Allergy, Asthma & Respiratory Disease

The Korean Academy of Asthma, Allergy and Clinical Immunology (대한천식알레르기학회)
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A case of Stevens-Johnson syndrome with acquired hemophilia complication

  • Hyo-In Rhyou (Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine) ;
  • Jeong Nyeo Lee (Department of Laboratory Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine) ;
  • Sung-Nam Lim (Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine) ;
  • Chan-Sun Park (Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine)
  • Received : 2023.12.22
  • Accepted : 2024.02.27
  • Published : 2024.10.30
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Abstract

Autoimmune diseases have been observed in patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN); however, acquired hemophilia, which can develop owing to autoimmune diseases, has not yet been reported SJS/TEN patients. A 74-yearold male patient, who had been treated for SJS after allopurinol exposure, was referred to our clinic due to hypotension and mucosal erosions. He was suspected to have septic shock due to widespread mucosal involvement caused by SJS, which improved after 1 week of intensive conservative treatment that included antibiotics and systemic corticosteroids. However, a rapid increase in prothrombin time (> 60 seconds) and activated partial thromboplastin time (> 120 seconds) was confirmed, and the mixture of the 2 materials did not improve decreased factor VIII activity (18.7%). The patient was diagnosed with acquired hemophilia, which was probably associated with SJS. In this case, acquired hemophilia, a fatal and rare autoimmune disease, occurred concurrently with SJS.

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References

  1. Lerch M, Mainetti C, Beretta-Piccoli BT, Harr T. Current perspectives on Stevens-Johnson syndrome and toxic epidermal necrolysis. Clin Rev Allergy Immunol 2018;54:147-76.
  2. Kumar R, Das A, Das S. Management of Stevens-Johnson syndrome-toxic epidermal necrolysis: looking beyond guidelines! Indian J Dermatol 2018;63:117-24.
  3. Shanbhag SS, Chodosh J, Fathy C, Goverman J, Mitchell C, Saeed HN. Multidisciplinary care in Stevens-Johnson syndrome. Ther Adv Chronic Dis 2020;11:2040622319894469.
  4. Yang CW, Cho YT, Chen KL, Chen YC, Song HL, Chu CY. Long-term sequelae of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis. Acta Derm Venereol 2016;96:525-9.
  5. Tiede A, Collins P, Knoebl P, Teitel J, Kessler C, Shima M, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica 2020;105:1791-801.
  6. Kessler CM, Knobl P. Acquired haemophilia: an overview for clinical practice. Eur J Haematol 2015;95:36-44.
  7. El Demerdash DM, Ayad A, Tawfik N. Acquired hemophilia A (AHA): understanding, underdiagnosed, undertreated medical condition. Egypt J Intern Med 2022;34:12.
  8. Franchini M, Capra F, Nicolini N, Veneri D, Manzato F, Baudo F, et al. Drug-induced anti-factor VIII antibodies: a systematic review. Med Sci Monit 2007;13:RA55-61.
  9. Konstantinov K, Dolladille C, Gillet B, Alexandre J, Aouba A, Deshayes S, et al. Drug-associated acquired hemophilia A: an analysis based on 185 cases from the WHO pharmacovigilance database. Haemophilia 2023;29:186-92.
  10. Pishko AM, Doshi BS. Acquired hemophilia A: current guidance and experience from clinical practice. J Blood Med 2022;13:255-65.
  11. Fan WY, Zhai QR, Ma QB, Ge HX. Toxic epidermal necrolysis with systemic lupus erythematosus: case report and review of the literature. Ann Palliat Med 2022;11:2144-51.
  12. Sakurai Y, Takeda T. Acquired hemophilia A: a frequently overlooked autoimmune hemorrhagic disorder. J Immunol Res 2014;2014:320674.
  13. Schep SJ, Schutgens REG, Fischer K, Voorberg J, Boes M. Role of regulatory cells in immune tolerance induction in hemophilia A. Hemasphere 2021;5:e557.
  14. Yoshioka N, Suto A, Abe R, Saito N, Murata J, Hayashi-Ujiie I, et al. Disturbed balance in three subpopulations of CD4(+)Foxp3(+) regulatory T cells in Stevents-Johnson syndrome and toxic epidermal necrolysis patients. Clin Immunol 2013;148:89-91.