Bullous pemphigoid is a common associated disorder with acquired haemophilia A.

Abstract

Although the estimated incidence of acquired haemophilia A (AHA) in Singapore is similar to those reported in the literature, we have observed differences in the frequency of their associated diseases, particularly bullous pemphigoid (BP). We investigated the actual incidence of BP among our AHA cohort, their clinical characteristics and treatment outcomes. 6 out of 37 (16%) patients with AHA had BP, making it the most common underlying disorder in our cohort. The median age at diagnosis of AHA was 76years old with a female preponderance. Most patients had their AHA diagnosed after BP with the median time between BP to AHA diagnosis being 107.5days. Initial haemostasis was achieved, and factor VIII inhibitor was eliminated in all patients with a median time of disappearance being 52days. Two patients had recurrence of their factor VIII inhibitor during the tapering of their immunosuppression. There was no relationship between the relapse of BP and AHA. This study suggests that BP is a common association with AHA. These patients respond well to bypassing agent and immunosuppression. However, they have a higher recurrence of factor VIII inhibitor and should be monitored closely for relapse during the tapering period of their immunosuppression.