title: Decreased fibrinolytic potential and morphological changes of fibrin structure in dermatitis herpetiformis.
abstract: BACKGROUND
  Recently, high prevalence of cryofibrinogenaemia has been observed in plasma of untreated dermatitis herpetiformis (DH) patients, and the pathological IgA and TG3 deposits in the papillary dermis were found to co-localize with fibrin and fibrinogen.
  
  
  OBJECTIVE
  To study the fibrinolytic potential in plasma of untreated, dapsone and or/gluten-free diet treated DH patients as well as the in vitro effect of dapsone on the fibrinolytic profile.
  
  
  METHODS
  Plasma samples of 23 DH patients, 19 healthy subjects and 5 pemphigus vulgaris patients were investigated by a turbidimetric-clot lysis assay. Out of them 5 DH plasma samples representing different fibrinolytic parameters, and 3 healthy controls were selected for parallel fibrin clot preparation. The clot fibrin structure was examined by scanning electron microscopy (SEM), and the diameters of 900 fibrin fibres were determined in each clot.
  
  
  RESULTS
  A significantly prolonged clot lysis time was detected in untreated DH patients. The turbidity values of DH plasma clots indicated an altered fibrin structure that was also confirmed by SEM: significantly thicker fibrin fibers were observed in untreated, TG3 antibody positive DH patients compared to healthy controls, whereas the fiber diameters of dapsone-treated patients were similar or thinner than the control values. In line with the structural changes of fibrin, the fibrinolytic profile of 5 DH patients under dapsone treatment approached the control values.
  
  
  CONCLUSIONS
  This study revealed that the fibrinolytic potential was impaired in the plasma of untreated DH patients, whereas dapsone corrected the fibrinolytic defect. These data suggest a pathogenic role for plasma-derived factors in the development of skin symptoms and add a new aspect to the long-known beneficial, symptomatic effect of dapsone in active DH.
authors.affiliation: Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary; Doctoral School of Clinical Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: Anna
authors.initials: A
authors.lastname: Görög
authors.affiliation: Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
authors.forename: Krisztián
authors.initials: K
authors.lastname: Németh
authors.affiliation: Institute of Materials and Environmental Chemistry, Research Centre for Natural Science, Hungarian Academy of Sciences, Budapest, Hungary.
authors.forename: László
authors.initials: L
authors.lastname: Szabó
authors.affiliation: Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
authors.forename: Balázs
authors.initials: B
authors.lastname: Mayer
authors.affiliation: Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
authors.forename: Pálma
authors.initials: P
authors.lastname: Silló
authors.affiliation: Department of Medical Biochemistry, Semmelweis University, Budapest, Hungary.
authors.forename: Krasimir
authors.initials: K
authors.lastname: Kolev
authors.affiliation: Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary. Electronic address: skarpati@t-online.hu.
authors.forename: Sarolta
authors.initials: S
authors.lastname: Kárpáti
mesh_terms: D000328:Adult; D000368:Aged; D001777:Blood Coagulation; D016022:Case-Control Studies; D003449:Cryoglobulinemia / Q000097:blood; D003622:Dapsone / Q000627:therapeutic use; D003874:Dermatitis Herpetiformis / Q000097:blood* / Q000188:drug therapy; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005337:Fibrin / Q000737:chemistry*; D005340:Fibrinogen / Q000737:chemistry; D005342:Fibrinolysis; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007700:Kinetics; D008297:Male; D008855:Microscopy, Electron, Scanning; D008875:Middle Aged; D009391:Nephelometry and Turbidimetry; D012867:Skin / Q000378:metabolism; D055815:Young Adult
chemical_list: D003622:Dapsone; D005337:Fibrin; D005340:Fibrinogen
keywords: Dapsone; Dermatitis herpetiformis; Fibrin; Fibrinolysis; Transglutaminase 3