Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disorder caused by circulating autoantibodies against factor VIII (FVIII). In approximately 50% of the patients, the condition is associated with autoimmune diseases, cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid (BP) is a chronic autoimmune subepidermal blistering disease associated with tissue-bound and circulating autoantibodies against BP antigens 180 (BP180) and 230 (BP230). AHA-associated BP has a high mortality rate; hence, the understanding of this disease must improve.

A 69-year-old man presented with erythema, blisters, blood blisters, and crusts accompanied by severe pruritus for more than 20 days, and ecchymosis and swelling on his left upper arm for 3 days. Pathological examination revealed a subepidermal blister that contained eosinophils. Laboratory tests showed that the BP180 autoantibody levels had increased, isolated activated partial thromboplastin time was notably prolonged (115.6 s), and coagulation FVIII activity was extremely low (< 1.0%). Furthermore, the FVIII inhibitor titer had greatly increased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospital for effective treatment; however, he died after 2 days.

AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effective treatment are necessary.

Hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare disease in which patients present with varying degrees of bleeding and positive lupus anticoagulant with reduced prothrombin on laboratory tests. This article reports a case of HLAS in a middle-aged woman with recurrent gingival bleeding and epistaxis as the first presentation. After admission, tests revealed prolonged prothrombin time (PT), activated partial thromboplastin time (APTT), and reduced coagulation factor II activity, and positive lupus anticoagulant (LA). Meanwhile, the patient had symptoms of dry mouth and dry eyes for a long time, and the examination of autoantibodies, tear secretion test and salivary gland emission computed tomography (ECT) were consistent with the diagnosis of Sjogren's syndrome. The final diagnosis was HLAS secondary to Sjogren's syndrome. After treatment with methylprednisolone and cyclophosphamide, the coagulation disorder gradually improved, and no recurrent bleeding occurred. HLAS is a rare clinical case, which reminds medical staff to be alert to the possibility of HLAS when encountering patients with unexplained prolonged APTT and PT and positive lupus anticoagulant.