Severe bleeding in a case of acquired factor VIII inhibitor associated with generalized pustular psoriasis

Antibodies against factor VIII occur in about 15-35% of hemophilia A patients and induce refractoriness to factor VIII substitution. In very rare cases, factor VIII inhibitors also develop in association with various autoimmune and chronic inflammatory diseases, hematologic malignancies, solid tumors, certain drugs, dermatologic conditions, and in puerperium. In the majority of cases, the clinical course is characterized by severe hemorrhages. Strategies to treat such inhibitors are controversial. We present the case of a patient with generalized pustular psoriasis who developed severe bleeding complications due to an acquired factor VIII inhibitor, after lymph node and skin biopsy. Initial treatment with high doses of human factor VIII concentrate was unsuccessful. Hemorrhage was partially controlled by the administration of recombinant activated factor VII. Subsequently, the human factor VIII was reinitiated in association with immunosuppressive therapy with prednisone. Within 8 weeks this combined modality achieved a complete remission of bleeding, a normalization of coagulation parameters and a complete disappearance of the inhibitor. Our case illustrates that, although the clinical course in patients with acquired factor VIII inhibitor is not predictable and the inhibitor may disappear spontaneously, combined modality with replacement and immunosuppressive therapy should be considered for patients with severe and refractory hemorrhages.