• Sara Jasmine Al-shami Department of Dermatology, ”Elias” University Emergency Hospital, Bucharest, Romania
  • Adelina Popa UMF ”Carol Davila”& Department of Dermatology, Elias University Emergency Hospital, Bucharest
  • Raluca Gabriela Miulescu ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania & Department of Pediatrics, ”Grigore Alexandrescu” Children´s Clinical Emergency Hospital, Bucharest, Romania & Department of Dermatology, Valenii de Munte Country Hospital, Romania
  • Mihai Cristian Dumitrașcu ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania & Department of Obstetrics&Gynecology, University Emergency Hospital, Bucharest, Romania
  • Claudia Mehedințu ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania & Department of Obstetrics&Gynecology, ”Nicolae Malaxa” Clinical Hospital, Bucharest, Romania
  • Florica Sandru ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania &Department of Dermatology, ”Elias” University Emergency Hospital, Bucharest, Romania
Keywords: Hemophilia A, Acquired hemophilia, pemphigus vulgaris


Pemphigus vulgaris is a rare autoimmune disorder involving painful blisters and erosions on the skin and mucous membranes caused by antibodies directed against desmoglein 1 and 3. Acquired hemophilia A (AHA) is a relatively rare coagulation disorder caused by the spontaneous presence of anti-factor VIII immunoglobulin G antibodies (IgG1 and IgG4), known to cause spontaneous clinically significant hemorrhages into the skin and soft tissues in patients with no previous known diagnosis of bleeding disorders. This coagulopathy has been described in conjunction with autoimmune bullous skin diseases, malignancies, and drug reactions. Only a few cases of such interaction have been documented, we herein report a case of a 50-year-old woman with a history of pemphigus vulgaris who has developed extensive ecchymosis on her right arm and right calf. A diagnosis of AHA was made considering the modified coagulation tests. Initiation of treatment with factor VIII led to clinical improvement, however, the patient stopped showing up for further follow-ups.



[1] D.M. Peraza, ”Blistering diseases-Pemphigus vulgaris” in Skin disorders, MSD Manual Consumer Version, 2021.
[2] S. Shetty, M. Bhave, K. Ghosh, ”Acquired hemophilia A: Diagnosis, aetiology, clinical spectrum and treatment options”, Autoimmunity Reviews, vol. 10, no. 6, pp. 311-316, 2011.
[3] P. Knoebl, P. Marco, F. Baudo et al., “Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2),” Journal of Thrombosis and Haemostasis, vol. 10, no. 4, pp. 622–631, 2012.
[4] N.H. Kim, A. Yang, R.S. Kisner. ”Antibody response in endemic pemphigus foliaceus”, J Invest Dermatol, vol. 128, pp. 498, 2008.
[5] O. Arakaki, Y. Yamamoto, R. Awazawa, K. Nonaka, ”Case of linear immunoglobulin A bullous dermatosis associated with acquired haemophilia”, J Dermatol, vol. 35, pp. 437-446, 2008.
[6] T. Nagano, M. Tani, Y. Hiramatsu, et al., ”A case of epidermolysis bullosa acquisita with bleeding tendency due to factor VIII inhibitor (acquired haemophilia)” Br J Dermatol, vol. 151, pp. 716-717, 2004.
[7] F.J. Halbertsma, P.W. van der Linden, E.P. Mauser-Bunschoten ”A patient with acquired haemophilia A and pemphigus”, Neth J Med, vol. 52, pp. 190-192, 1998.
[8] A. Shimizu, A. Ishiko, T. Ota, K. Tsunoda, M. Amagai, T. Nishikawa, ”IgG binds to desmoglein 3 in desmosomes and causes a desmosomal split without keratin retraction in a pemphigus mouse model”, J Invest Dermatol, vol. 122, pp. 1145-1153, 2004.
[9] K. Kridin, S.Z. Sagi, R. Bergman, ”Mortality and Cause of Death in Patients with Pemphigus” Acta Derm Venereol, vol. 97, no. 5, pp. 607-611, May 2017.
[10] D.Y. Hsu, J. Brieva, A.A. Sinha, S.M. Langan, J.I. Silverberg, ”Comorbidities and inpatient mortality for pemphigus in the U.S.A”, Br J Dermatol, vol. 174, no. 6, pp. 1290-1298, 2016.
[11] O. Ishikawa, A. Tamura, K. Ohnishi, Y. Miyachi, ”Pemphigus vulgaris associated with acquired hemophilia A due to factor VIII inhibitor”, Acta Dermato-Venereologica, vol. 73, no. 3, pp. 229-230, 1993.
[12] F. Baudo, T. Caimi, F. De Cataldo, ”Diagnosis and treatment of acquired haemophilia”, Haemophilia, vol. 16, pp. 102-106, 2010.
[13] A. Huth-Kuhne, F. Baudo, P. Collins, et al., ”International recommendations on the diagnosis and treatment of patients with acquired haemophilia A”, Haematologica, vol. 94, pp. 566-575, 2009.
[14] R. L. Bitting, S. Bent, Y. Li, J. Kohlwes, ”The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis”, Blood Coagul Fibrinolysis, vol. 20, no. 7, pp. 517-523, Oct. 2009.
[15] D.W. Sborov, G.M. Rodgers, ”Acquired hemophilia a: a current review of autoantibody disease” Clin Adv Hematol Oncol, vol. 10, no. 1, pp. 19-27, Jan. 2012.
[16] Y. Zeng, R. Zhou, X. Duan, D. Long, ”Rituximab for eradicating inhibitors in people with acquired haemophilia A” The Cochrane Database of Systematic Reviews, vol. 7, no. 7, Cd011907, 2016.
[17] M. Franchini, P. M. Mannucci, “Inhibitor eradication with rituximab in hemophilia: where do we stand?” British Journal of Haematology, vol. 165, no. 5, pp. 600–608, 2014.
[18] P.W. Collins, S. Hirsch, T. P. Baglin et al., “Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation”, Blood, vol. 109, no. 5, pp. 1870–1877, 2007.
How to Cite
Al-shami, S., Popa, A., Miulescu, R., Dumitrașcu, M., Mehedințu, C., & Sandru, F. (2022). ACQUIRED HEMOPHILIA A IN A PATIENT WITH PEMPHIGUS VULGARIS -CASE REPORT. Romanian Journal of Clinical Research, 4(2).