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- W2009978995 abstract "Erdheim-Chester disease is a rare, life threatening lipoid granulomatosis1 with fewer than 100 cases described in the world literature. The disease typically affects the long bones and symmetrical sclerosis of the diaphyseal and metaphyseal regions is pathognomonic. Extraskeletal manifestations may affect the lungs, pericardium, aorta, retroperitoneum, skin, and orbits and diabetes insipidus occurs in approximately 30% of cases. Erdheim-Chester disease is characterised microscopically by an infiltrate of lipid laden foamy macrophages (histiocytes), scattered Touton giant cells, chronic inflammatory cells, and fibrosis. The foamy macrophages can be distinguished from Langerhans cells on the basis of negative results on staining for S-100 protein and CD1a. Treatment of the disease has been on an ad hoc basis and no treatment regimen has been shown to be clearly superior.This study documents the clinical findings in a patient with Erdheim–Chester disease, investigates the pathogenesis, and provides a rational basis for effective treatment.This white man, aged 45, developed aching in his legs, night sweats, lethargy, and impotence in October 1988, for which no cause was found. His night sweats resolved by July 1989 and he was discharged. He presented in November 1990 with reduced vision (6/9) in the left eye, bilateral proptosis of 12 months’ duration, chemosis, ophthalmoplegia, and optic disc oedema. He still had sexual dysfunction and lethargy and now also had leg oedema and thrombocythaemia. At that time his thyroid function was normal, but erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) were moderately elevated. A computed tomography (CT) scan of the orbits showed bilaterally enhancing masses lying predominantly within the muscle cone and encasing both optic nerves. An orbital biopsy in November 1990 showed an inflammatory picture. There was no evidence of vasculitis on muscle biopsy and a clinical diagnosis of orbital pseudotumour was made. He was initially treated …" @default.
- W2009978995 created "2016-06-24" @default.
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- W2009978995 date "2004-06-01" @default.
- W2009978995 modified "2023-10-18" @default.
- W2009978995 title "Treatment of Erdheim-Chester disease with cladribine: a rational approach" @default.
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- W2009978995 doi "https://doi.org/10.1136/bjo.2003.035584" @default.
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