title: [Erdheim-Chester disease].
abstract: METHODS
  A 55-year-old female was admitted complaining of musculoskeletal pain and weakness of both lower extremities for a number of years. Due to a hypothalamic mass of unknown aetiology a diabetes insipidus, a gonadotrophic, somatotrophic and a partially corticotrophic insufficiency had developed.
  
  
  METHODS
  Laboratory investigations yielded elevated levels of several inflammatory parameters (C-reactive protein, blood sedimentation rate, fibrinogen and thrombocytes). Serological parameters indicating a systemic rheumatic disorder were absent. X-ray examination revealed combined osteolytic and osteoblastic lesions within the distal parts of both femora and within the proximal portions of both tibiae. MRI showed signal alterations and (99m)Technetium bone scan exhibited a considerably increased uptake. Histopathologically, a biopsy of the left tibia showed multifocal small infiltrates of foamy histiocytes indicating Erdheim-Chester disease (ECD).
  
  
  METHODS
  Under treatment with glucocorticosteroids musculoskeletal complaints improved, but re-appeared following dose reduction. A therapeutic trial using methotrexat did not affect the complaints.
  
  
  CONCLUSIONS
  The Erdheim-Chester syndrome is considered to belong to diseases with a proliferation of the monocytic-histiocytic and dendritic cellular system. In the presence of symmetric musculoskeletal symptoms associated with osteosclerotic and osteolytic lesions particularly occurring in the long bones of the lower extremities and concomitant with elevated serum markers of inflammation, the Erdheim-Chester disease should be taken into account. To date, no validated therapy exists.
authors.affiliation: Abteilung Nephrologie und Rheumatologie, Georg-August-Universität Göttingen. mkoziolek@gmx.de
authors.forename: M J
authors.initials: MJ
authors.lastname: Koziolek
authors.forename: E
authors.initials: E
authors.lastname: Kunze
authors.forename: A
authors.initials: A
authors.lastname: Müller
authors.forename: V
authors.initials: V
authors.lastname: Thiem
authors.forename: A K
authors.initials: AK
authors.lastname: Scheel
authors.forename: D
authors.initials: D
authors.lastname: Müller
authors.forename: G A
authors.initials: GA
authors.lastname: Müller
authors.forename: F
authors.initials: F
authors.lastname: Strutz
mesh_terms: D001706:Biopsy; D031249:Erdheim-Chester Disease / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D011859:Radiography; D013977:Tibia / Q000473:pathology; D013997:Time Factors
chemical_list: D005938:Glucocorticoids