Fatima Zahraa Sassine, Méria Saci, Oana Pascaru, Sandra Aloui, Michele Sanchez
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 3
Issue: 6
Date of Publication: 2026/06/14
Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by autoantibodies directed against coagulation factor VIII, leading to spontaneous or trauma-related hemorrhage. Unlike congenital hemophilia, AHA predominantly affects the elderly and may be associated with autoimmune diseases, malignancies, pregnancy, or medications. Bullous pemphigoid (BP), a subepidermal autoimmune blistering disorder, has been reported as a potential trigger for AHA due to persistent immune dysregulation.
We report the case of an 84-year-old woman with a history of bullous pemphigoid treated successfully with topical corticosteroids eight months prior, who presented with a rapidly progressive purpuric plaque and uncontrolled bleeding following minor trauma. Laboratory evaluation revealed isolated prolongation of activated partial thromboplastin time (aPTT: 78 seconds) with normal prothrombin time. Mixing studies failed to correct the aPTT, suggesting the presence of an inhibitor. Further workup confirmed markedly reduced factor VIII activity (1%) and the presence of factor VIII inhibitors (18 Bethesda units), establishing the diagnosis of acquired hemophilia A. The patient was treated with recombinant activated factor VII (rFVIIa) for acute bleeding control and immunosuppressive therapy with prednisone and cyclophosphamide for inhibitor eradication. Complete remission was achieved at 12 weeks, with normalization of factor VIII levels and disappearance of inhibitors.
This case highlights the importance of considering acquired hemophilia A in elderly patients presenting with unusual bleeding manifestations, particularly in the context of prior autoimmune disease. The association between bullous pemphigoid and AHA, though rare, underscores the need for vigilance regarding immune-mediated complications in patients with autoimmune blistering disorders.
Dermatology, hematology, acquired hemophilia A, bullous pemphigoid, factor VIII inhibitors, autoimmune disease, elderly, bleeding disorder
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