Bullous pemphigoid and hypercoagulability: a review

Abstract

Introduction: Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by autoantibodies against hemidesmosomal proteins in the basal membrane zone. The presence of a high incidence of thrombotic events has led to the identification of a hypercoagulable state in BP patients.

Areas covered: This review highlights the interactions between coagulation and immune-inflammatory responses based on the currently available literature, as well as individual changes in characteristic coagulation parameters in BP. This review is based on publications up to August 2024 that were retrieved by a selective search in the PubMed database.

Expert opinion: The hypercoagulable state and bullous pemphigoid (BP) have a reciprocally enhancing effect on each other. For clinicians, it is crucial to closely monitor the fluctuations in circulating coagulation markers among BP patients, such as D-dimer, fibrinogen, and fibrin degradation products (FDP). Furthermore, considering the interplay between coagulation and immune-inflammatory responses in BP, targeting the shared pathways in treatment strategies could be beneficial for patients who exhibit both BP and a hypercoagulable state.

Keywords: Bullous pemphigoid; coagulation parameter; hypercoagulability; thrombo-prophylaxis; thrombosis.