- Onset is usually between 5 and 10 years of age, but can be from birth.
- Develops in <20% of patients with eosinophillic granuloma
- Skeletal and visceral involvement with a chronic course
- Minority of cases can be fatal
- Classic triad of exophthalmos, diabetes insipidus, and rarefied defects of skull occurs in less than 10% of patients
- Exophthalmos is caused by retro-orbital invasion
- Diabetes insipidus caused by sphenoid invasion
- Reticulo-nodular pattern of pulmonary involvement often observed
- Other evidence of dissemination: anemia; splenomegaly; lymphadenopathy; and involvement of pericardium, liver, and retroperitoneum.
- Skin involvement can occur as well, leading to yellow nodules and ulcers on palate, gums, and vulva
- Jaw involvement can lead to tooth loss
Last Updated on June 27, 2024
🔗 Knowledge Graph & Clinical Correlates
OrthopaedicsOne Living Semantic Network🛠️ Operative Techniques
- Wrist Implant Arthroplasty Hand and Wrist
- Wrist disarticulation Hand and Wrist
- Wrist Dennervation Hand and Wrist
- Wrist arthroscopy Hand and Wrist
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