About: Chronic multifocal Langerhans cell histiocytosis     Goto   Sponge   NotDistinct   Permalink

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Chronic multifocal Langerhans cell histiocytosis, previously known as Hand–Schüller–Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs. The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone (lytic bone lesions often in the skull), and diabetes insipidus (excessive thirst and passing urine), although around 75% of cases do not have all three features. Other features may include a fever and weight loss, and depending on the organs involved there maybe rashes, asymmetry of the face, ear infections, signs in the mouth and the appearance of advanced gum disease. Features relating to lung and liver disease may occur.

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rdfs:label
  • Chronic multifocal Langerhans cell histiocytosis (en)
  • داء هاند-شولر-كريستيان (ar)
  • Болезнь Хенда — Шюллера — Крисчена (ru)
rdfs:comment
  • Болезнь Хенда — Шюллера — Крисчена или липидный гранулёматоз — это генерализованная форма гистиоцитоза X. Характеризуется хроническим волнообразным течением. (ru)
  • داء كثرة المنسجات لخلايا لانغرهانس متعدد البؤر المزمن (بالإنجليزية:Hand-Schüller-Christian Disease) المعروف سابقًا باسم داء هاند شولر كريستيان، نمط من داء كثرة المنسجات لخلايا لانغرهانس، يصيب أعضاء متعددة. تترافق الحالة تقليديًا باجتماع المميزات التالية: انتفاخ العينين، تهدم العظم (الآفات الانحلالية في العظام)، السكري الكاذب (العطش والتبول الزائدين)، 75% من حالاته لا تبدي هذه المميزات الثلاثة معًا. تتضمن الأعراض الأخرى الحمى وخسارة الوزن، ووفقًا للعضو المتأثر يمكن أن تظهر الاندفاعات الجلدية أو عدم تناظر الوجه أو العدوى الأذنية أو العلامات الفموية أو مظهر أمراض دواعم السن. ويمكن أن تحدث أعراض وعلامات مرتبطة بإصابة الكبد أو الرئتين. (ar)
  • Chronic multifocal Langerhans cell histiocytosis, previously known as Hand–Schüller–Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs. The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone (lytic bone lesions often in the skull), and diabetes insipidus (excessive thirst and passing urine), although around 75% of cases do not have all three features. Other features may include a fever and weight loss, and depending on the organs involved there maybe rashes, asymmetry of the face, ear infections, signs in the mouth and the appearance of advanced gum disease. Features relating to lung and liver disease may occur. (en)
foaf:name
  • Chronic multifocal Langerhans cell histiocytosis (en)
name
  • Chronic multifocal Langerhans cell histiocytosis (en)
foaf:depiction
  • http://commons.wikimedia.org/wiki/Special:FilePath/Hans-Schuller-Christian.jpg
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diagnosis
DiseasesDB
ICD
MeshID
  • D006646 (en)
onset
  • Age 2-6 (en)
symptoms
  • *Triad of bulging eyes, breakdown of bone, diabetes insipidus * Other symptoms eg. bone pain, facial asymmetry, ear infections, teeth/gum problems, liver and lung disease signs. (en)
treatment
  • Surgery, chemotherapy, radiation therapy (en)
caption
  • A child with Hand-Schüller-Christian Disease (en)
causes
  • Genetic mutation in the MAPKinase pathway (en)
field
  • Dermatology (en)
frequency
  • Rare (en)
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