title: Cutaneous lymphatic malformations in disappearing bone (Gorham-Stout) disease: a novel clue to the pathogenesis of a rare syndrome.
abstract: BACKGROUND
  Gorham-Stout disease is an unusual, progressive syndrome of unknown etiology characterized by mono- or polyostotic osteolysis most often affecting children and young adults. The onset is insidious and the disease progresses to extensive and potentially disabling osteolysis often unresponsive to therapeutic intervention. Although bone and soft tissue lesions are the most frequent manifestations of Gorham-Stout disease, skin lesions can occur and may provide a clue to the pathogenesis of this rare syndrome.
  
  
  OBJECTIVE
  Our aim was to describe characteristics of vascular skin lesions of this rare condition using magnetic resonance imaging and histomorphological analysis.
  
  
  METHODS
  The case of a 36-year-old man with Gorham-Stout disease of the left leg and foot is reported.
  
  
  RESULTS
  This case was remarkable for its prominent lymphatic vascular malformations involving the skin and soft tissues adjacent to the diseased bone-a previously undescribed abnormality, which preceded osteolysis for several years. Magnetic resonance imaging played a key role in defining the extent of disease in skin and soft tissues.
  
  
  CONCLUSIONS
  It is difficult to assess the true incidence of hemangiomatosis in the earlier reports on Gorham-Stout disease in which hemangiomatous cutaneous lesions were mentioned but not described or illustrated.
  
  
  CONCLUSIONS
  A vascular process with angiomatous histological features is considered to be the pathological hallmark of Gorham-Stout disease, but the specific type of this vascular process is still under debate. Our report highlights a lymphatic malformative nature of Gorham-Stout disease, thereby contributing to a better understanding and characterization of this rare disease entity.
authors.affiliation: Department of Dermatology, Bethesda-Krankenhaus Duisburg. bruch-gerharz@med.uni-duesseldorf.de
authors.forename: Daniela
authors.initials: D
authors.lastname: Bruch-Gerharz
authors.forename: Claus-Dieter
authors.initials: CD
authors.lastname: Gerharz
authors.forename: Helger
authors.initials: H
authors.lastname: Stege
authors.forename: Jean
authors.initials: J
authors.lastname: Krutmann
authors.forename: Michael
authors.initials: M
authors.lastname: Pohl
authors.forename: Rainer
authors.initials: R
authors.lastname: Koester
authors.forename: Thomas
authors.initials: T
authors.lastname: Ruzicka
mesh_terms: D000328:Adult; D005269:Femur / Q000000981:diagnostic imaging; D006801:Humans; D008208:Lymphatic System / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D010015:Osteolysis, Essential / Q000175:diagnosis / Q000209:etiology / Q000473:pathology*; D011859:Radiography; D012867:Skin / Q000473:pathology*