II. Pathophysiology

  1. Dendritic Cell disorder

III. Associated Conditions

  1. Letterer-Siwe
  2. Hand-Schuller-Christian Syndrome
  3. Histiocytosis X with Bone involvement

IV. Epidemiology

  1. Ages 20-40 years old
  2. Prior Tobacco use (90%)

VI. Complications

  1. Pneumothorax (10-30%)
  2. Diabetes Insipidus
  3. Lytic bone lesions (5-10%)

VII. Management

  1. None available

VIII. Coarse

  1. Unpredictable prognosis
  2. Mortality 2-6%

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