Comparison between the CNS manifestations of the common histiocytic disorders
| Erdheim-Chester Disease | Langerhans Cell Histiocytosis | Rosai-Dorfman Disease | |
|---|---|---|---|
| Demographics | M > F, sixth decade of life | M > F, first 2 decades of life | M > F, second to third decade of life |
| BRAFV00E mutation | Positive (more than LCD) | Positive | N/A |
| Histopathology | Foamy histiocytes, CD68+, CD1a- | Mononucleated dendritic cells, CD1a+, Birbeck granules | Multinucleated histiocytes, CD68+, S-100+, CD1a-; may be associated with immunoglobulin G4-related disease |
| Neurodegeneration and atrophy | + | ++ | + |
| Intraparenchymal lesions | Present (often multiple, more edema) | Present (often solitary, lesser edema) | Present (often multiple, more edema) |
| HPA involvement | + | +++ | Rare |
| Extra-axial lesions | ++ | + | + |
| Craniofacial involvement | Sclerotic lesions (calvarial involvement is less common) | Lytic lesions (calvarial involvement is more common) | Rare |
| Orbital involvement | ++ | + | + |
| Vascular involvement | + | − | − |
Note:—M indicates male; F, female; N/A, not applicable.