Intracranial Erdheim-Chester Disease Mimicking Parafalcine Meningioma: Report of Two Cases and Review of the Literature

Background: Erdheim-chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, which typically presents with bilateral symmetric osteosclerosis and multi-organ involvement. Lesions may be intraosseous or extraosseous and involve the heart, pulmonary system, CNS, and skin in order of decreasing likelihood. Objective: The objective of this study is to discuss a case of erdheim-chester disease and conduct a review of the literature. Case: We describe a rare case of erdheim-chester in an asymptomatic 37-year-old male who was diagnosed after suffering a right ulnar injury. Subsequent evaluation revealed a solitary radiolucent ulnar lesion without multi-system involvement. Results & Conclusion: The case is unique in its solitary distribution, lytic radiographic appearance, and asymptomatic presentation preceding pathologic fracture. This presentation may simulate multiple other bone lesions.

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THU0373 Clinical presentation of erdheim-chester disease: Data from a cohort of 10 patients and review of the literature

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2012-eular.2338 ◽

2013 ◽

Vol 71 (Suppl 3) ◽

pp. 281.3-282

Author(s):

G. Cavalli ◽

B. Guglielmi ◽

C. Campochiaro ◽

A. Berti ◽

M.G. Sabbadini ◽

...

Keyword(s):

Clinical Presentation ◽

Review Of The Literature ◽

Erdheim Chester Disease ◽

Erdheim Chester ◽

Chester Disease

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

International Urology and Nephrology ◽

10.1007/s11255-010-9724-9 ◽

2010 ◽

Vol 42 (4) ◽

pp. 1107-1112 ◽

Cited By ~ 11

Author(s):

Jose Emilio Sanchez ◽

Carmen Mora ◽

Manuel Macia ◽

Juan F. Navarro

Keyword(s):

Renal Failure ◽

Case Report ◽

Review Of The Literature ◽

End Stage Renal Failure ◽

Erdheim Chester Disease ◽

End Stage ◽

Erdheim Chester ◽

Chester Disease

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Erdheim chester disease: A case report and review of the literature

Case Reports in Clinical Pathology ◽

10.5430/crcp.v7n1p22 ◽

2020 ◽

Vol 7 (1) ◽

pp. 22

Author(s):

Rémie Philippe Elia ◽

ATALLAH Adnan ◽

AKIKI Béatrice ◽

WAKED Hani ◽

ZEIDAN Marwan ◽

...

Keyword(s):

Case Report ◽

Braf V600e ◽

Bone Lesions ◽

Review Of The Literature ◽

Rare Form ◽

Immune Profile ◽

Systemic Involvement ◽

Erdheim Chester Disease ◽

Erdheim Chester ◽

Chester Disease

Erdheim Chester disease is a rare form of non-Langerhans histiocytosis with frequent BRAF V600E mutations. It is mainly characterized by multifocal osteosclerotic bone lesions with or without systemic involvement. The histologic image is consistent with a histiocytic proliferation of foamy cells in a polymorphic background. The main difference from the Langerhans histiocytosis is the immune profile with mainly S100, CD1a, and langerin negative. The overall prognosis is dependent on extraskeletal involvement. Herein, we present a typical presentation of Erdheim Chester disease with a review of the literature.

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Erdheim-Chester Disease: A Case Report and Review of the Literature

Journal of Clinical Imaging Science ◽

10.25259/jcis_68_2020 ◽

2020 ◽

Vol 10 ◽

pp. 37

Author(s):

Hema Merai ◽

David Collas ◽

Ashish Bhagat ◽

Uday Mandalia

Keyword(s):

Systemic Disease ◽

Review Of The Literature ◽

Skeletal System ◽

Radiological Findings ◽

Rare Form ◽

Birbeck Granules ◽

Erdheim Chester Disease ◽

Life Threatening ◽

Erdheim Chester ◽

Chester Disease

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans’ cell histiocytosis characterized by xanthogranulomatous infiltration of foamy histiocytes surrounded by fibrosis. ECD may be asymptomatic or present as a multi-systemic disease with life-threatening manifestations, most commonly involving the skeletal system. Immunohistochemical staining demonstrates cells that are CD68+, CD1a–, and S100– with an absence of Birbeck granules. We report a case of a 69-year old male patient who presented with neurological symptoms – eventually thought to be separate to his diagnosis of ECD. It represents the ability to diagnose ECD based just on radiological findings in an otherwise asymptomatic individual.

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