Document Detail

Coagulation activation in autoimmune bullous diseases.
MedLine Citation:
PMID:  19737228     Owner:  NLM     Status:  MEDLINE    
The main autoimmune blistering skin disorders are pemphigus vulgaris (PV) and bullous pemphigoid (BP). They differ in the inflammatory infiltrate, which is more intense in BP. Inflammation is known to activate coagulation in several disorders. Local and systemic activation of coagulation was evaluated in BP and PV. We studied 20 BP patients (10 active and 10 remittent), 23 PV patients (13 active and 10 remittent) and 10 healthy subjects. The coagulation markers prothrombin fragment F1+2 and D-dimer were measured by enzyme-immunoassays in plasma. The presence of tissue factor (TF), the main initiator of blood coagulation, was evaluated immunohistochemically in skin specimens from 10 patients with active PV, 10 patients with active BP and 10 controls. Plasma F1+2 and D-dimer levels were significantly high in active BP (P = 0.001), whereas in active PV the levels were normal. During remission, F1+2 and D-dimer plasma levels were normal in both BP and PV. TF immunoreactivity was found in active BP but neither in active PV nor in normal skin. TF reactivity scores were higher in active BP than in controls or active PV (P = 0.0001). No difference in TF scores was found between active PV and controls. BP is associated with coagulation activation, which is lacking in PV. This suggests that BP but not PV patients have an increased thrombotic risk. The observation that thrombotic complications occur more frequently in BP than in PV further supports this view.
A V Marzano; A Tedeschi; D Spinelli; D Fanoni; C Crosti; M Cugno
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  158     ISSN:  1365-2249     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-09-09     Completed Date:  2009-10-09     Revised Date:  2013-05-31    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  31-6     Citation Subset:  IM    
Department of Anesthesiology, Intensive Care and Dermatological Sciences, University of Milan, Milan, Italy.
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MeSH Terms
Aged, 80 and over
Biological Markers / blood
Blood Coagulation*
Case-Control Studies
Eosinophils / immunology
Fibrin Fibrinogen Degradation Products / analysis
Lymphocytes / immunology
Middle Aged
Pemphigoid, Bullous / blood,  immunology*,  physiopathology
Pemphigus / blood,  immunology*,  physiopathology
Peptide Fragments / blood
Skin / chemistry,  immunology
Statistics, Nonparametric
Thromboplastin / analysis
Young Adult
Reg. No./Substance:
0/Biological Markers; 0/Fibrin Fibrinogen Degradation Products; 0/Peptide Fragments; 0/fibrin fragment D; 0/prothrombin fragment 1.2; 9001-26-7/Prothrombin; 9035-58-9/Thromboplastin

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