Management of an elderly male patient with subconjunctival bleeding associated with pemphigus in the emergency department

A 82-year-old man suffering from pemphigus and Parkinson’s disease presented at the emergency department of the Misericordia Hospital in Grosseto (Tuscany, Italy) with subconjunctival bleeding. Laboratory blood tests showed a prolonged activated thromboplastin time (aPTT), with normal prothrombin time (PT) and slightly reduced haemoglobin. The negative family and personal history of haemorrhagic disease rose the suspicion of the presence of an acquired inhibitor. The patient was referred for further diagnostics to the University Hospital of Siena where second level tests were performed at the Coagulation Unit. The aPTT mixture test revealed a non-correction both at room temperature and, more markedly, after incubation at 37°C for 2h, confirming the presence of an intrinsic pathway inhibitor. Among the measured factors, only the activity level of factor VIII was extremely low. The titration of FVIII inhibitor confirmed the diagnosis of Acquired Haemophilia A (AHA).