Autoimmune bullous diseases (AIBDs) are rare, chronic conditions characterized by blisters and erosions on the skin and mucous membranes. Acquired hemophilia A (AHA) is a rare bleeding disorder in which autoantibodies target coagulation factor VIII, often associated with other autoimmune diseases. However, the simultaneous occurrence of AIBD and AHA is rare, with limited understanding of their combined impacts. We aimed to synthesize the available evidence and provide insights into clinical manifestations, treatment strategies, and survival outcomes of such patients. We searched for relevant literature in four databases: PubMed, Web of Science, EMBASE, and Scopus, and combined patient information from our hospital to provide a comprehensive analysis. A total of 80 patients were included. Through log-rank tests, older age (p = 0.03) and bullous pemphigoid (BP) (p = 0.047) were associated with shorter survival time. In the multivariate Cox proportional hazards regression model, mortality was only associated with older age (HR: 1.13, p = 0.02). Patients presenting both AIBD and AHA exhibit intricate clinical presentations and are at risk of severe, potentially fatal outcomes. Clinicians should remain vigilant regarding bleeding in AIBD patients, particularly those with BP and those in the geriatric population.