Фолликулярный дискератоз Дарье

Адаскевич В.П. Фолликулярный дискератоз Дарье. Consilium Medicum. 2022;24(8):497–503. DOI:10.26442/20751753.2022.8.201830

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Adaskevich UP. Darier's follicular dyskeratosis. Consilium Medicum. 2022;24(8):497–503. DOI:10.26442/20751753.2022.8.201830

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Аннотация

Фолликулярный дискератоз Дарье (синонимы – болезнь Дарье, болезнь Дарье–Уайта) – редкое генетическое заболевание с аутосомно-доминантным типом наследования, которое относится к группе акантолитических дерматозов и характеризуется нарушением процессов ороговения с поражением кожи, ногтей, слизистых оболочек полости рта и гениталий. Болезнь Дарье вызывается мутацией в гене ATP2A2. Эта мутация изменяет работу насоса SERCA2 и приводит к нарушению гомеостаза кальция в кератиноцитах и снижению межклеточной адгезии. Заболевание проявляется в себорейных и интертригинозных областях коричневатыми папулами с кератотической поверхностью, которые иногда сливаются в мацерированные бляшки. Типичные изменения ногтей включают красные и белые продольные полосы, заканчивающиеся V-образными выемками на свободном крае ногтевых пластин. Клиническими вариантами болезни Дарье являются буллезный, геморрагический, комедоновый и линейно-сегментарный типы, а также бородавчатый акрокератоз. Болезнь Дарье часто связана с нервно-психическими расстройствами. Обострение может вызываться суперинфекцией золотистого стафилококка или вирусом простого герпеса. Гистология при болезни Дарье характеризуется выраженным дискератозом. Для местной терапии применяются кератолитические средства, антисептическая обработка, местные кортикостероиды. Среди других методов лечения наиболее эффективны системные ретиноиды. Абляционные методы (дермабразия, CO2-лазер, Er:YAG-лазер) эффективны на ограниченных участках.

Ключевые слова: фолликулярный дискератоз, болезнь Дарье, патогенез, клиника, диагностика, терапия

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Darier's follicular dyskeratosis (synonym: Darier’s disease, Darier–White’s disease) is a rare genetic disease with an autosomal dominant type of inheritance, which belongs to the group of acantholytic dermatoses and is characterized by a violation of keratinization processes with lesions of the skin, nails, mucous membranes of the oral cavity and genitals. Darier’s disease is caused by a mutation in the ATP2A2 gene. This disrupts the operation of the SERCA2 pump and leads to a violation of calcium homeostasis in keratinocytes and a decrease in intercellular adhesion. Darier’s disease is manifested by brownish papules in seborrheic and intertriginal areas with a keratotic surface, which can merge into macerated plaques. Typical nail changes in Darier’s disease include red and white longitudinal stripes ending in V-shaped notches on the free edge of the nail plates. Warty acrokeratosis, as well as bullous, hemorrhagic, comedonic and linear-segmental types are clinical variants of Darier’s disease. Darier’s disease is often associated with neuropsychiatric disorders. Exacerbation may be caused by superinfection with Staphylococcus aureus or by herpes simplex virus. Histology in Dariere’s disease is characterized by pronounced dyskeratosis. For local therapy, keratolytic agents are important, as well as antiseptic treatment to avoid superinfection. In addition, local corticosteroids are used. Among the systemic methods of treatment, the systemic retinoids are the most effective. Ablative methods of treatment (dermabrasion, CO2 laser, Er:YAG laser) are effective in limited areas.

Keywords: follicular dyskeratosis, Darier’s disease, pathogenesis, clinic, diagnosis, therapy

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Авторы

В.П. Адаскевич*

УО «Витебский государственный медицинский университет», Витебск, Республика Беларусь
*vitebsk.derma@mail.ru

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Uladzimir P. Adaskevich*

Vitebsk State Medical University, Vitebsk, Republic Belarus
*vitebsk.derma@mail.ru

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