The differential diagnosis of isolated prolongation of the activated partial thromboplastin time (aPTT) is vast. An infrequent but severe cause is acquired hemophilia A (AHA) which is due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, post-partum, auto-immune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. AHA was completely cured and BP well-controlled with a short-term treatment consisting of methylprednisolone and rituximab.
Key Words
Acquired hemophilia, bullous pemphigoid
What is already known about the topic?
The differential diagnosis of TCA elevations is broad. Acquired haemophilia, although rare, should be systematically evoked because of its hemorrhagic risk. Its treatment will consist of corticosteroids and other immunosuppressants if necessary.
What does this article bring up for us?
This article provides a concise and systematic diagnostic approach to any isolated elevation of TCA. Acquired haemophilia is frequently associated with autoimmune pathologies, including bullous pemphigoid. We discuss pathogenic hypotheses for this association.