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

Общие понятия

Диффузные болезни соединительной ткани с иммунными нарушениями (диффузная коллагеновая болезнь, коллагенозы) – группа аутоиммунных заболеваний, в основе которых лежит дезорганизация соединительной ткани, обусловленная аутоиммунными процессами и нарушением микроциркуляции. Основными представителями диффузных болезней соединительной ткани (ДБСТ) являются: системная и кожные формы красной волчанки, системная склеродермия, локализованная склеродермия (морфеа), склеротический лишай, дерматомиозит, смешанное заболевание соединительной ткани, ревматоидный артрит и синдром Шегрена.

Поражение кожи является почти постоянным, а нередко, первым симптомом ДБСТ. Выделяют такие формы ДБСТ, при которых дерматологические проявления доминируют в течение многих лет, а иногда всей жизни больного, являясь при этом единственным симптомом болезни. Вопрос о том, являются ли кожные и системные формы красной волчанки и склеродермии единым процессом, остается открытым, хотя имеются достаточно убедительные данные, свидетельствующие о возможности трансформации кожных форм красной волчанки и некоторых форм ограниченной склеродермии в системные процессы. Определенные трудности для дерматологов представляет собой дифференциальная диагностика кожных проявлений ДБСТ с другими дерматозами, имеющими с ними выраженное клиническое сходство.

Общая характеристика диффузных болезней соединительной ткани с иммунными нарушениями

В основу объединения ДБСТ в одну группу положен ряд общих клинических, морфологических и иммунологических признаков.

Впервые было обращено внимание на общность патоморфологических изменений, заключающихся в прогрессирующей дезорганизации соединительной ткани, возникающей вследствие нарушений иммуногенеза и микроциркуляции. Основным патоморфологическим признаком при этом является «фибриноидное набухание коллагена», что дало основание в 1942 году Клемпереру с соавт. объединить данные заболевания под названием «диффузная коллагеновая болезнь» или «коллагеноз». Оказалось, однако, что при коллагенозе дезорганизация соединительной ткани касается всех ее компонентов: волокнистых структур, межуточного аморфного вещества, сосудов, клеток, нервных волокон, в связи с чем в настоящее время вместо названия коллагенозы употребляют термин «диффузные болезни соединительной ткани с иммунными нарушениями».

Различают четыре фазы дезорганизации соединительной ткани при ДБСТ: мукоидное набухание, фибриноидное изменение, развитие клеточных реакций и склероз ткани.

1. Мукоидное набухание характеризуется перераспределением и накоплением в аморфном веществе соединительной ткани гликозаминогликанов (кислых мукополисахаридов): хондроитинсульфата, гиалуроновой кислоты и других в связи с их высвобождением из комплексов с белками. Это поверхностная и обратимая стадия дезорганизации соединительной ткани.

2. Фибриноидное изменение соединительной ткани связано с проникновением в ткань вследствие повышенной сосудистой проницаемости белков плазмы (альбуминов, глобулинов, фибриногена, причем последний быстро превращается в фибрин). Гликозаминогликаны вступают в нерастворимые соединения с фибриногеном при его переходе в фибрин, откладываются как на коллагеновых волокнах, так и между ними. В течение этого процесса происходит патологическая рекомбинация тканевых и плазменных белков и полисахаридов. Вся вновь образующаяся гетерогенная масса, пропитывающая соединительную ткань, носит название фибриноида, в котором всегда обнаруживаются иммунные комплексы. В результате в коллагеновых волокнах происходят необратимые процессы вплоть до некроза (фибриноидный некроз).

3. Клеточные реакции. Вокруг очагов фибриноидных изменений (некроза) наблюдается очаговая (узелки, гранулемы) или диффузная пролиферация гистиоцитов, фибробластов, макрофагов, лимфоцитов, плазматических клеток.

4. Склероз возникает в связи с пролиферацией фибробластов с последующим коллагенообразованием.


Для ДБСТ характерна одновременность проявлений различных фаз дезорганизации соединительной ткани: наличие как старых, так и свежих (при обострении) процессов. Хроническое волнообразное течение, постоянная смена периодов затихания и обострения процесса, наслаивания одной фазы дезорганизации на другую создают чрезвычайно полиморфную морфологическую картину тканевых изменений.

Ведущую роль в патогенезе ДБСТ играют аутоиммунные процессы. Все ДБСТ считаются классическим примером органонеспецифических аутоиммунных болезней, характерными чертами которых являются: 1) образование аутоантител; 2) наличие в очагах поражения комплексов антиген – антитело; 3) гипергаммаглобулинемия; 4) скопление в пораженных тканях плазматических и лимфоидных клеток; 5) недостаточность Т-системы иммунитета (дефект Т-супрессоров); 6) эффективность кортикостероидов и других иммунодепрессантов; 7) сочетание с другими аутоиммунными процессами (тиреоидит Хашимото, гемолитическая анемия, тромбоцитопеническая пурпура).

Важную роль в патогенезе ДБСТ играет сосудистая патология, которая характеризуется воспалением сосудов, преимущественно микроциркуляторного русла.

Общность патогенеза и патоморфологических изменений определяет принципиальное клиническое сходство ДБСТ, что, прежде всего, выражается в полисистемности патологического процесса с частым вовлечением сосудов, серозных оболочек, кожи, внутренних органов, нервной системы.

Внутри группы ДБСТ отличаются друг от друга по преимущественной локализации патологического процесса: при системной склеродермии в основном поражается соединительная ткань кожи и внутренних органов, при системной красной волчанке наблюдаются деструктивные изменения соединительной ткани почек, сердца, сосудов, кожи и гематотологические изменения (анемия, лейкопения, тромбоцитопения); при дерматомиозите отмечается системное поражение поперечнополосатой и гладкой мускулатуры. Возможно также развитие переходных форм ДБСТ (overlaps-синдромы), выражающихся в сочетании у одного и того же больного признаков красной волчанки, дерматомиозита или склеродермии.



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