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...")
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