title: Collagen XVIII and fibronectin involvement in bullous scleroderma.
abstract: BACKGROUND
  Endostatin, an anti-angiogenic C-terminal fragment of collagen XVIII, has been recently reported to play a role in scleroderma pathogenesis, but collagen XVIII immunohistochemistry in scleroderma skin has still not been performed. Bullous scleroderma, a rare form of scleroderma, may have altered angiogenic and lymphangiogenic characteristics.
  
  
  OBJECTIVE
  Our aim is to report a rare case of bullous scleroderma, studying the presence of fibronectin and collagens type I, III and XVIII in sclerodermic skin.
  
  
  METHODS
  We describe the progression of bullous scleroderma in a 67-year-old patient since the first symptoms. Histological and immunohistochemical aspects of skin biopsies are compared to normal skin from a patient without scleroderma and are correlated with the pathogenesis of the disease. Indirect immunofluorescence measured by laser confocal microscopy allows quantitative determination of fibronectin and collagens type I, III and XVIII.
  
  
  CONCLUSIONS
  Dermo-epidermal cleavage, fibrosis and inflammation are the main histological findings. The dermal distribution and amounts of collagens and in the scleroderma patient are similar to normal skin. Conversely, both fibronectin and collagen XVIII are increased in scleroderma skin, suggesting their involvement in the pathogenesis of bullous scleroderma.
authors.affiliation: Hospital Semper, Belo Horizonte, Minas Gerais, Brazil. sorayasantos@doutoradermatologista.com.br
authors.forename: Soraya Neves Marques Barbosa dos
authors.initials: SN
authors.lastname: Santos
authors.forename: Gisele Viana de
authors.initials: GV
authors.lastname: Oliveira
authors.forename: André Luiz Pasqua
authors.initials: AL
authors.lastname: Tavares
authors.forename: André Ricardo
authors.initials: AR
authors.lastname: Massensini
authors.forename: Lorenza Machado de Souza
authors.initials: LM
authors.lastname: Carvalhaes
authors.forename: Ritva
authors.initials: R
authors.lastname: Reljasvaara
authors.forename: Gregory Thomas
authors.initials: GT
authors.lastname: Kitten
mesh_terms: D000368:Aged; D043170:Collagen Type XVIII / Q000378:metabolism*; D005260:Female; D005353:Fibronectins / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D012594:Scleroderma, Localized / Q000378:metabolism / Q000473:pathology*; D012872:Skin Diseases, Vesiculobullous / Q000378:metabolism / Q000473:pathology*
chemical_list: D043170:Collagen Type XVIII; D005353:Fibronectins