Systemic histiocytic disorders of adults: ECD, LCH, and RDD
| . | ECD . | LCH . | RDD . |
|---|---|---|---|
| Histopathologic features | |||
| CD68 | + | + | + |
| CD163 | + | + | + |
| CD1a | − | + | − |
| CD207 | − | + | − |
| S100 | − or weakly + | + | + |
| Factor XIIIa | + | − | − |
| Touton giant cells | + | − | − |
| Other characteristic features of lesional histiocytes | Xanthomatous features | Birbeck granules on electron microscopy | Intracytoplasmic lymphocytes (emperipolesis) |
| Fibrosis | |||
| Organ system involvement | |||
| Skin24,95 | Xanthelasma | Scaly erythematous patches | Firm, indurated papules |
| Yellow or red-brown plaques | |||
| Heart15 | Pericardial effusion | Reported, but uncharacteristic | Reported, but uncharacteristic |
| Myocardial infiltration, right atrial mass | |||
| Periaortic sheathing (“coated aorta”) | |||
| Lungs43,96 | Interlobular septal thickening, ground-glass or centrilobular opacities on CT | Nodular and cystic changes in upper and middle lobes | Reported, but uncharacteristic |
| Retroperitoneum | Perinephric infiltration | Reported, but uncharacteristic | Reported, but uncharacteristic |
| Liver and spleen | Rare | Uncommon but constitutes high-risk disease | Reported, but uncharacteristic |
| Bone33 | Femurs and tibia | Craniofacial bones, proximal limbs, pelvis, scapula | Reported, but uncharacteristic |
| Bone pain | |||
| Lymph nodes | Reported, but uncharacteristic | Uncommon but constitutes high-risk disease | Cervical LN, axillary, inguinal, paraaortic, or mediastinal |
| CNS48,97,98 | Cerebellar or brain stem lesions | Cerebellar or brain stem lesions | Dural-based lesions |
| Dural-based lesions | Dural-based lesions | ||
| Brain parenchymal lesions | Brain parenchymal lesions | ||
| Noninfiltrative neuro-degeneration |
| . | ECD . | LCH . | RDD . |
|---|---|---|---|
| Histopathologic features | |||
| CD68 | + | + | + |
| CD163 | + | + | + |
| CD1a | − | + | − |
| CD207 | − | + | − |
| S100 | − or weakly + | + | + |
| Factor XIIIa | + | − | − |
| Touton giant cells | + | − | − |
| Other characteristic features of lesional histiocytes | Xanthomatous features | Birbeck granules on electron microscopy | Intracytoplasmic lymphocytes (emperipolesis) |
| Fibrosis | |||
| Organ system involvement | |||
| Skin24,95 | Xanthelasma | Scaly erythematous patches | Firm, indurated papules |
| Yellow or red-brown plaques | |||
| Heart15 | Pericardial effusion | Reported, but uncharacteristic | Reported, but uncharacteristic |
| Myocardial infiltration, right atrial mass | |||
| Periaortic sheathing (“coated aorta”) | |||
| Lungs43,96 | Interlobular septal thickening, ground-glass or centrilobular opacities on CT | Nodular and cystic changes in upper and middle lobes | Reported, but uncharacteristic |
| Retroperitoneum | Perinephric infiltration | Reported, but uncharacteristic | Reported, but uncharacteristic |
| Liver and spleen | Rare | Uncommon but constitutes high-risk disease | Reported, but uncharacteristic |
| Bone33 | Femurs and tibia | Craniofacial bones, proximal limbs, pelvis, scapula | Reported, but uncharacteristic |
| Bone pain | |||
| Lymph nodes | Reported, but uncharacteristic | Uncommon but constitutes high-risk disease | Cervical LN, axillary, inguinal, paraaortic, or mediastinal |
| CNS48,97,98 | Cerebellar or brain stem lesions | Cerebellar or brain stem lesions | Dural-based lesions |
| Dural-based lesions | Dural-based lesions | ||
| Brain parenchymal lesions | Brain parenchymal lesions | ||
| Noninfiltrative neuro-degeneration |
Highly disease-specific findings are presented in bold.