title: [Xanthoma disseminatum with marked mucocutaneous involvement].
abstract: METHODS
  When aged 23 years, a now 36-year-old man was first diagnosed as having xanthomas on the upper arms and shoulders. Xanthomas then progressed, affecting both the skin and the laryngo-pharyngeal mucosa. They were so marked that several laser-surgical interventions for their removal in the phayngo-laryngeal tract were necessary to ensure unimpaired breathing. There were also extensive confluent symmetrical cutaneous xanthomas over the upper and lower arms, the face, neck and trunk. Xanthomas and scars in the pharynx and larynx necessitated marked nasal breathing.
  
  
  METHODS
  There was no laboratory evidence of abnormal lipid metabolism. The concentrations of cholesterol, triglycerides, lipoprotein (a), apolipoprotein A-1, apolipoprotein B, apolipoprotein E phenotype and steroles were all normal. The biochemical composition of LDL, VLDL and HDL particle was also unremarkable. Histological examination of resected xanthomas revealed dense infiltrations of the interstitial spaces by foam-cell histiocytes with multiple lipid vacuoles, typical of xanthoma disseminatum.
  
  
  METHODS
  Neither probucol nor cholesterol synthesis enzyme inhibitors nor glucocorticoid medication influenced the xanthomas. The only effective treatment was removal of the most unsightly or obstructing lesions. But the sars left removal in the mucocutaneous regions caused obstruction in the laryngopharyngeal tract.
  
  
  CONCLUSIONS
  The cause of xanthoma disseminatum remains unknown. Skeletal muscle can also be extensively infiltrated. This case shows similarities to Erdheim-Chester disease, another are xanthomatous condition.
authors.affiliation: Abteilung Gastroenterologie und Hepatologie, Medizinische Hochschule Hannover.
authors.forename: U J
authors.initials: UJ
authors.lastname: Tietge
authors.forename: H
authors.initials: H
authors.lastname: Maschek
authors.forename: A
authors.initials: A
authors.lastname: Schneider
authors.forename: A E
authors.initials: AE
authors.lastname: Gawehn
authors.forename: S
authors.initials: S
authors.lastname: Wagner
authors.forename: M P
authors.initials: MP
authors.lastname: Manns
authors.forename: H H
authors.initials: HH
authors.lastname: Schmidt
mesh_terms: D000328:Adult; D001132:Arm; D002908:Chronic Disease; D003131:Combined Modality Therapy; D018450:Disease Progression; D004359:Drug Therapy, Combination; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male; D009092:Mucous Membrane / Q000473:pathology; D012867:Skin / Q000473:pathology*
vernacular_title: Xanthoma disseminatum mit ausgeprägter mukokutaner Beteiligung.