Case Report

Acquired Hemophilia in Associaton With Pemphigus Vulgaris;An Uncommon Coexistence: A Case Report

Hemophilia and pemphigus

Abstract

Acquired Hemophilia (AHA) is a relatively rare disease that occurs in patients with no previous family history of hemophilia. The spontaneous development of autoantibodies (IgG1 and IgG4) against factor VIII has been reported as the most probable cause of AHA. AHA has been reported in association with other conditions, including some autoimmune bullous skin diseases, such as bullous pemphigoid, pemphigus vulgaris, and pemphigus foliaceous. To the best of our knowledge, only 21 cases of AHA with skin autoimmune bullous diseases have been reported so far. Herein, we report a 63-year-old male with a previous history of pemphigus vulgaris who developed large ecchymotic areas on his lower abdomen and forearms after the second infusion of rituximab. Based on coagulation factors evaluation, he was diagnosed with AHA. Treatment with factor VII led to the improvement in his coagulation status, but unfortunately, he passed away because of inferior wall myocardial infarction four days later.

[1] Shety S, Bhave M, Ghosh K. Acquired hemophilia A: Diagnosis, aetology, clinical spectrum and treatment optons. Autoimmunity Reviews. 2011; 10(6):311-6. [DOI:10.21542/gcsp.2016.10] [PMID]
[2] Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired haemophilia: Review and meta-analysis focused on therapy and prognostc factors. Britsh Journal of Haematology. 2003; 121(1):21-35. [DOI:10.21542/gcsp.2016.10] [PMID]
[3] Ishikawa O, Tamura A, Ohnishi K, Miyachi Y. Pemphigus vulgaris associated with acquired hemophilia A due to factor VIII inhibitor. Acta Dermato-Venereologica. 1993; 73(3):229-30. [PMID]
[4] Makita S, Aoki T, Watarai A, Aida A, Katayama T, Danbara M, et al. Acquired hemophilia associated with autoimmune bullous diseases: A report of two cases and a review of the literature. Internal Medicine. 2013; 52(7):807-10. [DOI:10.21542/gcsp.2016.10] [PMID]
[5] Wernham AG, Peng C, Bailiļ¬€ B, Ilchyshyn A. Pemphigus foliaceus and acquired haemophilia: A rare but important associaton with lifethreatening consequences. Clinical and Experimental Dermatology. 2018; 43(7):825-8. [DOI:10.21542/gcsp.2016.10] [PMID]
[6] Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T. The clinical phenotype of pemphigus is defned by the ant-desmoglein autoantbody profle. Journal of the American Academy of Dermatology. 1999; 40(2 Pt 1):167-70. [PMID] [DOI:10.21542/gcsp.2016.10]
[7] Arakaki O, Yamamoto Y, Awazawa R, Nonaka K, Taira K, Asato Y, et al. Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia. The Journal of Dermatology. 2008; 35(7):437-46. [DOI:10.21542/gcsp.2016.10] [PMID]
[8] Zeng Y, Zhou R, Duan X, Long D. Rituximab for eradicatng inhibitors in people with acquired haemophilia A. The Cochrane Database of Systematc Reviews. 2016; 7(7):Cd011907. [PMID]
Files
IssueVol 6 No 3 (2021): May-June QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/crcp.v6i3.7124
Keywords
Pemphigus Pemphigus vulgaris Hemophilia

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Balighi K, Daneshpazhooh M, Mahmoudi H, Shakoei S, Aryanian Z, Tavakolpour S, Azizpour A. Acquired Hemophilia in Associaton With Pemphigus Vulgaris;An Uncommon Coexistence: A Case Report. CRCP. 2021;6(3):90-93.