Erdheim–Chester disease presenting as malignant exophthalmos

Rozenberg, I.; Wechsler, Janine; Koenig, F; Brun, Bernard; Lardé, D; Raulo, Y; Hazard, J; Pinaudeau, Y; Coscas, G.

doi:10.1259/0007-1285-59-698-173

Cited by 36 publications

(12 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…With regard to the extent of peri-aortic disease, our case seems to be unusual. Orbital infiltration manifested as a retrobulbar tumor with possible contrast enhancement and was bilateral in all eight cases where it was documented [9,31,33,34]. In a patient with a cerebral lesion contrast enhancement on MR scan over a period of 8 days has been described [37].…”

Section: Discussionmentioning

confidence: 98%

Erdheim-Chester disease: a case report and literature overview

et al. 1999

View full text Add to dashboard Cite

Erdheim-Chester (EC) disease belongs to the group of lipoid granulomatosis. Symmetric sclerosis of the meta- and diaphysis of long tubular bones are pathognomonic radiologic changes. Additionally, other skeletal segments can be affected. Extraskeletal manifestations can occur in almost all organs; lungs, pericardium, retroperitoneum, skin, and orbita play particularly important roles. The last case of 38 cases of Erdheim-Chester disease with an extraordinary mediastinal and perirenal involvement is described. For the second time following the initial description by Chester, an axial skeletal pattern of eburnated vertebra is shown.

show abstract

Section: Discussionmentioning

confidence: 98%

Erdheim-Chester disease: a case report and literature overview

et al. 1999

View full text Add to dashboard Cite

show abstract

“…Death frequently has a cardiovascular cause (heart failure, valvular pathology, renovascular hypertension or pericarditis [11]). Recently, a variant of ECD characterized by fulminant multisystem cell histiocytic proliferation in which death occurred within 3 months of presentation has been reported [16].…”

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester disease as cause of end-stage renal failure: a case report and review of the literature

et al. 2010

View full text Add to dashboard Cite

“…[1][2][3] Steroid therapy has been reported to improve exophthalmos, 6 7 renal and bone involvement 7 and, in combination with vinblastine plus adriamycin or cyclophosphamide, exophthalmos, renal and retroperitoneal disease. 8 Radiotherapy transiently relieves bone pain but not exophthalmos. Improvement in respiratory symptoms has been described with steroids alone 2 9 and in combination with cyclophosphamide and etoposide, 3 but no previous reports have shown objective improvement.…”

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone

Bourke

Nicholson²,

Gibson³

2003

Thorax

View full text Add to dashboard Cite

Erdheim-Chester disease is a rare multisystem histiocytic disorder in which death most commonly results from pulmonary or cardiac involvement. We report the case history of a 55 year old man with this condition with extensive and progressive pulmonary disease. He had no response to prednisolone alone, but treatment with prednisolone plus cyclophosphamide was associated with a rapid improvement in symptoms, lung function, and the chest radiographic appearance. He subsequently showed a symptomatic, functional, and radiological deterioration, followed by a marked slowing in the rate of decline. He currently remains stable 41 months after treatment was initiated. This is the first report of pulmonary Erdheim-Chester disease showing improvement in both lung function and symptoms with any form of treatment.

show abstract

Erdheim–Chester disease presenting as malignant exophthalmos

Cited by 36 publications

References 15 publications

Erdheim-Chester disease: a case report and literature overview

Erdheim-Chester disease: a case report and literature overview

Erdheim-Chester disease as cause of end-stage renal failure: a case report and review of the literature

Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone

Contact Info

Product

Resources

About