Highlights
- •Acquired Hemophilia A (AHA) can be rarely associated with Bullous Pemphigoid (BP).
- •AHA management should consider patient’s frailty.
- •Immunosuppression should be carefully tapered to avoid FVIII inhibitors relapse.
Abstract
Acquired Hemophilia A (AHA) is a rare autoimmune disorder, caused by the development
of circulating autoantibodies against coagulation factor VIII (FVIII). AHA is associated
with bullous pemphigoid in 2% of patients. We report a case of a 74-year-old man admitted
with anemia and a tense subcutaneous and muscular hematoma in the right thigh. Blood
analysis confirmed AHA. The patient had a recent diagnosis of bullous pemphigoid.
Response to bypass agents and corticosteroids was good with bleeding control and normalization
of FVIII and negative autoantibodies, respectively. In a 3-month follow-up period
after tapering and stopping prednisolone, a relapse occurred, and immunosuppression
was reinitiated. An early diagnosis and effective treatment in AHA are essential to
reduce morbimortality. A careful tapering of immunosuppression is important to minimize
FVIII inhibitor recurrence, as observed in this case.
Keywords
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References
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Article info
Publication history
Published online: November 29, 2021
Accepted:
November 27,
2021
Received in revised form:
November 22,
2021
Received:
October 2,
2021
Identification
Copyright
© 2021 Elsevier Ltd. All rights reserved.
