Array
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Уровень 1
Уровень 2
Уровень 3
Уровень 4
Уровень 5
Уровень 6
Уровень 7
Уровень 9
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                    [~LESSON_ID] => 1287
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                    [~SORT] => 500
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                    [~EDGE_SORT] => 500
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                    [~IS_CHILDS] => 0
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                    [~SORT] => 500
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                    [~LESSON_ID] => 1258
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                    [~SORT] => 510
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                    [~LESSON_ID] => 1259
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                    [~SORT] => 500
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                    [~SORT] => 500
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                    [~LESSON_ID] => 1261
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                    [~LESSON_ID] => 1262
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                    [~IS_CHILDS] => 0
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~IS_CHILDS] => 0
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                    [~SORT] => 500
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                    [~CHAPTER_ID] => 1258
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                    [~TYPE] => LE
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                    [~DEPTH_LEVEL] => 2
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                    [~#LESSON_PATH] => 1060.1258.1264
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                    [~LESSON_ID] => 1265
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~LESSON_ID] => 1266
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                    [~IS_CHILDS] => 0
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                    [~SORT] => 500
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                    [~ID] => 1266
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                    [~TYPE] => LE
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                    [~DEPTH_LEVEL] => 2
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                    [~#LESSON_PATH] => 1060.1258.1266
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                    [~IS_CHILDS] => 0
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~EDGE_SORT] => 500
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                    [~IS_CHILDS] => 0
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~LESSON_ID] => 1270
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                    [~SORT] => 500
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                    [~SORT] => 500
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                    [~DEPTH_LEVEL] => 2
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                    [~SORT] => 500
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                    [URL] => /docschool/level8/chapter01293/?LESSON_PATH=1060.1293
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                    [~LESSON_ID] => 1295
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Уровень 9: Диффузные болезни соединительной ткани

Представляет

Раводин Роман Анатольевич
Дерматовенеролог, дерматоонколог, дерматокосметолог
Доктор медицинских наук, доцент
Категория: первая
Консультирует взрослых и детей
Город: Санкт-Петербург
Стаж: 15 лет
Базовое образование:
Военно-медицинская академия им. С.М. Кирова с золотой медалью, 1999
Дополнительное образование:
Адъюнктура при кафедре кожных и венерических болезней Военно-медицинской академии (2002-2005)
Основное место работы:
ООО "НПЦ ЛОГОДЕРМ", Северо-Западный государственный медицинский университет им. И.И. Мечникова,кафедра дерматовенерологии
Должность:
Доцент
Профессиональный опыт:
Занимается лечением и диагностикой всего спектра дерматовенерологических заболеваний, уделяя особое внимание редким и атипичным формам. При необходимости самостоятельно берёт соскобы и мазки, микроскопирует их и выдаёт заключение. Выполняет лазерное удаление невусов (родинок) и опухолей кожи. Является автором и соавтором более 70 научных работ по дерматовенерологии, включая два изобретения.

Девятый уровень включает 25 занятий.

 Он посвящён диффузным болезням соединительной ткани, встречающимся в практике врача-дерматолога: системной и кожным формам красной волчанки, системной склеродермии, морфеа (бляшечной склеродермии), склерозирующему лишаю, дерматомиозиту, смешанному заболеванию соединительной ткани.

 Уровень содержит свыше 400 клинических, дерматоскопических и гистологических фотографий с их подробным описанием.

 По его окончании вы познакомитесь с современной клинической характеристикой различных форм красной волчанки, склеродермии, дерматомиозита, узнаете об особенностях клинической диагностики склерозирующего лишая и смешанного заболевания соединительной ткани.

 Учитывая частое поражение кожи лица при этих заболеваниях, особое внимание уделено вопросам дифференциальной диагностики, включая клинические и дерматоскопические изображения с их описанием и комментариями.

 Приведены наиболее эффективные схемы лечения и профилактики, основанные на зарубежных и Российских клинических рекомендациях по дерматовенерологии 2015.

 После каждого занятия доступен тест для самопроверки (кнопка Тесты), а в конце уровня – Итоговый тест по всему пройденному материалу.

[~DETAIL_TEXT] =>

Уровень 9: Диффузные болезни соединительной ткани

Представляет

Раводин Роман Анатольевич
Дерматовенеролог, дерматоонколог, дерматокосметолог
Доктор медицинских наук, доцент
Категория: первая
Консультирует взрослых и детей
Город: Санкт-Петербург
Стаж: 15 лет
Базовое образование:
Военно-медицинская академия им. С.М. Кирова с золотой медалью, 1999
Дополнительное образование:
Адъюнктура при кафедре кожных и венерических болезней Военно-медицинской академии (2002-2005)
Основное место работы:
ООО "НПЦ ЛОГОДЕРМ", Северо-Западный государственный медицинский университет им. И.И. Мечникова,кафедра дерматовенерологии
Должность:
Доцент
Профессиональный опыт:
Занимается лечением и диагностикой всего спектра дерматовенерологических заболеваний, уделяя особое внимание редким и атипичным формам. При необходимости самостоятельно берёт соскобы и мазки, микроскопирует их и выдаёт заключение. Выполняет лазерное удаление невусов (родинок) и опухолей кожи. Является автором и соавтором более 70 научных работ по дерматовенерологии, включая два изобретения.

Девятый уровень включает 25 занятий.

 Он посвящён диффузным болезням соединительной ткани, встречающимся в практике врача-дерматолога: системной и кожным формам красной волчанки, системной склеродермии, морфеа (бляшечной склеродермии), склерозирующему лишаю, дерматомиозиту, смешанному заболеванию соединительной ткани.

 Уровень содержит свыше 400 клинических, дерматоскопических и гистологических фотографий с их подробным описанием.

 По его окончании вы познакомитесь с современной клинической характеристикой различных форм красной волчанки, склеродермии, дерматомиозита, узнаете об особенностях клинической диагностики склерозирующего лишая и смешанного заболевания соединительной ткани.

 Учитывая частое поражение кожи лица при этих заболеваниях, особое внимание уделено вопросам дифференциальной диагностики, включая клинические и дерматоскопические изображения с их описанием и комментариями.

 Приведены наиболее эффективные схемы лечения и профилактики, основанные на зарубежных и Российских клинических рекомендациях по дерматовенерологии 2015.

 После каждого занятия доступен тест для самопроверки (кнопка Тесты), а в конце уровня – Итоговый тест по всему пройденному материалу.

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Уровень 10
Уровень 11
Уровень 12
Уровень 13

Красная волчанка (патогенез, классификация)

Этиология и патогенез.

Этиология заболевания неизвестна. Патогенез заболевания связывают с ультрафиолетовым облучением, вирусными инфекциями, медикаментами и другими факторами, которые запускают аутоиммунные процессы, при этом происходит ускорение программируемой гибели (апоптоза) различных клеток (лимфоцитов периферической крови, кератиноцитов и др.) и нарушение процесса их элиминации из организма. В результате этого накапливается ядерный материал клеток (рибонуклеопротеины, двуспиральная ДНК и другие ядерные антигены). В норме белки системы комплемента присоединяются к апоптозным клеткам и стимулируют их фагоцитоз макрофагами, выступая в роли опсонинов. Поскольку при красной волчанке характерна гипокомплементемия, то апоптозные клетки распознаются не макрофагами, а дентритными антигенпрезентирующими клетками, которые перерабатывают ядерные антигены и запускают аутоиммунное воспаление. Полагают, что в процессе инициации аутоиммунного воспаления ключевая роль принадлежит фактору некроза опухоли-альфа (TNF-α) и системе Toll-подобных рецепторов. TNF-α вырабатывают как сами кератиноциты под воздействием различных факторов (например, УФО), так и дендритные клетки. Последние представляют переработанные ядерные антигены Т-лимфоцитам (CD4), которые стимулируют В-лимфоциты к секреции аутоантител, имеющих важное диагностическое значение при данном заболевании. При красной волчанке иммунный ответ направлен против нуклеопротеиновых антигенов (комплексов нуклеиновых кислот и белков), которые образуются в процессе апоптоза и напоминают чужеродные вирусные частицы. При кожных (несистемных) формах красной волчанки преобладают нарушения клеточного иммунитета (II тип реакций по Джеллу и Кумбсу), тогда как при СКВ чаще встречаются иммунокомплексные поражения (III тип реакций по Джеллу и Кумбсу).

Предрасполагающие факторы:

1. Наследственная предрасположенность (это полигенное заболевание с неполной пенетрантностью генов) – увеличение HLA-B27, HLA-B8, HLA-DR2, HLA-DR3, HLA-DQA0102 и уменьшение HLA-А2.


2. Диффузные болезни соединительной ткани у родственников.


3. Длительное пребывание на солнце, морозе, ветре (дворники, фермеры, строители, рыбаки).


4. Первый фототип кожи (по Фитцпатрику).


5. Очаги хронической инфекции в организме.


6. Солнечное (ультрафиолетовое) излучение.


7. Приём медикаментов (эстрогенов, тербинафина, ингибиторов фактора некроза опухоли-α, противосудорожных препаратов, ингибиторов протонной помпы, блокаторов кальциевых каналов, ингибиторов АПФ, β-блокаторов, лефлуномида и др.).


8. Вирусные инфекции (краснуха, цитомегаловирусная инфекция, вирус Эбштейн-Бар).


9. Травмы кожи.


10. Курение.


11. Беременность.


12. Стресс.

Классификация красной волчанки.


Красная волчанка (КВ) представляет собой один из вариантов ДБСТ, характеризующийся широким спектром клинических форм. Она может протекать с поражением только кожи, кожи и внутренних органов или одних внутренних органов. Впервые ее описал дерматолог Cazenave в 1851 г. В течение многих лет ее считали чисто кожным заболеванием. В 1872 г. дерматолог Kaposi впервые описал системные поражения при красной волчанке. Позднее было установлено, что системная красная волчанка (СКВ) представляет собой генерализованный процесс с поражением различных внутренних органов с кожными изменениями и без них. Долгое время в отечественной литературе выделяли системную красную волчанку и кожные формы красной волчанки (дискоидную и диссеминированную).

Вместе с накоплением информации об этом заболевании, изменились и представления о её формах, в зарубежной литературе используется доработанная классификация J. Gilliam и др. (1982), получившая мировое признание. В соответствии с этой классификацией выделяют специфические и неспецифические поражения кожи при красной волчанке. Среди специфических поражений различают острую, подострую и хроническую формы заболевания.

Классификация кожных форм КВ (Gronhagen C.M., 2014; Gilliam J.N., 1982; Sontheimer R.D., 1997):

I. Специфические проявления

1. Острая кожная КВ:

·        Локализованная

·        Распространённая

·        Подобная токсическому эпидермальному некролизу (очень редко)

2. Подострая кожная КВ:

·       Кольцевидная

·       Папулосквамозная/псориазиформная

·       Везикулобуллезная (редко)

·       Подобная токсическому эпидермальному некролизу (очень редко)

3. Хроническая кожная КВ:

·       Дискоидная:

        – Локализованная

        – Распространённая (Диссеминированная КВ)

·       Гипертрофическая/веррукозная

·       Глубокая/волчаночный панникулит

·       Опухолевидная/папуломуцинозная КВ

·       Ознобленная КВ (вследствие обморожения)

·       КВ слизистых оболочек (полости носа, рта, конъюнктивы, гениталий)

·       Лихеноидная дискоидная КВ (КВ/красный плоский лишай оверлап-синдром).

II. Неспецифические проявления КВ

1. Поражение сосудов кожи:

·       Вторичный лейкоцитокластический васкулит:

         – Пальпируемая пурпура

         – Уртикарный васкулит

·       Васкулопатии:

          – Болезнь Дегоса-подобные поражения

          – Ливедо-васкулит

·       Телеангиэктазии околоногтевых валиков

·       Сетчатое ливедо

·       Тромбофлебит

·       Феномен Рейно

·       Эритромелалгия

2. Нерубцовые алопеции:

·         Люпус-алопеция

·         Телогеновая алопеция

·         Гнездная алопеция

3. Склеродактилия

4. Ревматоидные узелки

5. Кальциноз кожи

6. Неспецифические буллезные поражения

7. Крапивница

8. Папуло-нодулярный муциноз

9. Анетодермия

10. Черный акантоз

11. Многоформная эритема

12. Язвы голени

13. Красный плоский лишай


Красная волчанка развивается преимущественно в возрасте 20-45 лет. Женщины болеют чаще мужчин (при системной красной волчанке соотношение составляет 8:1, при несистемных формах примерно 2:1).

Острая кожная форма красной волчанки практически всегда является проявлением системного процесса (СКВ), подострая кожная красная волчанка представляет собой переходную форму между системной и хроническими кожными формами. Эти варианты красной волчанки (подострая кожная красная волчанка и хронические кожные формы) являются единым процессом и могут трансформироваться в СКВ (в 3-5% случаев). Вместе с тем различия в характере клинических и иммунологических проявлений не исключают возможности рассматривать их как отдельные заболевания. Особую клиническую разновидность представляет собой так называемая медикаментозная красная волчанка (синдром красной волчанки), являющаяся одним из тяжелых вариантов лекарственной болезни.


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