Stevens-Johnson Syndrome Medication

  • Author: C Stephen Foster, MD, FACS, FACR, FAAO; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Sep 23, 2011
 

Medication Summary

The goal of pharmacotherapy in patients with Stevens-Johnson syndrome (SJS) is to reduce morbidity and to prevent complications. No specific drug treatment has been consistently shown to be beneficial in the treatment of SJS. The choice of antibiotic for infectious causes depends on the cause of that infection.

Clinical and laboratory evidence suggesting bloodstream infection mandates the use of antibiotics. The most common organisms include Staphylococcus aureus, Pseudomonas aeruginosa, and Enterobacteriaceae species.[26]

The use of systemic corticosteroids is controversial, but may be useful in high doses early in the disease. Morbidity and mortality actually may increase in association with corticosteroid use. For persistent or recurrent ocular inflammation, patients may benefit from short-term systemic corticosteroids and/or long-term immunosuppressive therapy, which may reduce severity of conjunctivitis and improve prognosis quod visum by reducing damage to ocular surface.

Human intravenous immunoglobulin (IVIG) has been described as both treatment and prophylaxis.

Next

Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Prednisone

 

Prednisone is an immunosuppressant for treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear neutrophil (PMN) activity.

Methylprednisolone (Solu-Medrol, Medrol, Depo-Medrol)

 

Methylprednisolone decreases inflammation by suppressing migration of PMNs and reversing increased capillary permeability.

Previous
Next

Immunosuppressants

Class Summary

These agents inhibit key factors of the immune system, reducing overall immune activity.

Cyclosporine (Sandimmune, Neoral)

 

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease for a variety of organs.

For children and adults, base dosing on ideal body weight.

Cyclophosphamide (Cytoxan, Neosar, Gengraf)

 

Cyclophosphamide is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with the growth of immune cells.

Previous
Next

Immune Globulins

Class Summary

These agents are used to improve clinical and immunologic aspects of the disease. They may decrease autoantibody production, and they may increase solubilization and removal of immune complexes.

Immune globulin intravenous (Gammaplex, Hizentra, Octagam, Privigen, Gammagard S/D)

 

IVIG neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including interferon-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; and promotes remyelination. IVIG may increase IgG levels (by 10%) in the cerebrospinal fluid.

Previous
 
Contributor Information and Disclosures
Author

C Stephen Foster, MD, FACS, FACR, FAAO  Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Rola Ba-Abbad, MBBS  Fellow in Vitreoretinal Division, Department of Ophthalmology, Lund University Hospital, Sweden

Rola Ba-Abbad, MBBS is a member of the following medical societies: American Academy of Ophthalmology and Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Erik Letko, MD  Corneal Consultants of Colorado

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Daniel J Dire, MD, FACEP, FAAP, FAAEM Clinical Professor, Department of Emergency Medicine, University of Texas Medical School at Houston; Clinical Professor, Department of Pediatrics, School of Medicine, University of Texas Health Sciences Center San Antonio

Daniel J Dire, MD is a member of the following medical societies: American Academy of Clinical Toxicology, American Academy of Emergency Medicine, American Academy of Pediatrics, American College of Emergency Physicians, and Association of Military Surgeons of the US

Disclosure: Talecris Biotherapeutics Honoraria Speaking and teaching

Mark T Duffy, MD, PhD Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic

Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience

Disclosure: Allergan - Botox Cosmetic Honoraria Speaking and teaching

Kilbourn Gordon III, MD, FACEP Urgent Care Physician

Kilbourn Gordon III, MD, FACEP is a member of the following medical societies: American Academy of Ophthalmology and Wilderness Medical Society

Disclosure: Nothing to disclose.

John D Halamka, MD, MS Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Catherine V Parrillo, DO, FACOP, FAAP, Retired, Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine

Catherine V Parrillo, DO, FACOP, FAAP, is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Steven J Parrillo, DO, FACOEP, FACEP Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Adjunct Professor, School of Health and Science, Philadelphia University; Medical Director and Faculty, Disaster Medicine and Management Masters Program, Philadelphia University

Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Matthew M Rice, MD, JD, FACEP Senior Vice President, Chief Medical Officer, Northwest Emergency Physicians of TeamHealth; Assistant Clinical Professor of Medicine, University of Washington School of Medicine

Matthew M Rice, MD, JD, FACEP is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Washington State Medical Association

Disclosure: Team Health Salary Employment

Erik D Schraga, MD Staff Physician, Department of Emergency Medicine, Mills-Peninsula Emergency Medical Associates

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. Roujeau JC. Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme. J Dermatol. Nov 1997;24(11):726-9. [Medline].

  2. French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int. Mar 2006;55(1):9-16. [Medline].

  3. Ahmed AR, Dahl MV. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mucocutaneous blistering diseases. Arch Dermatol. Aug 2003;139(8):1051-9. [Medline].

  4. Assier-Bonnet H, Aractingi S, Cadranel J, Wechsler J, Mayaud C, Saiag P. Stevens-Johnson syndrome induced by cyclophosphamide: report of two cases. Br J Dermatol. Nov 1996;135(5):864-6. [Medline].

  5. De Rojas MV, Dart JK, Saw VP. The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy. Br J Ophthalmol. Aug 2007;91(8):1048-53. [Medline]. [Full Text].

  6. Morel E, Escamochero S, Cabañas R, Díaz R, Fiandor A, Bellón T. CD94/NKG2C is a killer effector molecule in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Allergy Clin Immunol. Mar 2010;125(3):703-10, 710.e1-710.e8. [Medline].

  7. Inachi S, Mizutani H, Shimizu M. Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration. Arch Dermatol. Jul 1997;133(7):845-9. [Medline].

  8. Foster CS, Fong LP, Azar D, Kenyon KR. Episodic conjunctival inflammation after Stevens-Johnson syndrome. Ophthalmology. Apr 1988;95(4):453-62. [Medline].

  9. Murata J, Abe R, Shimizu H. Increased soluble Fas ligand levels in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis preceding skin detachment. J Allergy Clin Immunol. Nov 2008;122(5):992-1000. [Medline].

  10. French LE, Trent JT, Kerdel FA. Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome: our current understanding. Int Immunopharmacol. Apr 2006;6(4):543-9. [Medline].

  11. Hillebrand-Haverkort ME, Budding AE, bij de Vaate LA, van Agtmael MA. Mycoplasma pneumoniae infection with incomplete Stevens-Johnson syndrome. Lancet Infect Dis. Oct 2008;8(10):586-7. [Medline].

  12. Sendi P, Graber P, Lepère F, Schiller P, Zimmerli W. Mycoplasma pneumoniae infection complicated by severe mucocutaneous lesions. Lancet Infect Dis. Apr 2008;8(4):268. [Medline].

  13. Hällgren J, Tengvall-Linder M, Persson M, Wahlgren CF. Stevens-Johnson syndrome associated with ciprofloxacin: a review of adverse cutaneous events reported in Sweden as associated with this drug. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S267-9. [Medline].

  14. Mockenhaupt M, Messenheimer J, Tennis P, Schlingmann J. Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. Neurology. Apr 12 2005;64(7):1134-8. [Medline].

  15. Metry DW, Lahart CJ, Farmer KL, Hebert AA. Stevens-Johnson syndrome caused by the antiretroviral drug nevirapine. J Am Acad Dermatol. Feb 2001;44(2 Suppl):354-7. [Medline].

  16. Halevy S, Ghislain PD, Mockenhaupt M, et al. Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol. Jan 2008;58(1):25-32. [Medline].

  17. Belkahia A, Hillaire-Buys D, Dereure O, Guillot B, Raison-Peyron N. Stevens-Johnson syndrome due to mirtazapine - first case. Allergy. Oct 2009;64(10):1554. [Medline].

  18. Salama M, Lawrance IC. Stevens-Johnson syndrome complicating adalimumab therapy in Crohn's disease. World J Gastroenterol. Sep 21 2009;15(35):4449-52. [Medline]. [Full Text].

  19. Kardaun SH, Jonkman MF. Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol. 2007;87(2):144-8. [Medline].

  20. Fernando SL, Broadfoot AJ. Prevention of severe cutaneous adverse drug reactions: the emerging value of pharmacogenetic screening. CMAJ. Mar 23 2010;182(5):476-80. [Medline]. [Full Text].

  21. Hynes AY, Kafkala C, Daoud YJ, Foster CS. Controversy in the use of high-dose systemic steroids in the acute care of patients with Stevens-Johnson syndrome. Int Ophthalmol Clin. Fall 2005;45(4):25-48. [Medline].

  22. Khalili B, Bahna SL. Pathogenesis and recent therapeutic trends in Stevens-Johnson syndrome and toxic epidermal necrolysis. Ann Allergy Asthma Immunol. Sep 2006;97(3):272-80; quiz 281-3, 320. [Medline].

  23. Meth MJ, Sperber KE. Phenotypic diversity in delayed drug hypersensitivity: an immunologic explanation. Mt Sinai J Med. Sep 2006;73(5):769-76. [Medline].

  24. Strom BL, Carson JL, Halpern AC, et al. A population-based study of Stevens-Johnson syndrome. Incidence and antecedent drug exposures. Arch Dermatol. Jun 1991;127(6):831-8. [Medline].

  25. Bastuji-Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. Aug 2000;115(2):149-53. [Medline].

  26. de Prost N, Ingen-Housz-Oro S, Duong T, et al. Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. Medicine (Baltimore). Jan 2010;89(1):28-36. [Medline].

  27. Vera LS, Gueudry J, Delcampe A, et al. In vivo confocal microscopic evaluation of corneal changes in chronic Stevens-Johnson syndrome and toxic epidermal necrolysis. Cornea. May 2009;28(4):401-7. [Medline].

  28. Shammas MC, Lai EC, Sarkar JS, Yang J, Starr CE, Sippel KC. Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. Am J Ophthalmol. Feb 2010;149(2):203-213.e2. [Medline].

  29. Tseng SC. Acute management of Stevens-Johnson syndrome and toxic epidermal necrolysis to minimize ocular sequelae. Am J Ophthalmol. Jun 2009;147(6):949-51. [Medline].

  30. Paquet P, Paquet F, Al Saleh W, Reper P, Vanderkelen A, Piérard GE. Immunoregulatory effector cells in drug-induced toxic epidermal necrolysis. Am J Dermatopathol. Oct 2000;22(5):413-7. [Medline].

  31. Sotozono C, Ueta M, Koizumi N, et al. Diagnosis and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications. Ophthalmology. Apr 2009;116(4):685-90. [Medline].

  32. Sotozono C, Ueta M, Kinoshita S. Systemic and local management at the onset of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications. Am J Ophthalmol. Feb 2010;149(2):354; author reply 355. [Medline].

  33. Araki Y, Sotozono C, Inatomi T, et al. Successful treatment of Stevens-Johnson syndrome with steroid pulse therapy at disease onset. Am J Ophthalmol. Jun 2009;147(6):1004-11, 1011.e1. [Medline].

  34. Koh MJ, Tay YK. Stevens-Johnson syndrome and toxic epidermal necrolysis in Asian children. J Am Acad Dermatol. Jan 2010;62(1):54-60. [Medline].

  35. Patterson R, Dykewicz MS, Gonzalzles A, et al. Erythema multiforme and Stevens-Johnson syndrome. Descriptive and therapeutic controversy. Chest. Aug 1990;98(2):331-6. [Medline].

  36. Power WJ, Ghoraishi M, Merayo-Lloves J, Neves RA, Foster CS. Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology. Nov 1995;102(11):1669-76. [Medline].

  37. Hebert AA, Bogle MA. Intravenous immunoglobulin prophylaxis for recurrent Stevens-Johnson syndrome. J Am Acad Dermatol. Feb 2004;50(2):286-8. [Medline].

  38. Schneck J, Fagot JP, Sekula P, et al. Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: A retrospective study on patients included in the prospective EuroSCAR Study. J Am Acad Dermatol. Jan 2008;58(1):33-40. [Medline].

  39. Pehr K. The EuroSCAR study: cannot agree with the conclusions. J Am Acad Dermatol. Nov 2008;59(5):898-9; author reply 899-900. [Medline].

  40. Power WJ, Saidman SL, Zhang DS, et al. HLA typing in patients with ocular manifestations of Stevens-Johnson syndrome. Ophthalmology. Sep 1996;103(9):1406-9. [Medline].

Previous
Next
 
A patient with severe eye involvement associated with Stevens-Johnson syndrome. Note corneal neovascularization and conjunctivalization of the ocular surface.
Epithelial defect of the cornea with neovascularization and surface conjunctivalization.
Note extensive sloughing of epidermis from Stevens-Johnson syndrome. Courtesy of David F. Butler, MD.
Sheetlike desquamation on the foot in a patient with toxic epidermal necrolysis. Courtesy of Robert Schwartz, MD.
Hemorrhagic crusting of the mucous membranes in toxic epidermal necrolysis. Similar lesions are seen in Stevens-Johnson syndrome. Courtesy of Robert Schwartz, MD.
Note early cutaneous slough with areas of violaceous erythema.
Extensive sloughing on the face.
Note the presence of both 2-zoned atypical targetoid lesions and bullae.
Extensive blistering and sloughing on the back.
Extensive sloughing on the back.
Note extensive sloughing.
Low-power view showing full-thickness epidermal necrosis.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.