Hemorrhagic bullous pemphigoid: an infrequent presentation of acquired hemophilia secondary to Dipeptidyl-dipeptidase-4 inhibitors

Resumen

Introduction: Acquired hemophilia is a rare disease caused by neutralizing autoantibodies against factor VIII that has been associated with autoimmune blistering diseases such as bullous pemphigoid. Case: We present an 82-year-old patient with a history of bullous pemphigoid secondary to the use of dipeptidyl-dipeptidase-4 inhibitors (vildagliptin) who presented with macroscopic hematuria, multiple violaceous hematomas on the face and upper extremities. After the study, it was diagnosed as acquired hemophilia associated with bullous pemphigoid due to dipeptidyl-dipeptidase-4 inhibitors. Discussion: Acquired hemophilia is a rare complication of bullous pemphigoid. The main hypothesis explaining this association is the development of autoantibody cross-reactivity between homologous epitopes on factor VIII and BP-180. Dipeptidyl-dipeptidase-4 inhibitors would cause the formation of IgG4 antibodies against BP-180, and these antibodies also recognize factor VIII. Treatment of acquired hemophilia is based on case reports or case series. It is focused on eradicating autoantibodies with immunosuppressive drugs such as corticosteroids, cyclosporine or target therapies such as rituximab.