Stevens-Johnson Syndrome A Brief Summary

Source Uptodate Articles

Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe idiosyncratic reactions. They are most commonly triggered by medications, which are characterized by fever and mucocutaneous lesions leading to necrosis and sloughing of the epidermis. SJS and TEN are distinguished chiefly by severity and percentage of body surface involved.

Stevens-Johnson syndrome SJS is the less severe condition, in which skin sloughing is limited to less than 10 percent of the body surface. It is characterized by a prodrome of malaise and fever, followed by the rapid onset of erythematous or purpuric macules and plaque. The skin lesions progress to epidermal necrosis and sloughing. Mucosal membranes are affected in 92 to 100 percent of patients, usually at two or more distinct sites (ocular, oral, and genital)

Toxic epidermal necrolysis Toxic epidermal necrolysis (TEN), or Lyell's syndrome, involves sloughing of greater than 30 percent of the body surface area. TEN also begins with a prodrome of fever and malaise, although temperatures are typically higher than those seen with SJS, often exceeding 39 degrees Celsius. Mucous membranes are involved in nearly all cases.

TEN (continued) The skin lesions are widely distributed erythematous macules and patches, although about 50 percent of cases begin with diffuse erythema. The skin lesions progress to full-thickness epidermal necrosis leads. The ultimate appearance of the skin has been likened to that of extensive thermal injury.

SJS/TEN overlap syndrome  SJS/TEN overlap syndrome describes patients with involvement of greater than 10 percent, but less than 30 percent of body surface area.

Etiology Medications are the leading trigger of SJS and TEN in both adults and children. In adults, medications cause 30 to 50 percent of cases of SJS and up to 80 percent of cases of TEN. Infections are the next most common trigger of adult SJS (up to 15 percent). Rare causes of SJS and TEN include vaccinations, systemic diseases, chemical exposure, herbal medicines, and foods.

Medications The following groups of agents are most commonly implicated : Anti-gout agents (especially allopurinol) Antibiotics (sulfonamides >> penicillins > cephalosporins) Antipsychotics and anti-epileptics (including carbamazepine, dilantin,lamotrigine, and phenobarbital) Analgesics and non-steroidal anti-inflammatory agents (especially piroxicam)

HISTORY AND CLINICAL PRESENTATION Drug exposure commonly precedes the onset of symptoms by one to three weeks (average 14 days) in medication-related cases. Reexposure may result in onset of symptoms in as little as 48 hours.

Prodrome  SJS and TEN typically have a prodrome of fever and influenza-like symptoms one to three days before the development of mucocutaneous lesions. Fever is usually higher with TEN, and often exceeds 39 degrees Celsius. Skin tenderness, photophobia, and conjunctival itching or burning may be early symptoms in both conditions.

Clinical presentation The following signs and symptoms, when present early in the course of a drug reaction or illness, should alert clinicians to the possibility of SJS/TEN : Confluent erythema (erythroderma) Facial edema or central facial involvement Skin pain Palpable purpura Skin necrosis Blisters and/or epidermal detachment Mucous membrane erosions and crusting Swelling of tongue

Clinical presentation There may be multiorgan involvement. In the absence of complications, the disorder generally resolves sufficiently that the patient can be discharged from the hospital in two to four weeks.

diagnosis the diagnosis of SJS or TEN would be appropriate in a patient with: A suggestive history of antecedent drug exposure or illness A prodrome of acute-onset febrile illness and malaise Erythematous macules, targetoid lesions, or diffuse erythema progressing to vesicles and bullae Necrosis and sloughing of the epidermis (of varying degrees)

diagnosis The diagnosis of SJS or TEN is clinical. Histologic findings on skin biopsy are supportive, but not independently diagnostic. The differential diagnosis includes: erythema multiforme other types of severe medication reactions severe reactions to bacterial toxins (eg, toxic shock syndrome staphylococcal scalded skin syndrome) Kawasaki disease.

Management Early recognition and immediate withdrawal of any potentially causative agents are critical first steps in the management of SJS/TEN. Multiple specialists should be involved in the care of patients with SJS/TEN when possible, including experts in critical care plastic surgery Dermatology infectious disease Ophthalmology nutrition

To prevent possible sepsis Sepsis is the major cause of death. Sterile handling, infection control measures, topical antibiotic agents, and surveillance cultures of possible sites of superinfection are important components of prevention. 

Supportive care > adjunctive therapies Supportive care should be the primary focus of management of SJS/TEN. Beyond this, there is insufficient evidence to establish the benefit of any adjunctive therapies. Systemic glucocorticoids and intravenous gammaglobulin (IVIG) are commonly used at many centers, although not all. Other treatments: plasmapheresis, thalidomide.

Prognosis The mortality of SJS is 1 to 3 percent, while the mortality of TEN ranges from 25 to 35 percent. Predictors of mortality include older age at onset and greater extent of skin involvement. Long-term sequelae of the skin and eyes are common among survivors.

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