Stevens-Johnson Syndrome Medication
- Author: C Stephen Foster, MD, FACS, FACR, FAAO; Chief Editor: Hampton Roy Sr, MD more...
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.
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.
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.
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.
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].
French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int. Mar 2006;55(1):9-16. [Medline].
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].
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].
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].
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].
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].
Foster CS, Fong LP, Azar D, Kenyon KR. Episodic conjunctival inflammation after Stevens-Johnson syndrome. Ophthalmology. Apr 1988;95(4):453-62. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Kardaun SH, Jonkman MF. Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol. 2007;87(2):144-8. [Medline].
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].
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].
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].
Meth MJ, Sperber KE. Phenotypic diversity in delayed drug hypersensitivity: an immunologic explanation. Mt Sinai J Med. Sep 2006;73(5):769-76. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Hebert AA, Bogle MA. Intravenous immunoglobulin prophylaxis for recurrent Stevens-Johnson syndrome. J Am Acad Dermatol. Feb 2004;50(2):286-8. [Medline].
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].
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].
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].

