Podcast
Questions and Answers
Erdheim-Chester disease peaks in incidence at around 55 years of age.
Erdheim-Chester disease peaks in incidence at around 55 years of age.
True (A)
Langerhans Cell Histiocytosis typically presents in patients around the age of 20-30.
Langerhans Cell Histiocytosis typically presents in patients around the age of 20-30.
False (B)
Both Erdheim-Chester disease and Langerhans Cell Histiocytosis are caused by clonal proliferation of histiocytes.
Both Erdheim-Chester disease and Langerhans Cell Histiocytosis are caused by clonal proliferation of histiocytes.
True (A)
In Erdheim-Chester disease, lytic lesions are absent in radiological findings.
In Erdheim-Chester disease, lytic lesions are absent in radiological findings.
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The most prominent clinical feature of Erdheim-Chester disease is often diabetes insipidus.
The most prominent clinical feature of Erdheim-Chester disease is often diabetes insipidus.
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Erdheim-Chester disease can show areas of sclerosis in the epiphyses.
Erdheim-Chester disease can show areas of sclerosis in the epiphyses.
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Patients with Erdheim-Chester disease may experience exophthalmos as part of their clinical presentation.
Patients with Erdheim-Chester disease may experience exophthalmos as part of their clinical presentation.
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Study Notes
Erdheim-Chester Disease (ECD)
- ECD is characterized by histiocyte infiltration of the appendicular skeleton, causing fibrosis and sclerosis, evident on X-rays.
- Similar to Langerhans Cell Histiocytosis (LCH), but ECD peaks in incidence around age 55, while LCH typically presents between 5-10 years old.
- Both are caused by clonal histiocyte proliferation, with different markers aiding pathological distinction.
- Clinical and radiological overlap exists between ECD and LCH.
- Common clinical presentation includes elements of the histiocytosis triad: diabetes insipidus, exophthalmos, and bone pain. Bony pain is often the most noticeable symptom.
- X-rays show bilateral, symmetrical sclerosis in metadiaphyseal regions, sparing the epiphyses.
- Corticomedullary differentiation is blurred, and lytic lesions are also present, indicated by areas of lucency.
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Description
This quiz covers Erdheim-Chester Disease (ECD), including its characteristics, clinical presentation, and radiological findings. Learn about its similarities with Langerhans Cell Histiocytosis (LCH) and the age differences in incidence. Test your understanding of this rare disease and its diagnostic markers.
