Abstract
Acquired hemophilia is a rare disease resulting from autoantibodies against endogenous factor VIII (FVIII), which associates with bleeding and a high mortality rate. The pathophysiology is still unclear. Recent studies suggest genetic and environmental factors trigger the breakdown of immune tolerance. We report a 77-year-old Taiwanese man presented with multiple ecchymoses and some hemorrhagic blisters three weeks after SARS-CoV-2 mRNA (Moderna) vaccination. Isolated activated partial thromboplastin time (aPTT) prolongation was found. Acquired hemophilia A (AHA) was confirmed by low factor VIII (FVIII) activity and high titer of FVIII inhibitor. The pathohistology of skin biopsy further supported the concomitant diagnosis of bullous pemphigoid. To date, 6 cases of acquired hemophilia A following SARS-CoV-2 mRNA vaccination were reported worldwide. We reviewed and summarized the characteristics of these cases. We also discussed the rare finding of concomitant acquired hemophilia A and bullous pemphigoid. Bullous pemphigoid results from autoantibody against epithelial basement membrane zone of skin. In this article, we proposed possibility of SARS-CoV-2 mRNA vaccine associated autoimmunity against FVIII and epithelial basement membrane zone.
Keywords: Acquired hemophilia A; Bullous pemphigoid; SARS-CoV-2; mRNA vaccine.
【저자키워드】 SARS-CoV-2, mRNA vaccine., bullous pemphigoid, Acquired hemophilia A, 【초록키워드】 Autoimmunity, mRNA vaccination, vaccination, mRNA vaccine, Genetic, Diagnosis, Characteristics, pathophysiology, mRNA, bleeding, membrane, inhibitor, rare disease, epithelial, Moderna, autoantibody, hemophilia, acquired hemophilia, Hemorrhagic, immune tolerance, zone, high mortality rate, FVIII, Blister, recent, environmental factor, pemphigoid, resulting, reported, supported, activated, Isolated, Acquired, AHA, bullous, 【제목키워드】 mRNA vaccination, hemophilia, pemphigoid, bullous,