Coagulation and skin autoimmunity

Massimo Cugno, Alessandro Borghi, Simone Garcovich, Angelo Valerio Marzano

Research output: Contribution to journalReview articlepeer-review


Several lines of evidence indicate that the immune system, inflammation, and coagulation are simultaneously activated in autoimmune and immune-mediated skin diseases. Pro-inflammatory cytokines such as interleukin-6 and tumor necrosis factor-alpha induce the expression of the main initiator of coagulation, i.e., tissue factor. The proteases of coagulation in turn act on protease-activated receptors inducing the expression of various pro-inflammatory cytokines triggering inflammation. The cross-talk among immune system, inflammation, and coagulation amplifies and maintains the activation of all three pathways. This review focuses on three skin disorders as chronic spontaneous urticaria (CSU), angioedema, and bullous pemphigoid (BP), in which the relationships among the three systems have been investigated or their clinical consequences are relevant. Markers of thrombin generation, fibrinolysis, and inflammation have been reported to be increased in the plasma during flares of CSU and angioedema, as well as in the active phase of BP, with the marker levels reverting to normal during remission. The coagulation activation seems to be important only at local level in CSU and angioedema while both at local and systemic levels in BP which is the only condition associated with an increased thrombotic risk. The prothrombotic state in autoimmune skin diseases raises the question of the indication of anticoagulant treatment, particularly in the presence of other cardiovascular risk factors.

Original languageEnglish
Article number1407
JournalFrontiers in Immunology
Issue numberJUN
Publication statusPublished - Jan 1 2019


  • Angioedema
  • Atopic dermatitis
  • Autoimmunity
  • Bullous pemphigoid
  • Coagulation
  • Dermatitis herpetiformis
  • Psoriasis
  • Urticaria

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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