ICD-11 code : XH1VJ3;
Preferred Label : Erdheim-Chester disease;
ICD-11 definition : Erdheim-Chester disease is a Class IIa (non-Langerhans cell) histiocytosis of unknown
aetiology characterized by a xanthogranulomatous infiltration of viscera, bones, retroperitoneum
and skin by lipid-laden histiocytes and surrounding fibrosis. It most commonly presents
in adult life with bone pain, typically around the knees and ankles. Exophthalmos,
diabetes insipidus, cutaneous xanthomata, pulmonary fibrosis and a broad range of
complications resulting from retroperitoneal and CNS infiltration are all well recognized.;
Origin ID : 1395439137;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Erdheim-Chester disease is a Class IIa (non-Langerhans cell) histiocytosis of unknown
aetiology characterized by a xanthogranulomatous infiltration of viscera, bones, retroperitoneum
and skin by lipid-laden histiocytes and surrounding fibrosis. It most commonly presents
in adult life with bone pain, typically around the knees and ankles. Exophthalmos,
diabetes insipidus, cutaneous xanthomata, pulmonary fibrosis and a broad range of
complications resulting from retroperitoneal and CNS infiltration are all well recognized.
