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.
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.
Erdheim-Chester disease can show areas of sclerosis in the epiphyses.
Erdheim-Chester disease can show areas of sclerosis in the epiphyses.
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.
Flashcards
Erdheim-Chester Disease (ECD)
Erdheim-Chester Disease (ECD)
A rare disease characterized by an abnormal accumulation of histiocytes, primarily in the appendicular skeleton, leading to fibrosis and sclerosis.
Histiocytosis
Histiocytosis
A group of disorders affecting the histiocytes, characterized by the abnormal proliferation of these immune cells.
Langerhans Cell Histiocytosis (LCH)
Langerhans Cell Histiocytosis (LCH)
A form of histiocytosis often diagnosed in children and young adults, characterized by the proliferation of Langerhans cells.
Sclerotic Bone Lesions
Sclerotic Bone Lesions
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Bony Pain
Bony Pain
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Histiocytosis Triad
Histiocytosis Triad
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Metadiaphyseal Sclerosis
Metadiaphyseal Sclerosis
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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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