Table 2
Differential diagnosis of Erdheim-Chester disease
| Diagnosis | Affected organs | Clinical symptoms | Pathology |
|---|---|---|---|
| CNS, central nervous system; GIT, gastrointestinal tract. | |||
| Erdheim-Chester disease | Bones of lower extremities, CNS, retroperitoneum, lung, heart, liver, spleen, skin, orbit | Bone pain, fever, weight loss, weakness, exophthalmos, diabetes insipidus,dysuria, abdominal pain, ataxia, paresis | Mononuclear cells, foamy histiocytes (CD68+, CD1a−, S-100−), lymphocytes, Touton giant cells, fibrosis |
| Neurosarcoidosis | Pituitary gland, mostly associated with enlarged hilary lymph nodes | Diabetes insipidus, adenopituitary failure, amenorrhoea/galactorrhoea syndrome | Epitheloid granulomas with Langhans’ giant cells and lymphocytes |
| Langerhans’ cell histiocytosis | Skin, bones, lymph nodes, ear,gum, lung, GIT, liver, CNS | Petechial haemorrhages, erosive and ulcerative lesions, lymphadenopathy,bone pain, fractures, dyspnoea, malabsorption, diabetes insipidus | Proliferation of Langerhans cells (CD1a+, S-100+), intracytoplasmic Birbeck granules on electron microscopy |
| Hypophysitis | Adenohypophysis (rarely neurohypophysis) | Headache, diabetes insipidus, amenorrhoea/galactorrhoea syndrome | Common finding: lymphoplasmocytic infiltration with focal or diffuse destruction |
| Lymphocytic | |||
| Granulomatous | |||
| Xanthomatous | |||
| Xanthogranulomatous | |||
| Necrotising | |||
