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},
"code": "L51",
"name": "Эритема многоформная",
"icd_name": "Эритема многоформная",
"gender": 0,
"age_min": 0,
"age_max": 100,
"cause": [
3,
0
],
"periodicity": 1,
"slug": "l51_eritema_mnogoformnaya",
"lead": "острое заболевание кожи и слизистых с полиморфными высыпаниями и наклонностью к рецидивам преимущественно в осенний и весенний периоды. ",
"description": "Многоформная экссудативная эритема возникает в основном у молодых людей и лиц среднего возраста.",
"etiology": "<p>К настоящему моменту не удалось выявить точные причины инфекционно-аллергической формы заболевания. Существует большая вероятность того, что причиной экссудативной эритемы является очаговая инфекция. Это может быть хронический аппендицит, пульпит, гайморит и пр. Этот фактор выявляется примерно у 70% всех заболевших. У таких больных обнаруживается высокая сенсибилизация (чувствительность) к различным инфекционным агентам – кишечной палочке, стафилококку и пр. Во время рецидива экссудативной эритемы у больных отмечается снижение устойчивости организма, понижение Т-клеточного иммунитета. Таким образом, основной причиной развития данной формы многоформной эритемы является развитие иммунодефицитного состояния, которое, в свою очередь, провоцируется: хронической инфекцией; переохлаждением; заражением вирусной инфекцией; гелио-магнитными воздействиями. Причиной появления токсико-аллергической разновидности экссудативной эритемы является прием лекарственных средств. Чаще всего, подобная реакция организма наблюдается на прием барбитуратов, препаратов тетрациклинового ряда, сульфаниламидов.<br /><br /></p>",
"pathogenesis": "",
"diagnostics": "<p align=\"justify\">Для диагностики заболевания на консультации дерматолога проводится тщательный осмотр высыпаний и дерматоскопия. При сборе анамнеза уделяется внимание связи с инфекционным процессом или введением лекарственных препаратов. Для подтверждения диагноза многоформной экссудативной экземы и исключения других заболеваний с поверхности пораженных участков кожи и слизистой берут мазки-отпечатки.</p>\r\n<p align=\"justify\">Многоформную экссудативную эритему дифференцируют с пузырчаткой, диссеминированной формой СКВ, узловатой эритемой. Быстрая динамика сыпи, отрицательный симптом Никольского и отсутствие акантолиза в мазках-отпечатках позволяют дифференцировать многоформную экссудативную эритему от пузырчатки.</p>\r\n<p align=\"justify\">При фиксированных формах многоформной экссудативной эритемы необходимо проводить дифференциальную диагностику с сифилитическими папулами. Отсутствие бледных трепонем при исследовании в темном поле, отрицательные реакции ПЦР, РИФ и RРR позволяют исключить сифилис.</p>",
"treatment": "<p align=\"justify\">ечение в остром периоде зависит от клинических проявлений многоформной экссудативной эритемы. При частом рецидивировании, поражении слизистых, диссеминированных высыпаниях на коже, появлении некротических участков в центре элементов сыпи пациенту показано однократное введение 2 мл дипроспана.</p>\r\n<p align=\"justify\">При токсико-аллергической форме первоочередной задачей лечения является определение и выведение из организма вещества, спровоцировавшего возникновение многоформной экссудативной эритемы. Для этого пациенту назначается обильное питье, энтеросорбенты, мочегонные средства. При впервые возникшем случае заболевания или при наличие в анамнезе данных о самостоятельном быстром разрешении его рецидивов введение дипроспана, как правило, не требуется.</p>\r\n<p align=\"justify\">При любой форме многоформной экссудативной эритемы показана дессенсибилизирующая терапия: супрастин, тавегил, тиосульфат натрия и др. Антибиотики применяют только в случае вторичного инфицирования высыпаний.</p>\r\n<p align=\"justify\">Местное лечение многоформной экссудативной эритемы заключается в применении аппликаций из антибиотиков с протеолитическими ферментами, смазывании пораженных участков кожи антисептиками (р-р хлоргексидина или фурацилина) и кортикостероидными мазями, в состав которых входят антибактериальные препараты (триоксазин, дермазолин). При поражении слизистой полости рта назначают полоскание отваром ромашки, ротоканом, смазывание облепиховым маслом.</p>\r\n<p align=\"justify\">Профилактика рецидивов многоформной экссудативной эритемы при инфекционно-аллергической форме тесно связана с выявлением и устранением хронических инфекционных очагов и герпетической инфекции. Для этого пациенту может потребоваться консультация отоларинголога, стоматолога, уролога и других специалистов. При токсико-аллергическом варианте многоформной экссудативной эритеме важно не допускать приема провоцирующего заболевание медикамента.</p>",
"prevention": "<p>Профилактика многоформной эритемы заключается в своевременной санации очагов инфекции, а также, в исключении применения лекарств и особенно антибиотиков без назначения врача.<br /><br /></p>",
"clinical_picture": "<p>Симптомы и проявления эритемы зависят от формы заболевания.</p>\r\n<p>Инфекционно-аллергическая разновидность эритемы может начинаться как грипп. При инфекционно-аллергической разновидности эритемы заболевание, как правило, начинается остро. Первые симптомы могут несколько напоминать грипп, больные экссудативной эритемой начинают ощущать <a href=\"/symptom/utomlyaemost/\" title=\"Перейти на страницу симптома Утомляемость\">недомогание</a>, часто отмечаются боли в мышцах, суставах, першение в горле. Через сутки после начала заболевания появляется <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">сыпь</a>, которая является характерным симптомом экссудативной эритемы. <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">Сыпь</a> появляется на коже и слизистой рта, реже поражаются слизистые половых органов. Слизистые при многоформной эритеме поражаются примерно у третьей части заболевших. Изолированное поражение оболочек рта (без высыпаний на коже) отмечается только у 5% заболевших. Как правило, через несколько суток после появления кожного синдрома общие симптомы проходят, однако, невысокая температура может держаться несколько недель. Для экссудативной эритемы характерно появление на коже пятен или плоских папул, цвет высыпаний ярко-розовый или красный. Пятна быстро увеличиваются в размерах, их центральная часть западает и приобретает синюшный цвет, в то время как периферические края охраняют красную окраску. В некоторых случаях, в центральной части пятен могут появиться пузыри с прозрачным или кровянистым содержимым. Реже отмечается появление пузырей на неизмененной коже. Кожа стоп — это одно из мест локализации многоформной экссудативной эритемы. <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">Высыпания</a> при экссудативной эритеме локализуются на коже стоп, кистей, голеней, предплечий. При поражении и слизистой рта экссудативная <a href=\"/symptom/pokrasnenie_kozhi/\" title=\"Перейти на страницу симптома Покраснение кожи\">эритема</a> имеет более тяжелое течение. <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">Высыпания</a> в этом случае локализуются на губах, небе, щеках. Сначала на слизистых образуется отек, потом появляются пузыри. Вскоре они вскрываются с образованием крайне болезненных эрозий, которые склоны к слиянию в обширную раневую поверхность. <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">Эрозии</a> могут покрываться сероватым налетом, при снятии которого поверхность слизистой начинает кровоточить. Поверхность эрозий покрываются кровянистыми корками разной толщины, а в случае вторичного инфицирования пораженной слизистой корки приобретают серый цвет. У некоторых больных экссудативной эритемой поражение охватывает всю слизистую рта, у других – появляются единичные элементы высыпаний. При значительной площади поражения площади рта возникает сильный болевой синдром. Больные многоформной эритемой не могут нормально открыть рот, это обстоятельство делает невозможной речь и прием пищи. Наличие кариеса, пародонтоза способствует усугублению процесса. При многоформной эритеме <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">высыпания</a>, появившиеся на коже, разрешаются через 2 — 3 недели. <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">Высыпания</a> на слизистых не заживают по 4 — 6 недель. В большинстве случаев, многоформная <a href=\"/symptom/pokrasnenie_kozhi/\" title=\"Перейти на страницу симптома Покраснение кожи\">эритема</a> имеет рецидивирующее течение, обострения чаще случаются в периоды межсезонья. Редко отмечаются тяжелые случаи, при которых рецидивы заболевания следуют один за другим. Симптомы токсико-аллергической эритемы мало отличатся от описанных выше. При этой форме <a href=\"/symptom/syp/\" title=\"Перейти на страницу симптома Сыпь\">высыпания</a> могут носить распространенный характер или быть фиксированными. В первом случае, практически у всех заболевших отмечается поражение слизистой рта.<br /><br /></p>",
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},
"code": "L10",
"name": "Пузырчатка [пемфигус]",
"icd_name": "Пузырчатка [пемфигус]",
"gender": 0,
"age_min": 18,
"age_max": 100,
"cause": [
3,
0
],
"periodicity": 4,
"slug": "l10_puzyrchatka_pemfigus",
"lead": "хроническое заболевание аутоиммунной природы, которое характеризуется появлением пузырей на клинически здоровой коже и слизистых оболочках.",
"description": "Не заразное, крайне тяжелое заболевание кожи.",
"etiology": "<p>Наиболее вероятными причиной пузырчатки являются нарушения аутоиммунных процессов, в результате чего клетки организма становятся для иммунной системы антителами. Нарушение антигенной структуры клеток эпидермиса происходит под воздействием внешних факторов, в частности воздействие ретровирусов и агрессивных условий окружающей среды.</p>",
"pathogenesis": "<p>Повреждающее действие на клетки эпидермиса и выработка специфических антигенов приводит к нарушению связи между клетками в результате чего и образуются пузыри.</p>",
"diagnostics": "<p>Диагностика пузырчатки осуществляется врачом-дерматологом и основана на совокупности результатов осмотра, цитологического, гистологического и иммунологического обследований. При всех формах заболевания отмечают отложение иммуноглобулинов G в поверхностных слоях кожи.</p>",
"treatment": "<p align=\"justify\">Все пациенты больные пузырчаткой должны находиться на диспансерном наблюдении у дерматолога, рекомендован щадящий режим работы, отсутствие физических нагрузок и избегание инсоляции. Частая смена нательного и постельного белья предотвращает присоединение вторичной инфекции.</p>\r\n<p> </p>\r\n<p align=\"justify\">Показано назначение глюкокортикостероидов сразу в высоких дозах, так как иначе терапевтического эффекта достигнуто не будет, после купирования острых проявлений пузырчатки дозировки гормональных препаратов постепенно снижают до минимально эффективных. В лечении пузырчатки применяют методы экстракорпоральной гемокоррекции: гемосорбцию, криоаферез и мембранный плазмаферез. </p>\r\n<p> В качестве местного лечения пузырчатки используют анилиновые красители и неагрессивные антисептические растворы.</p>\r\n<p>Прогноз пузырчатки всегда неблагоприятный, так как в случае отсутствия адекватного лечения смерть пациентов наступает довольно быстро от присоединившихся осложнений. Длительная гормональная терапия в высоких дозах повышает риск возникновения побочных эффектов, но при отказе от глюкокортикостероидов пузырчатка начинает рецидивировать</p>",
"prevention": "",
"clinical_picture": "<p align=\"justify\">Пузырчатка имеет длительное волнообразное течение, и отсутствие адекватного лечения приводит к нарушению общего состояния пациента.</p>\r\n<p> </p>\r\n<p align=\"justify\">При вульгарной форме пузырчатки пузыри локализуются по всему телу, имеют разный размер и наполнены серозным содержимым, при этом покрышка на пузырях вялая и тонкая.</p>\r\n<p> </p>\r\n<p align=\"justify\">Дебютирует вульгарная пузырчатка как правило на слизистой рта и носа, а потому пациенты длительно и безуспешно получают терапию у стоматологов и отоларингологов. На этой стадии пузырчатки пациенты предъявляют жалобы на боль во время приема пищи и при разговоре, гиперсаливацию и специфический неприятный <a href=\"/symptom/zapah_izo_rta/\" title=\"Перейти на страницу симптома Запах изо рта\">запах изо рта</a>. Продолжительность этого периода от трех месяцев до года, после чего пузырчатка принимает распространенный характер и в воспалительный процесс вовлекаются кожные покровы.</p>\r\n<p>Иногда пациенты не замечают наличие пузырей из-за их небольшого размера и тонкой покрышки, пузыри вскрываются быстро, а потому основные жалобы больных пузырчаткой на данном периоде – это болезненные <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">эрозии</a>. Проводится длительная и безуспешная терапия стоматитов, прежде чем диагностируют пузырчатку. Пузыри, которые локализуются на коже, имеют тенденцию к самопроизвольному вскрытию с обнажением эрозированной поверхности и с остатками покрышки, которая ссыхается в корки.</p>\r\n<p> <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">Эрозии</a> при пузырчатке ярко-розовые, с гладкой глянцевой поверхностью, отличаются от эрозий при других заболеваниях склонностью к периферическому росту и к генерализации с формированием обширных очагов поражения. Если пузырчатка принимает такое течение, то общее состояние пациента ухудшается, развивается интоксикация, присоединяется вторичная инфекция и без должного лечения такие пациенты умирают.</p>\r\n<p> При вульгарной пузырчатке синдром Никольского положительный в очаге поражения и порой на здоровой коже - при незначительном механическом воздействии происходит отслойка верхнего слоя эпителия.</p>",
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"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Наиболее вероятными причиной пузырчатки являются нарушения аутоиммунных процессов, в результате чего клетки организма становятся для иммунной системы антителами. Нарушение антигенной структуры клеток эпидермиса происходит под воздействием внешних факторов, в частности воздействие ретровирусов и агрессивных условий окружающей среды.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Повреждающее действие на клетки эпидермиса и выработка специфических антигенов приводит к нарушению связи между клетками в результате чего и образуются пузыри. Факторы риска при пузырчатке не установлены, но у лиц с наследственной предрасположенностью процент заболеваемости выше.</span></p>",
"pathogenesis": "",
"diagnostics": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Клинические проявления особенно на первоначальных этапах болезни малоинформативны, и поэтому опрос пациента позволяет избежать ошибочного диагноза. Лабораторные исследования позволяют заподозрить пузырчатку, так в мазках-отпечатках при цитологическом исследовании обнаруживаются акантолитические клетки. При гистологическом исследовании обнаруживается внутриэпидермальное расположение пузырей.</span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Больные ставятся на диспансерный учёт, они должны избегать чрезмерных физических нагрузок и стрессов, как можно чаще менять одежду и постельное бельё, соблюдать диету и правила гигиены.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Основу лечения составляет медикаментозная терапия:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Высокие дозы глюкокортикоидов (Полькортолон, Метипред, Дексаметазон, Преднизолон). Если наблюдается регресс заболевания, дозировка постепенно уменьшается.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При заболеваниях ЖКТ назначаются пролонгированные глюкокортикоиды – Дипроспан, Метипред-депо, Депо-медрол.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Лечение дополняется гормональными средствами, недостаток которых – высокая вероятность осложнений: депрессии, бессонницы, артериальной гипертензии, повышенной возбудимости, ангиопатии, увеличения веса, тромбоза, язвы (эрозии) желудка (кишечника), стероидного диабета.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При ухудшении состояния назначаются препараты для восстановления слизистой желудка (Альмагель и др.), диета, предполагающая минимум жиров и углеводов, максимум белка и витаминов.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Параллельно принимаются иммуносупрессоры и цитостатики – Азатиоприн, Метотрексат, Сандиммун.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Чтобы избежать нарушения электролитного баланса, рекомендуется приём калия и кальция.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Дополнительные методы лечения:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Очищение крови – гемодиализ, плазмаферез, гемосорбция. Подобные процедуры удаляют из крови иммуноглобулины, токсические вещества и циркулирующие иммунные комплексы. Они особенно полезны пациентам с гипертиреозом, атеросклерозом, сахарным диабетом.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Фотохимиотерапия – воздействие ультрафиолетовых лучей параллельно с G-метоксипсораленом снижает активность клеток крови и направляет её в русла сосудов, очищает организм от токсинов и иммуноглобулинов.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Местное лечение – мази с глюкокортикоидами, спреи с ксилокаином, лидокаином и другими местными анестетиками, растворы с анилиновыми красителями (бриллиантовый зелёный, Фукорцин), ванны с перманганатом калия, обработка язв Куриозоном.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Диета – из рациона исключают продукты, способные вызвать аллергию, грубую пищу, простые углеводы, соль, консервы. В меню вводят продукты с высоким содержанием белка и витаминов. При образовании пузырей в ротовой полости рекомендуются супы-пюре и мягкие каши, исключающие механическое повреждение слизистой оболочки.</span></li>\r\n</ul>\r\n<p> </p>",
"prevention": "<p><span id=\"docs-internal-guid-f64e4608-7fff-1f25-0349-f35a80423c3c\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Специфических мер нет. Профилактика болезни проста и универсальна. Она заключается в строгом соблюдении элементарных принципов гигиены, отсутствии вредных привычек, здоровом питании с высоким содержанием белка и активном, в независимости от возраста, подвижном образе жизни.</span></span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Некоторые симптомы пузырчатки характерны для всех видов и форм. Это стремительный прогресс при отсутствии лечения и волнообразность – болезнь то затихает, то усиливается снова. Другие признаки зависят от вида и формы заболевания:</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Вульгарная (обыкновенная) пузырчатка. Пузыри разного размера распространяются по всему телу. Они имеют вялую и тонкую покрышку (поверхность), внутри заполнены прозрачной или полупрозрачной жидкостью – серозным экссудатом. Чаще всего первые пузырьки появляются на слизистой рта и носа, из-за чего появляется:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Боль при жевательных движениях, глотании и разговоре.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Усиленное слюноотделение.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Боль при высмаркивании.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Неприятный запах изо рта.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Патологический процесс на слизистых затягивается на длительное время – от 3 месяцев до 1 года. Потом волдыри появляются на разных участках тела. Часто это происходит настолько стремительно, что пациент не замечает их образования. Иногда пузырьки лопаются, на их месте образуются эрозии ярко-розового цвета с гладкой, глянцевой поверхностью, вызывающие боль, потом формируются сухие корки. Как правило, они распространяются от центра скопления пузырьков к краям и образуют обширные зоны. При диагностике пузырчатки обыкновенной проба Никольского даёт положительный результат – при незначительном воздействии на кожу в очаге поражения отслаивается верхний слой. Во время болезни ощущается слабость, повышается температура.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Эритематозная пузырчатка. Сначала пузырьки появляются на гpyди, шее, лице и волосистой части головы, симптомы похожи на себорею – чёткие границы зон поражения, быстрое самовскрытие пузырей с вялой и дряблой поверхностью, эрозии, бурые или желтоватые корочки разной толщины на их месте. Синдром Никольского при этом имеет локальный характер, но постепенно затрагивает другие участки тела.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Вегетирующая пузырчатка. Доброкачественное заболевание, которое протекает много лет, не ухудшая самочувствие пациентов. Сначала пузырьки появляются в естественных складках кожи, вокруг рта, носа и ушей, в области пoлoвых органов и aнaльного отверстия. Они самопроизвольно вскрываются, на их месте образуются эрозии с неприятно пахнущим серозно-гнойным или серозным налётом, окружённые пустулами. При постановке диагноза болезнь нужно дифференцировать от хронической вегетирующей пиодермии, проба Никольского даёт положительный результат только в зонах поражения.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Листовидная пузырчатка. Сначала плоские, слегка возвышающиеся над кожей пузыри появляются на теле, потом образуются на слизистых оболочках. Характерный признак – одновременное наличие пузырьков и корочек, наслаивающихся друг на друга.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Бразильская пузырчатка. Встречается только в странах Латинской Америки (Венесуэле, Парагвае, Перу, Боливии, Аргентине и Бразилии и т. д.), на других континентах никогда не выявлялась. Причина возникновения до сих пор не установлена, но скорее всего заболевание имеет инфекционную природу. Чаще бразильская пузырчатка диагностируется у женщин моложе 30 лет, поражает только кожный покров. Сначала появляются плоские пузырьки, потом они вскрываются и покрываются чешуйчатыми расслаивающимися корочками, под которыми образуются эрозии, незаживающие по нескольку лет. Синдром Никольского на поражённых участках даёт положительный результат, болезнь вызывает сильный дискомфорт – сопровождается болью и жжением.</span></p>",
"image": null,
"image_alt": null,
"standard_type": 0,
"danger": 31,
"published": 1,
"parent": 6722,
"block_rubric": 121,
"standards": []
},
{
"id": 6722,
"symptoms": [
{
"id": 3049,
"synonyms": [
{
"name": "водянистый пузырек"
},
{
"name": "Кожная мозоль"
},
{
"name": "Волдыри"
}
],
"body_points": [
{
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"disease_count": 19,
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},
"code": "L10",
"name": "Пузырчатка [пемфигус]",
"icd_name": "Пузырчатка [пемфигус]",
"gender": 0,
"age_min": 18,
"age_max": 100,
"cause": [
3,
0
],
"periodicity": 4,
"slug": "l10_puzyrchatka_pemfigus",
"lead": "хроническое заболевание аутоиммунной природы, которое характеризуется появлением пузырей на клинически здоровой коже и слизистых оболочках.",
"description": "Не заразное, крайне тяжелое заболевание кожи.",
"etiology": "<p>Наиболее вероятными причиной пузырчатки являются нарушения аутоиммунных процессов, в результате чего клетки организма становятся для иммунной системы антителами. Нарушение антигенной структуры клеток эпидермиса происходит под воздействием внешних факторов, в частности воздействие ретровирусов и агрессивных условий окружающей среды.</p>",
"pathogenesis": "<p>Повреждающее действие на клетки эпидермиса и выработка специфических антигенов приводит к нарушению связи между клетками в результате чего и образуются пузыри.</p>",
"diagnostics": "<p>Диагностика пузырчатки осуществляется врачом-дерматологом и основана на совокупности результатов осмотра, цитологического, гистологического и иммунологического обследований. При всех формах заболевания отмечают отложение иммуноглобулинов G в поверхностных слоях кожи.</p>",
"treatment": "<p align=\"justify\">Все пациенты больные пузырчаткой должны находиться на диспансерном наблюдении у дерматолога, рекомендован щадящий режим работы, отсутствие физических нагрузок и избегание инсоляции. Частая смена нательного и постельного белья предотвращает присоединение вторичной инфекции.</p>\r\n<p> </p>\r\n<p align=\"justify\">Показано назначение глюкокортикостероидов сразу в высоких дозах, так как иначе терапевтического эффекта достигнуто не будет, после купирования острых проявлений пузырчатки дозировки гормональных препаратов постепенно снижают до минимально эффективных. В лечении пузырчатки применяют методы экстракорпоральной гемокоррекции: гемосорбцию, криоаферез и мембранный плазмаферез. </p>\r\n<p> В качестве местного лечения пузырчатки используют анилиновые красители и неагрессивные антисептические растворы.</p>\r\n<p>Прогноз пузырчатки всегда неблагоприятный, так как в случае отсутствия адекватного лечения смерть пациентов наступает довольно быстро от присоединившихся осложнений. Длительная гормональная терапия в высоких дозах повышает риск возникновения побочных эффектов, но при отказе от глюкокортикостероидов пузырчатка начинает рецидивировать</p>",
"prevention": "",
"clinical_picture": "<p align=\"justify\">Пузырчатка имеет длительное волнообразное течение, и отсутствие адекватного лечения приводит к нарушению общего состояния пациента.</p>\r\n<p> </p>\r\n<p align=\"justify\">При вульгарной форме пузырчатки пузыри локализуются по всему телу, имеют разный размер и наполнены серозным содержимым, при этом покрышка на пузырях вялая и тонкая.</p>\r\n<p> </p>\r\n<p align=\"justify\">Дебютирует вульгарная пузырчатка как правило на слизистой рта и носа, а потому пациенты длительно и безуспешно получают терапию у стоматологов и отоларингологов. На этой стадии пузырчатки пациенты предъявляют жалобы на боль во время приема пищи и при разговоре, гиперсаливацию и специфический неприятный <a href=\"/symptom/zapah_izo_rta/\" title=\"Перейти на страницу симптома Запах изо рта\">запах изо рта</a>. Продолжительность этого периода от трех месяцев до года, после чего пузырчатка принимает распространенный характер и в воспалительный процесс вовлекаются кожные покровы.</p>\r\n<p>Иногда пациенты не замечают наличие пузырей из-за их небольшого размера и тонкой покрышки, пузыри вскрываются быстро, а потому основные жалобы больных пузырчаткой на данном периоде – это болезненные <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">эрозии</a>. Проводится длительная и безуспешная терапия стоматитов, прежде чем диагностируют пузырчатку. Пузыри, которые локализуются на коже, имеют тенденцию к самопроизвольному вскрытию с обнажением эрозированной поверхности и с остатками покрышки, которая ссыхается в корки.</p>\r\n<p> <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">Эрозии</a> при пузырчатке ярко-розовые, с гладкой глянцевой поверхностью, отличаются от эрозий при других заболеваниях склонностью к периферическому росту и к генерализации с формированием обширных очагов поражения. Если пузырчатка принимает такое течение, то общее состояние пациента ухудшается, развивается интоксикация, присоединяется вторичная инфекция и без должного лечения такие пациенты умирают.</p>\r\n<p> При вульгарной пузырчатке синдром Никольского положительный в очаге поражения и порой на здоровой коже - при незначительном механическом воздействии происходит отслойка верхнего слоя эпителия.</p>",
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