No abstract available.
Erdheim-Chester Disease

No abstract available.
Erdheim-Chester Disease

Erdheim-Chester Disease (ECD) is a rare form of non Langerhans' cell histiocytosis. The etiology of ECD is unknown yet. The disease is characterized by the infiltration of lipid-laden histiocytes with foamy to bones and various organs. The heterogeneous manifestations of ECD vary among different individuals. This results in a presentation that may vary from an indolent focal disease to a life threatening organ failure. This review focuses on the etiology, clinical presentation, diagnostic criteria and treatment of Erdheim-Chester disease (ECD).
Erdheim-Chester Disease ; Humans

No abstract available.
Erdheim-Chester Disease*

Child ; Erdheim-Chester Disease ; Female ; Humans

Humans ; Erdheim-Chester Disease/genetics* ; Pedigree ; Mutation

ECD is a rare, non-Langerhans form of histiocytosis. The first case was reported in 1930 by Chester and the term ECD was first described by Jaffe in 1972. Many authors have suggested that ECD is a part of the spectrum of Langerhans cell histiocytosis(LCH), whereas others believe ECD as a distinct lipoidosis because of the symmetric distribution of the long bone lesions on the roentgenogram and the lack of Langerhans granules and S-100 antibody staining in the foamy histiocytes. We experienced a case of Erdheim-Chester disease (ECD) with periodic night fever, myalgia and both knee pain, the first case reported in Korea.
Erdheim-Chester Disease* ; Fever* ; Histiocytes ; Histiocytosis ; Knee* ; Korea ; Lipidoses ; Myalgia

Humans ; Erdheim-Chester Disease/diagnostic imaging* ; Diagnostic Imaging

Coronary Disease ; complications ; Erdheim-Chester Disease ; complications ; Humans ; Male ; Middle Aged

Erdheim-Chester disease (ECD) is non-Langerhans cell histiocytosis that leads to multi-organ involvement with unknown etiology. We report a 64-year-old woman with a dry cough and dyspnea. She had interlobular septal thickening and ground-glass opacity in both lungs, suggesting lymphangitic lung metastasis. However, she was diagnosed with ECD after a long bone and immunohistological evaluation.
Cough ; Dyspnea ; Erdheim-Chester Disease ; Female ; Histiocytosis ; Humans ; Lung ; Middle Aged ; Neoplasm Metastasis ; Retroperitoneal Fibrosis