Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder with no actual known incidence rate. It primarily affects multiple organs via increased expression of proinflammatory cytokines, eventually leading to histiocyte activation and infiltration at multiple sites. A 31-year-old virgin female presented with progressive generalized bone pain and blurred vision. She underwent a comprehensive clinical and paraclinical evaluation. Based on the final results, the diagnosis of ECD was established. We started treatment mainly using Interferon-α and corticosteroids, estrogen, levothyroxine, and desmopressin acetate. In adults with a mysterious chronic disease affecting multiple organs, we should always consider ECD as a differential diagnosis. Although ECD is still rare, the detection rate of this disease has increased significantly in recent decades. A comprehensive clinical and paraclinical evaluation is necessary to make a definitive diagnosis and determine the extent of the disease.
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