• Erdheim-Chester disease

Breuil et al reported on a patient with Erdheim-Chester disease.¹ We would like to add our experience in a patient who had a good response to steroids. A 46 year old housewife, born in Maceio (Brazil), complained of pain in her legs for a period of eight months, which interfered with her sleep; there were no constitutional symptoms, such as weight loss, asthenia, or fever. On physical examination, there was no systemic involvement or bone inflammation, except for tenderness on palpation of both tibiae. An x ray examination showed bilateral and symmetrical cortical osteosclerosis of the diaphysial regions of the tibiae, which was better illustrated with magnetic resonance imaging (MRI) which showed a low signal on T₁ and a high signal on T₂ of the medullar canal (fig 1). Bone scintigraphy showed intense hypercapitation at the upper and medial regions of the right tibia and the medial region of the left tibia (fig 2). Other MR images (brain, thorax, and abdomen) were normal. A bone biopsy showed only osteosclerosis, which has been described in this disease.²

She was treated with steroids for six months (prednisone 20 mg/day) and responded well, being asymptomatic and receiving no treatment since December 2000. As bone pain is the first symptom of this xanthogranulomatosis, rheumatologists should be able to recognise the disease in an early phase when treatment gives a better outcome.

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Figure 1

MRI showing a low signal on T₁ and high signal on T₂ of the medullar canal.

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Figure 2

Bone scintigraphy with intense hypercapitation of the tibias.