Erdheim-Chester disease and radiobiphosphonates bone scan: from a simple peripheral form to a rare axial location: A case report.

The Erdheim-Chester disease is a rare form of non Langherans histiocytosis. It can be expressed by a multi systemic disease interesting: bone, lung, heart, brain, retro peritoneum, skin and retro-orbital tissue. The diagnosis is established under the radiological aspect, bone scan uptake and immunohistological profile. The bone lesions are almost constant and rather characteristic of bone scintigraphy. We related the case of a young female patient of 26 years old with Erdheim-Chester bone disease. Diagnosis was revealed by diabetes insipidus (nerve damage) and diffuses bones pain. Histological confrontation was obtained on a biopsy, guided by abnormalities described in bone scan. The evolution under interferon was marked by clinical and scintigraphic worsening. Through this clinical observation we will discuss the interest of bone scintigraphy exploration in the monitoring of this disease under a therapy most often not codified.