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Erdheim-Chester disease associated with retroperitoneal xanthogranuloma

1979; Wiley; Volume: 52; Issue: 615 Linguagem: Inglês

10.1259/0007-1285-52-615-232

1748-880X

F G Simpson, Philip Robinson, G. J. Hardy, M S Losowsky,

Myeloproliferative Neoplasms: Diagnosis and Treatment

In 1930 Chester, working with the Viennese pathologist Erdheim, described two patients with an unusual lipidosis showing distinctive bone changes. Sorensen (1964) described one patient with similar radiographic appearances and bone histology, and a fourth case was added by Jaffe (1972) who designated the condition “Erdheim-Chester disease”. Histology showed granulomata containing lipidladen histiocytes but the bone lesions differed from those seen in Hand-Schuller-Christian disease both in distribution and in the radiographic appearance. Similar histological appearances occur in retroperitoneal xanthogranuloma (Oberling 1935), but of the 36 cases of this condition described up to 1976, in only one (Melicow 1953) were bone lesions mentioned. We here report a further patient with Erdheim-Chester disease in whom autopsy revealed retroperitoneal xanthogranuloma, suggesting a common underlying pathology for these two rare conditions. A 62-year-old male caucasian presented with a two week history of colicky lower abdominal pain, watery diarrhoea, anorexia, weight loss and hesitancy of micturition. There were no symptoms referable to his skeletal system. Examination showed only pallor and smooth enlargement of the liver. Haemoglobin was 8.8g/dl, ESR55mm/1hr (Westergren), blood urea 13 mmol/1, serum lipids and thyroid function tests were normal. Small bowel enema and sigmoidoscopy were normal. Barium enema showed tethering of the sigmoid loop in the right iliac fossa and distal diverticular disease. IVU showed bilateral hydronephrosis and hydroureter extending to the bladder, but no ureteric displacement.