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Paediatric Erdheim-Chester disease with aggressive skin manifestations
The British Journal of Dermatology
|January 13, 2017
Summary
Erdheim-Chester disease (ECD), a rare histiocytosis, presented uniquely in a young boy with widespread skin and bone issues. This case highlights generalized skin lesions and the youngest reported pediatric onset of ECD.
Area of Science:
- Histiocytosis
- Pediatric rare diseases
- Dermatology
Background:
- Erdheim-Chester disease (ECD) is a rare systemic non-Langerhans cell histiocytosis.
- ECD involves the accumulation of CD68+, CD1a- foamy histiocytes.
- Pediatric ECD is exceptionally rare, with limited description of its cutaneous manifestations.
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