Langerhans cell histiocytosis

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Revision as of 17:02, 17 May 2023 by Aidan (talk | contribs) (Created page with "== Background == * Rare inflammatory or neoplastic process that can effect a number of organs, characterized by the granulomatous inflammation involving Langerhans cells (denfritic antigen-presenting cells) * Most common under 15 years of age, but can present at any age == Clinical Manifestations == * Rash is most common feature, with general appearance similar to seborrheic dermatitis or atopic dermatitis (in children) ** Scaly papules, nodules, plaques ** Ma...")
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Background

  • Rare inflammatory or neoplastic process that can effect a number of organs, characterized by the granulomatous inflammation involving Langerhans cells (denfritic antigen-presenting cells)
  • Most common under 15 years of age, but can present at any age

Clinical Manifestations

  • Rash is most common feature, with general appearance similar to seborrheic dermatitis or atopic dermatitis (in children)
    • Scaly papules, nodules, plaques
    • May have petechiae, bloody crusting, or indurated nodules
  • Bony involvement happens in most patients (80%)
    • Usually incidental
    • Usually solitary
    • Can involve skull, hip, pelvis, femur, or ribs
  • Lung involvement is less common (20%)
  • Lymphadenopathy in 30%
  • Possibly hepatosplenomegaly
  • Possibly infiltration of pituitary causing diabetes insipidus, with polyuria, polydipsia, and hypernatremia