Rare Diseases Symptoms Automatic Extraction

Immunopathological analysis of Erdheim-Chester disease with massive ascites.

[erdheim-chester disease]

We treated a 77-year-old woman with pleural and pericardial effusion and ascites. Initially, collagen vascular disease was suspected due to the presence of anti-centromere antibodies and suspected complication of pulmonary arterial hypertension. However, soft-tissue abnormalities surrounding the bilateral kidneys detected on computed tomography (CT) and symmetrical lesions of the long bones detected on bone scintigraphy made us consider a diagnosis of Erdheim-Chester disease (ECD), which is a rare form of histiocytosis. We immunochemically analyzed the cells derived from the ascites in detail and confirmed the diagnosis. Immunocytochemical analyses may therefore help to achieve a better understanding of the pathogenesis of this rare disease.

Diseases presenting "pericardial effusion" symptom

  • acute rheumatic fever
  • cushing syndrome
  • erdheim-chester disease
  • familial mediterranean fever
  • primary effusion lymphoma
  • pyomyositis
  • systemic capillary leak syndrome

This symptom has already been validated