Acquired Haemophilia A Associated with Bullous Pemphigoid

Abstract

We would like to submit this manuscript for an image report entitled ‘Acquired haemophilia A associated with bullous pemphigoid’. We present the case of an 81-year-old female first investigated for a blistering dermatosis and diagnosed with bullous pemphigoid, who several weeks later developed extensive atraumatic ecchymosis with a grossly prolonged activated partial thromboplastin time consistent with acquired haemophilia. There is a known association between bullous pemphigoid and autoimmune haemophilia but less than 50 cases have been reported, and as such it remains rare. However, it is a potentially life-threatening complication of bullous pemphigoid and should be suspected in patients presenting with mucocutaneous bleeding. We describe the investigations leading to this diagnosis, summarise the management of acquired haemophilia, and illustrate the case with clinical photographs.