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Results for “Erdheim-Chester Disease”

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At least 19 recordsLinked to original sources

PubMed1990

Premature alveolar bone loss in Erdheim-Chester disease.

Erdheim-Chester disease is a rare histiocytosis also known as lipoid granulomatosis. Oral findings have not been reported previously to our knowledge. This case report documents evidence of oral sequelae of Erdheim-Chester disease. A patient whose course was followed for 10 years at the National Institutes of Health had premature alveolar bone resorption. He underwent full-mouth extraction at age 29 years because of severe periodontitis. Histopathologic evidence of Erdheim-Chester disease was demonstrated in the periodontal soft tissues. In the ensuring years, accelerated resorption of the residual ridges precluded the use of conventional dentures. We recommend early preventive dental management for patients with Erdheim-Chester disease.

Adult↗

PubMed1991

[Erdheim-Chester disease].

Erdheim-Chester disease (ECD) is characterized by lipid granuloma in the long tubular bones, which leads to pathognomonic symmetrical sclerosis of their metaphyses and diaphyses. Lipid granuloma may also be present in numerous other mesenchymal tissues, especially lung, orbit and retroperitoneal space. The clinical course and prognosis of the disease depend on these lesions. Reviewing 30 cases published since 1931 and a personal case with S100 positive cells, we present the typical radiological and clinical findings. There is striking resemblance to chronic disseminated histiocytosis X.

Bone Diseases↗

PubMed1991

Orbital and eyelid involvement with Erdheim-Chester disease. A report of two cases.

Erdheim-Chester disease is an idiopathic condition characterized by infiltration of the heart, lungs, retroperitoneum, bones, and other tissues by a fibrosing xanthogranulomatous process composed of xanthomatous histiocytes and Touton giant cells. This condition is often fatal, with death due to cardiomyopathy, severe lung disease, or chronic renal failure. Ocular findings with this potentially fatal disease are rare. We report the clinical and histopathologic findings in two cases of bilateral xanthelasmas and bilateral orbital infiltrates in association with Erdheim-Chester disease. The first patient was a 38-year-old man with cardiovascular and renal disease and severe retroperitoneal fibrosis. The massive orbital infiltration produced bilateral blindness. The second patient was a 77-year-old man with severe cardiovascular disease and retroperitoneal fibrosis. The diagnosis was confirmed in both patients with retroperitoneal and orbital biopsies. Both patients had the unusual occurrence of bilateral xanthelasmas with bilateral, diffuse orbital masses, eye findings that should alert the clinician to the possibility of this serious systemic disease.

Adult↗

PubMed1990

Scintigraphic findings and follow up in Erdheim-Chester disease.

Two cases of Erdheim-Chester disease are presented: a 26-year-old white male patient with lipoidgranulomatosis of numerous long and flat bones and infiltration of pericardium, pleura, liver, spleen, thyroid, skin, conjunctiva, gingiva, and false vocal cord; and a 54-year-old white male with involvement of bones, orbits, brain, pericardium, and retroperitoneum. The scintigraphic findings in this disease are described, and a comprehensive review of the 27 previously reported cases is given including an assessment of the value of scintigraphy for diagnosis and follow up of this rare disease.

Adult↗

PubMed1991

[Cerebral manifestations of Erdheim-Chester disease].

Cerebral manifestations of Erdheim-Chester disease are variable, giving a picture like that of multiple sclerosis. White matter lesions are located mainly in cerebellum and pons and lipid granulomas in the meninges. An asymptomatic lesion in the choroid plexus, with prolonged uptake of Gd-DTPA is described for the first time.

Bone Diseases↗

PubMed1991

Erdheim-Chester disease. Case report with autopsy findings.

Erdheim-Chester disease is a rare pathologic entity characterized by symmetrical radiodensities in the metaphyseal and the diaphyseal portions of the long bones. Fibrosis, osteoblastic cortical bone deposition, and fibroxanthomatous granulomas with lipid-laden macrophages and multinucleated giant cells, which have a particular tropism for connective and adipose tissues, are the pathologic hallmarks. To our knowledge, 27 cases have been reported in the literature since the entity was first described in 1930. Protean clinical features range from a focal and asymptomatic process to a multisystemic infiltrative disease. We describe the clinical course of a new case and review the extensive pathologic findings at autopsy, including those demonstrated by light and electron microscopy and cytochemical and immunocytochemical studies.

Adipose Tissue↗

PubMed2026

Cerebrovascular involvement in Erdheim-Chester disease: a case report and systematic literature review.

BACKGROUND: Intracranial perivascular/vascular infiltrations and stenoses related to Erdheim-Chester disease (ECD), often associated with ischemic events, are rarely documented. This study aims to characterize intracranial perivascular/vascular infiltrations and stenoses. METHODS: We first report a new case of strokes revealing ECD with intracranial arterial involvement. We then searched all English- and French-language publications from database inception to November 2025 across 12 different search interfaces, including grey literature sources. Vascular involvement was defined by the presence of intracranial perivascular/vascular infiltrations and stenosis on imaging and/or histopathological evidence of small-vessel involvement. Cases with intracranial nodules or masses abutting vessels but without clear longitudinal perivascular infiltration were excluded. RESULTS: We present a case of recurrent strokes with intracranial vertebral and basilar artery wall stenosis, and aortitis. Initially diagnosed as giant cell arteritis, the patient was treated with corticosteroids, cyclophosphamide followed by methotrexate, but relapsed. The identification of tibial osteosclerosis led to the diagnosis of ECD, with a favorable response to anakinra. Twelve relevant articles were retrieved, in addition to our own case. Most patients exhibited focal cerebrovascular signs (11/13) and associated parenchymal involvement (11/13). Intracranial perivascular/vascular infiltrations and stenoses involved the carotid arteries (7/13), the vertebrobasilar arteries (1/13), or both territories (4/13). Aorta was involved in 8/12 cases. Among the nine patients with available follow-up data, five had poor overall or neurovascular outcomes. CONCLUSIONS: Intracranial perivascular/vascular infiltrations and stenoses, which leads to recurrent focal ischemic events, represents a likely underdiagnosed CNS pattern in ECD, referred to as "cerebrovascular ECD", which worsens overall prognosis. Vascular imaging should be included in brain MRI protocols for patients with ECD, given the overlap with parenchymal involvement.

Humans↗

PubMed1989

Cerebral Erdheim-Chester disease: persistent enhancement with Gd-DTPA on MR images.

A case of Erdheim-Chester disease with intracerebral masses containing characteristic lipid-laden histiocytes is presented. These unusual lesions remained enhanced on magnetic resonance images obtained 8 days after injection of gadolinium diethylenetriaminepentaacetic acid (DTPA) dimeglumine. Chemical analysis of a biopsy specimen revealed a high concentration of gadolinium. Findings suggest that the Gd-DTPA complex or possibly a gadolinium-containing metabolite may be retained for extended periods in this unusual type of histiocytic lesion.

Brain↗

PubMed1988

A xanthogranulomatous process encircling large blood vessels (Erdheim-Chester disease?).

The case of a strange type of generalized xanthogranulomatosis, ending by a lethal kidney complication, is described in a woman aged 68. The clinical symptoms of the patient were not characteristic and did not lead to the actual diagnosis. The process was located along the basal brain arteries, it adhered to the adventitia of the descending thoracic aorta and of the coronary arteries. An identical xanthogranulomatous infiltrate was found in the peripelvic adipose tissue of both kidneys, where it led to stenosis of the proximal ends of both ureters; to a lesser extent such infiltrates appeared also in the periportal areas of the liver and in the bone marrow. Histological findings grant the possibility of the Erdheim-Chester disease. Differential diagnosis is subjected to discussion.

Aged↗

PubMed1992

Case report 710: Symmetrical eosinophilic granuloma of the lower extremities (proven) and Erdheim-Chester disease (probable).

We present a case of symmetrical EG of the lower extremities in a 36-year-old man. Several entities are considered in the differential diagnosis. However, many of the features bear a striking resemblance to ECD, which probably coexists in this case. A link between the two entities, EG and ECD, has been suggested by others. Future experience may confirm this hypothesis.

Adult↗

PubMed1992

Implant rehabilitation in Erdheim-Chester disease: a clinical report.

Successful osseointegration of endosseous titanium implants is thought to be dependent upon close apposition of bone to the implant surface. The integration of implants in this patient was achieved despite the lipid-laden histiocytic infiltration of the bone marrow. Presumably, enough unaffected stromal cells were present to allow sufficient bone formation for osseointegration of the implant fixtures. This result invites speculation regarding both the mechanism of osseointegration and the minimum surface area of bone-implant interface necessary for achieving and maintaining osseointegration of titanium implants. This patient is periodically examined to determine if the loaded fixtures will remain clinically immobile for a prolonged period.

Adipose Tissue↗

PubMed1979