Quentin Binet, Catherine Lambert, Laurine Sacre, Stéphane Eeckhoudt, Cédric HermansPublished in the journal : June 2017Category : Hématologie et Dermatologie
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.