Hematology and Oncology Book

Hand Disorders

  • Hand-Schuller-Christian Syndrome

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Hand-Schuller-Christian SyndromeAka: Histiocytosis X, Lipoid Histiocytosis

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  1. See Also
    1. Langerhans Cell Granulomatosis
  2. Symptoms
    1. Sore Oral Mucosa
    2. Skin Eruption
  3. Signs
    1. Classic Triad (10%)
      1. Lytic bone lesions (esp. Skull defects)
      2. Diabetes Insipidus
      3. Exophthalmos
    2. Oral Changes
      1. Gum swelling and necrosis
      2. Extrusion of teeth
    3. Rash
      1. Papular, seborrheic or petechial rash
      2. Minute xanthomatous Nodules
      3. Raised yellow to brown lesions in neck and axilla
    4. Growth retardation
    5. Developmental delay
    6. Lung changes
      1. See Pulmonary Histiocytosis X
  4. Labs
    1. Complete Blood Count
      1. Hemoglobin or Hematocrit consistent with Anemia
      2. White Blood Cell Count consistent with Leukopenia
      3. Platelet Count consistent with Thrombocytopenia
    2. Chemistry panel and Serum osmolarity
      1. Diabetes Insipidus changes
  5. Diagnosis
    1. Skin biopsy
    2. Bone Marrow Biopsy

Histiocytosis, Langerhans-Cell (C0019621)

Definition (MSH)Group of disorders characterized by monoclonal proliferation of CD1+ HISTIOCYTES. The demonstration of the LANGERHANS CELLS Birbeck granules is the gold standard of the phenotype. EOSINOPHILIC GRANULOMA makes up 60-80% of all the cases.
Definition (CSP)group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; and eosinophilic granuloma; Langerhans cells are components of the lesions.
Definition (NCI)A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001) -- 2003
ConceptsDisease or Syndrome (T047)
ICD9277.89
MSHD006646
EnglishDifferentiated progressive histiocytosis, Generalized Histiocytoses, Generalized Histiocytosis, Hand Schueller Christian Syndrome, Hand-Schueller-Christian Syndrome, Histiocytosis X, Histiocytosis X syndrome, Histiocytosis-X, Langerhan's cell histiocytosis, Langerhans cell disease, Langerhans cell granulomatosis, LANGERHANS CELL HISTIOCYTOSIS, Langerhans histiocytic syndrome, Langerhans' cell histiocytosis, Langerhans-Cell Granulomatosis, Langerhans-Cell Histiocytosis, LCH, LCH - Langerhan's cell histiocytosis, LETTERER SIWE DIS, Letterer Siwe Disease, Letterer-Siwe Disease, SCHUELLER CHRISTIAN DIS, Schueller Christian Disease, Schueller-Christian Disease
Spanishenfermedad de celulas de Langerhans, enfermedad de las celulas de Langerhans, granulomatosis de celulas de Langerhans, histiocitosis de celulas de Langerhans, histiocitosis progresiva diferenciada, histiocitosis X, sindrome de histiocitosis X, sindrome histiocitico de Langerhans
Parent ConceptsMetabolic Diseases (C0025517), Histiocytosis (C0019618), Lung Diseases, Interstitial (C0206062), Histiocytic and Dendritic Cell Neoplasms (C1334030), Malignant Childhood Neoplasm (C0278704), Histiocytosis, Langerhans-Cell (C0019621), Histiocytic syndrome (C0398597), Duplicate concept (C1274013), Langerhans cell histiocytosis - category (C1444010), Neoplasm of hematopoietic cell type (C1532168)
SourcesCSP, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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