Document Detail

Activation of coagulation in bullous pemphigoid and other eosinophil-related inflammatory skin diseases.
MedLine Citation:
PMID:  21488867     Owner:  NLM     Status:  MEDLINE    
Bullous pemphigoid (BP) is a skin disease caused by autoantibodies to hemidesmosomal proteins BP180 and BP230, with eosinophils participating in blister formation. Tissue factor (TF), the initiator of coagulation, is embodied within the eosinophil granules and exposed upon activation. We evaluated the coagulation activation in patients with BP (63), chronic urticaria (CU; 20), atopic dermatitis (AD; 14), cutaneous drug reactions (CDRs; six), psoriasis (20), dermatitis herpetiformis (DH; four) and primary cutaneous T cell lymphoma (CTCL; five), and in 40 healthy controls. Prothrombin fragment F1+2 and d-dimer (coagulation markers) were measured by enzyme-linked immunosorbent assay (ELISA) in all plasma samples and BP blister fluid. Skin TF expression was evaluated immunohistochemically in the patients and 20 controls. F1+2 and d-dimer levels were higher in BP plasma than in control plasma (P = 0·0001 for both), and dramatically high in blister fluid; both correlated positively with disease severity, esinophil counts and anti-BP180 antibodies (P = 0·006-0·0001). Plasma F1+2 and d-dimer levels were higher in the CU, AD and CDR patients than in controls (P = 0·0001 for all), but normal in the psoriasis, DH and CTCL patients. Skin TF was expressed in the BP (P = 0·0001), CU (P = 0·0001), AD (P = 0·001) and CDR patients (P = 0·01), but not in the psoriasis, DH or CTCL patients. Co-localization confocal microscopy studies confirmed eosinophils as the source of TF in 10 BP patients. The coagulation cascade is activated in BP and other eosinophil-mediated skin disorders, but not in non-eosinophil driven conditions. This hypercoagulability may contribute to inflammation, tissue damage and, possibly, thrombotic risk.
A V Marzano; A Tedeschi; E Berti; D Fanoni; C Crosti; M Cugno
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Publication Detail:
Type:  Journal Article     Date:  2011-04-13
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  165     ISSN:  1365-2249     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2011-06-03     Completed Date:  2011-08-29     Revised Date:  2013-06-30    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  44-50     Citation Subset:  IM    
Copyright Information:
© 2011 The Authors; Clinical and Experimental Immunology © 2011 British Society for Immunology.
Dipartimento di Medicina Interna, Università degli Studi di Milano, Italy.
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MeSH Terms
Aged, 80 and over
Autoantibodies / blood
Autoantigens / immunology
Blood Coagulation* / immunology
Cell Count
Disease Progression
Eosinophils / immunology,  metabolism*,  pathology
Fibrin Fibrinogen Degradation Products / metabolism
Lymphoma, T-Cell, Cutaneous / blood,  immunology*,  pathology
Membrane Glycoproteins / immunology
Middle Aged
Non-Fibrillar Collagens / immunology
Pemphigoid, Bullous / blood,  immunology*
Peptide Fragments / blood
Skin Neoplasms / blood,  immunology*,  pathology
Reg. No./Substance:
0/Autoantibodies; 0/Autoantigens; 0/BP230 protein, human; 0/Fibrin Fibrinogen Degradation Products; 0/Membrane Glycoproteins; 0/Non-Fibrillar Collagens; 0/Peptide Fragments; 0/collagen type XVII; 0/fibrin fragment D; 0/prothrombin fragment 1.2; 9001-26-7/Prothrombin; 9035-58-9/Thromboplastin

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