title: Congenital dystrophic epidermolysis bullosa (DEB) in Sprague Dawley rats: a case series.
abstract: BACKGROUND
  Epidermolysis bullosa is a rare skin disease caused by defects in the basement membrane and/or other dermoepidermal junction components.
  
  
  OBJECTIVE
  We describe a series of spontaneous cases of dystrophic epidermolysis bullosa (DEB) in a colony of Sprague Dawley rats investigated with histopathology, transmission electron microscopy (TEM) and inheritance pattern.
  
  
  METHODS
  Four, 4-day-old pups from a litter of Sprague Dawley rats developed blistering, haemorrhagic skin lesions and were euthanized. Age-matched controls from the same litter were normal. Several months later two more litters presented with identical findings. All three litters had the same sire, suggesting a genetic component.
  
  
  METHODS
  Skin from affected and control animals was evaluated histologically and with TEM. Unaffected sibling pairs from affected litters were bred in order to potentially reproduce the disease and determine the mode of inheritance.
  
  
  RESULTS
  Histologically, there was significant dermoepidermal clefting below the basement membrane with variable amounts of haemorrhage and cellular debris within the clefts. Ultrastructurally, clefting occurred below the basement membrane with an intact lamina densa and normal hemidesmosomes. Anchoring filaments were strikingly absent. Litters produced from phenotypically unaffected sibling pairs resulted in a total of four more litters with approximately a quarter of pups affected.
  
  
  CONCLUSIONS
  Based on the gross lesions, histopathological features and TEM determination of separation below the lamina densa and lack of normal anchoring fibrils, these cases are most consistent with DEB. This is the first report of naturally occurring, localized and reproducible recessive DEB in Sprague Dawley rats.
authors.affiliation: Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A&M University, 422 Raymond Stotzer Parkway, College Station, TX, 77845, USA.|Department of Biomedical and Veterinary Sciences, Virginia-Maryland Regional College of Veterinary Medicine, 245 Duck Pond Drive, Blacksburg, VA, 24061, USA.
authors.forename: Kristin B
authors.initials: KB
authors.lastname: Eden
authors.affiliation: Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A&M University, 422 Raymond Stotzer Parkway, College Station, TX, 77845, USA.
authors.forename: Ashley
authors.initials: A
authors.lastname: Peterson
authors.affiliation: Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A&M University, 422 Raymond Stotzer Parkway, College Station, TX, 77845, USA.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Payne
authors.affiliation: Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A&M University, 422 Raymond Stotzer Parkway, College Station, TX, 77845, USA.
authors.forename: Wayne V
authors.initials: WV
authors.lastname: Corapi
authors.affiliation: Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A&M University, 422 Raymond Stotzer Parkway, College Station, TX, 77845, USA.
authors.forename: Joanne
authors.initials: J
authors.lastname: Mansell
authors.affiliation: Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A&M University, 422 Raymond Stotzer Parkway, College Station, TX, 77845, USA.
authors.forename: Aline Rodrigues
authors.initials: AR
authors.lastname: Hoffman
mesh_terms: D000818:Animals; D016108:Epidermolysis Bullosa Dystrophica / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D020022:Genetic Predisposition to Disease; D051381:Rats; D017207:Rats, Sprague-Dawley; D012376:Rodent Diseases / Q000151:congenital* / Q000235:genetics / Q000473:pathology