ACQUIRED HEMOPHILIA A REVEALING A PEMPHIGUS: A CASE REPORT

Background: Acquired hemophilia A (AHA) is a rare bleeding disorder that affects one to two cases per million people annually and results from the development of autoantibodies directed against coagulation factor VIII.

Case presentation: We report the case of a 63-year-old woman admitted for surgical management of a knee fracture associated with a large hematoma. Laboratory investigations revealed an isolated prolongation of activated partial thromboplastin time (aPTT) not corrected by normal plasma, with a factor VIII activity of 1% and an inhibitor titer of 12.8 BU/mL. One month later, the patient developed cutaneous and mucosal hemorrhagic bullous lesions, and skin biopsy confirmed the diagnosis of pemphigus. She received recombinant factor VIII and high-dose corticosteroids, followed by cyclophosphamide and rituximab.

Conclusion: This case highlights an exceptional association between acquired hemophilia A and pemphigus, emphasizing the importance of early diagnosis and coordinated multidisciplinary management to prevent severe hemorrhagic complications.