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Erdheim-Chester syndrome

Erdheim-Chester syndrome Causes

The cause of this disease cannot be directly identified as of now.

Erdheim-Chester syndrome Definition

Erdheim-Chester disease is also known as polyostotic sclerosing histiocytosis or the Erdheim-Chester syndrome. This condition is a relatively rare form of non-Langerhans-cell histiocytosis. Usually, this condition affects people at their in middle age years. It usually involves infiltration of lipid-laden macrophages, giant cells which are multinucleated, an infiltrate of lymphocytes which are usually swelling, and histiocytes located in the bone marrow, and sclerosis of the long bones.

Erdheim-Chester syndrome Diagnosis

Erdheim-Chester syndrome are diagnosed mainly by a radiologic osteosclerosis and histology. Coming up with an accurate diagnosis are often difficult because it is very rare and because of the required differentiation of the condition from other disorders. Diagnosis done from a neurological imaging is often not definitive. Symmetrical cerebellar and pontine signal changes which are present on a T2-weighted image seem typical of Erdheim-Chester disease, but multiple sclerosis and some diseases of metabolism must be taken into consideration in using the differential diagnosis. Biopsies can also be done as a diagnostic test for this disease.

Erdheim-Chester syndrome Symptoms and Signs

Patients with Erdheim-Chester syndrome manifests symptoms like bone pain, retroperitoneal fibrosis, diabetes insipidus, exophthalmos, xanthomas, neurological signs, and Dyspnea which is caused by thickening of the interlobular septal and pleural tissues.

Erdheim-Chester syndrome Treatment

There are various treatment options to take to treat Erdheim-Chester syndrome. It includes surgical debulking, administration of high dose of corticosteroids, cycloporine, interferon, chemotherapy, and radiation therapy.

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