Acquired Haemophilia A Associated with Bullous Pemphigoid

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Sophie Evans
Jake Moss
Hannah Wainman


haemophilia, acquired haemophilia, bullous pemphigoid, bullous skin disease, autoimmune haemophilia, coagulopathy


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.


1. Tiede A, Collins P, Knoebl P, Teitel J, Kessler C, Shima M et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica. 2020;105(7):1791-1801.

2. Obaji S, Rayment R, Collins P. Mycophenolate mofetil as adjunctive therapy in acquired haemophilia A. Haemophilia. 2018;25(1):e59-e65.

3. Collins P, Chalmers E, Hart D, Jennings I, Liesner R, Rangarajan S et al. Diagnosis and management of acquired coagulation inhibitors: a guideline from UKHCDO. British Journal of Haematology. 2013;162(6):758-773.

4. Knoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Journal of Thrombosis and Haemostasis. 2012;10(4):622-631.

5. Abdul-Halim N, Ng H. Bullous pemphigoid is a common associated disorder with acquired haemophilia A. International Journal of Hematology. 2020;113(1):58-62.