Erdheim-Chester disease: case with chronic discharging sinus from bone

Dee, Paul M.; Westgaard, T; Langholm, Ruth

doi:10.2214/ajr.134.4.837

Cited by 17 publications

(5 citation statements)

References 1 publication

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“…15 The metaphyses of long tubular bones followed by those of the ribs are most commonly involved. 5,10,20,24 The type of bone activity varies from osteoclastic to osteoblastic patterns 16 and, occasionally, patients may be misdiagnosed as having Paget's disease. 12 Magnetic resonance imaging is of enormous value in diagnosing ECD.…”

Section: Radiological Investigationmentioning

confidence: 99%

Erdheim—Chester disease of the central nervous system

Babu¹,

Lansen²,

Chadburn³

et al. 1997

Journal of Neurosurgery

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The authors report two cases of Erdheim-Chester disease (ECD), an illness of unknown pathogenesis. Generally, this disease process involves the metaphyseal and diaphyseal portions of the long bones, the lungs, and the retroperitoneum; however, other tissues may be involved including the central nervous system (CNS). To date only two cases of CNS-related ECD have been reported. The present report adds to the literature by documenting two more recent cases of ECD involving the CNS. The clinical presentations of these cases, their radiological findings with special reference to magnetic resonance imaging, pathological determination, and clinical management are briefly reviewed.

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Section: Radiological Investigationmentioning

confidence: 99%

Erdheim—Chester disease of the central nervous system

Babu¹,

Lansen²,

Chadburn³

et al. 1997

Journal of Neurosurgery

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“…Erdheim-Chester disease is a very rare systemic progressively deteriorating lipoxanthogranulomatous infiltrative disorder of variable prognosis. Since the first description by William Chester and Jacob Erdheim in 1930 [1], about 60 cases based on the characteristic radiologic findings have been reported in the world literatures [6][7][8][9][10][11][12][13][14][15]. The pathologic descriptions mostly emphasized the presence of "lipid-laden" macrophages or histiocytes, or of a xanthogranulomatous infiltrative process [3,4,[16][17][18][19][20]].…”

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester Disease with Extensive Marrow Necrosis

Kim¹,

Choe

et al. 2001

Int J Surg Pathol

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Erdheim-Chester disease is a rare systemic disorder characterized by a fibrosing xanthogranulomatous infiltration of multiple organs. We report a case of Erdheim-Chester disease with diffuse necrosis leading to difficulty in making a prompt diagnosis. Radiologically, osteosclerotic lesions with osteolytic element involved metadiaphyses of both proximal tibia, and retroperitoneal infiltrations encasing both kidneys, both adrenals, and aorta were found. A biopsy of the tibia showed diffuse infiltration of foamy histiocytes, Touton-type giant cells, and fibroblastic cells associated with extensive coagulative necrosis. Immunohistochemically, foamy histiocytes were positive for CD68 and peanut agglutinin and negative for S-100 protein. A few Langerhans' cells, which were difficult to identify in hematoxylin-eosin stain, were highlighted by immunostain for S-100 protein. The patient received supportive therapy and was alive 1 1/2 years after diagnosis, with newly developed bilateral retrobulbar lesions and worsened heart failure.

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“…Moreover, the predominance of lipid‐laden histiocytes or foam cells in the patient's retroperitoneal tissues is diagnostic of ECD. It should be noted that whilst several authors believe there is little difference between ECD and forms of histiocytosis X, others maintain that these are distinct pathological processes [1, 7, 8, 17, 21]. Specifically, Hand–Schuller–Christian disease is characterized by proliferation of mature histiocytes and eosinophils, which commonly stain positively for the S‐100 antigen and usually contain Birbeck granules on electron microscopy.…”

Section: Discussionmentioning

confidence: 99%

“…First described in 1930 [1], Erdheim–Chester disease (ECD) appears to be rare, with only 60 cases in the published literature [2–24]. Even though the pathologic hallmark of ECD is infiltration of a wide variety of tissues by cells of macrophage and histiocyte lineage, it appears to be distinct from histiocytosis X [1].…”

Section: Introductionmentioning

confidence: 99%

Endocrine manifestations of Erdheim–Chester disease (a distinct form of histiocytosis)

Tritos

Weinrib

Kaye

1998

Journal of Internal Medicine

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). Endocrine manifestations of Erdheim-Chester disease (a distinct form of histiocytosis) (Case Report). J Intern Med 1998; 244: 529-35.Erdheim-Chester disease (ECD) is a disorder of unclear aetiology, characterized by exuberant histiocyte proliferation and a variable clinical course. We report the case of a woman with multiorgan involvement secondary to ECD. Central diabetes insipidus (CDI), hyperprolactinaemia, gonadotropin insufficiency and decreased insulinlike growth factor I levels were present, suggesting hypothalamic-pituitary dysfunction. The highintensity signal of the posterior pituitary on T1-weighted images was absent on magnetic resonance imaging, but no sellar mass lesions or stalk thickening were apparent. Additionally, our patient had bilateral adrenal enlargement. Even though ECD is a rare cause of neuroendocrine dysfunction or adrenal enlargement, it should be considered in patients with these disorders in the setting of multiorgan disease.

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Erdheim-Chester disease: case with chronic discharging sinus from bone

Cited by 17 publications

References 1 publication

Erdheim—Chester disease of the central nervous system

Erdheim—Chester disease of the central nervous system

Erdheim-Chester Disease with Extensive Marrow Necrosis

Endocrine manifestations of Erdheim–Chester disease (a distinct form of histiocytosis)

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