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 "histiocytosis" symptom

  • child syndrome
  • cutaneous mastocytosis
  • dentin dysplasia
  • dentinogenesis imperfecta
  • erdheim-chester disease
  • kallmann syndrome
  • lymphangioleiomyomatosis
  • omenn syndrome
  • papillon-lefèvre syndrome

This symptom has already been validated