диссеминированные и локализованные варианты течения дерматоза, которые различаются по генетической предрасположенности, триггерным факторам, подходам к лечению. В статье описаны варианты заболевания – эруптивный зудящий папулезный порокератоз, возникающий на конечностях фоне нарушений микроциркуляции, и гигантский, развившийся на коже туловища. Для всех вариантов заболевания характерна типичная клиническая картина в виде папул или бляшек, окруженных приподнятым роговым бортиком, а также общий гистологическая признак – тонкий столбик паракератотических клеток, проходящих через весь роговой слой [6]. Порокератоз Мибелли наследуется по аутосомно-доминантному типу, у мальчиков встречается в два раза чаще [7].
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