Histiocytosis

Hand-Schuller-Christian Syndrome

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Hand-Schuller-Christian Syndrome, Histiocytosis X, Lipoid Histiocytosis

  • Symptoms
  1. Sore Oral Mucosa
  2. Skin Eruption
  • 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
  • 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
  • Diagnosis
  1. Skin biopsy
  2. Bone Marrow Biopsy