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 enterobacteria species. [32]
The use of systemic corticosteroids is controversial, but may be useful in high doses early in the disease. Morbidity and mortality may actually 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.
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A patient with severe eye involvement associated with Stevens-Johnson syndrome. Note corneal neovascularization and conjunctivalization of the ocular surface.
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Epithelial defect of the cornea with neovascularization and surface conjunctivalization.
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Note extensive sloughing of epidermis from Stevens-Johnson syndrome. Courtesy of David F. Butler, MD.
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Sheetlike desquamation on the foot in a patient with toxic epidermal necrolysis. Courtesy of Robert Schwartz, MD.
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Hemorrhagic crusting of the mucous membranes in toxic epidermal necrolysis. Similar lesions are seen in Stevens-Johnson syndrome. Courtesy of Robert Schwartz, MD.
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Note early cutaneous slough with areas of violaceous erythema.
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Extensive sloughing on the face.
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Note the presence of both 2-zoned atypical targetoid lesions and bullae.
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Extensive blistering and sloughing on the back.
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Extensive sloughing on the back.
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Note extensive sloughing.
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Low-power view showing full-thickness epidermal necrosis.
