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- Abnormal proliferation of Langerhans cells which are a kind of macrophage/ dendritic cell
- aka Langerhans Cell Histocytosis
- Known as a "great mimicker"
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- Eosinophilic Granuloma = Mono-ostotic disease
- Polyostotic EG
- Polyostotic EG with Visceral involvement = Hand Schuller Christian (HSC) Disease.
- Triad of skull lytic lesion, Diabetes Insipidus, Exopthalmos
- Letter-Siwe Disease (LSD) - fatal form
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- Sporadic
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- A great mimicker
- Endosteal scalloping
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- Skeletal survey to look for polyostotic involvement - Skull most common location (50%)
- Skull - punched out lytic lesions
- Spine - vertebral plana - most common cause of Vertebral plana in children
- Long bones - endosteal scalloping
- Renal panel to look for diabetes insipidus
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- EG (solitatory type) is a self-limiting condition - observation
- Chemotherapy required for systemic condition (HSC)
- Surgery if impending fracture - intralesional curettage and bone grafting