Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

Abstract

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled. Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

Acquired Factor VIII Inhibitor Presenting as Occult GI Bleeding

“…Other cases highlight acquired factor VIII inhibitor in patients with underlying malignancy, interstitial lung disease, SLE, and bullous pemphigoid, and yet others presented with delayed wound healing. In this case, malignancy was ruled out and the cause of acquired factor VIII inhibitors remains uncertain [ 6 – 9 ].…”

Probable fondaparinux-associated bullous hemorrhagic dermatosis

“…7,10 Hemorrhagic bullae related to an acquired hemophilia (e.g., acquired factor VIII deficiency) are also immune related, and would be expected to change anticoagulation parameters such as international normalized ratio or partial thromboplastin time. 11 There was also no evidence of skin or systemic infection. Given the punch biopsy pathology report, the time correlation with the onset of fondaparinux, selfresolution of the lesions and a lack of plausible alternative explanations, we diagnosed a probable case of fondaparinuxinduced bullous hemorrhagic dermatosis according to the World Health Organization-The Uppsala Monitoring Centre causality assessment system.…”

Challenges facing community-dwelling adults with hemophilia: Implications for community-based adult education and nursing

“…Given that out-of-pocket expenses for hemophilia treatment are catastrophic for patients and their families in the developing world, [36][37][38] future research aimed at increasing advocacy for subsidized treatment costs and initiating government interventions for patients in these regions is very relevant. Additional research in the form of case reports, clinical studies, reviews, and economic evaluation studies are required to provide additional substantiation of promising evidence regarding complications related to this congenital disorder, 39,40 case management and treatment options, [41][42][43] and cost-effectiveness of treatment 22,[44][45][46][47][48][49] for various types of hemophilia. Finally, when providing educational information about hemophilia to communitydwelling adults, it is crucial to understand that these individuals are often confident, pragmatic, and goal-oriented learners and that in general, adults are affective learners, learners-in-transition, integrated learners, and risk takers whose culture is often reflected in their stories, activities, characteristics, and beliefs.…”