Rare Diseases Symptoms Automatic Extraction

Unusual orbital involvement in Erdheim Chester disease: a radiological diagnosis.

[erdheim-chester disease]

Erdheim-Chester disease (ECD) is an exceedingly rare, disseminated non-Langerhan cell histiocytosis with multisystem involvement, having characteristic sclerotic skeletal lesions. We present an unusual case primarily manifesting as an extensive orbital disease, with low-grade systemic involvement. Owing to its rarity and therefore lack of general awareness it remains a difficult clinical and pathologic diagnosis. Immuno-histochemistry of the biopsy specimen is diagnostic.

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