Stevens-Johnson Syndrome1

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Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS) is an immune-complex?mediated hypersensitivity complex that is a severe expression of erythema multiforme. It is now known also as erythema multiforme major. SJS typically involves the skin and the mucous membranes. While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis. SJS is a serious systemic disorder with the potential for severe morbidity and even death.

SJS is an immune-complex?mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies. Cocaine recently has been added to the list of drugs capable of producing the syndrome. In up to half of cases, no specific etiology has been identified.
In the US: Cases tend to have a propensity for the early spring and winter.
Internationally: SJS occurs with a worldwide distribution similar in etiology and occurrence to that in the US.

Patients with severe SJS die in 3-15% of cases.
Lesions may continue to erupt in crops for as long as 2-3 weeks. Mucosal pseudomembrane formation may lead to mucosal scarring and loss of function of the involved organ system. Esophageal strictures may occur when extensive involvement of the esophagus exists. Mucosal shedding in the tracheobronchial tree may lead to respiratory failure.
Ocular sequelae may include corneal ulceration and anterior uveitis. Blindness may develop secondary to severe keratitis or panophthalmitis in 3-10% of patients. Vaginal stenosis and penile scarring have been reported. Renal complications are rare.
Race: A Caucasian predominance has been reported.
Sex: Male-to-female ratio is 2:1.
Age: Most patients are in the second to fourth decade of their lives; however, cases have been reported in children as young as 3 months.

Typically, the disease process begins with a nonspecific upper respiratory tract infection.
This usually is part of a 1- to 14-day prodrome during which fever, sore throat, chills, headache, and malaise may be present.
Vomiting and diarrhea occasionally are noted as part of the prodrome.
Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions typically are nonpruritic.
A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases.
Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink.
Patients with genitourinary involvement may complain of dysuria or an inability to void.
A history of a previous outbreak of SJS or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.
Typical symptoms are as follows:
Cough productive of a thick purulent sputum

The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema.
The center of these lesions may be vesicular, purpuric, or necrotic.
The typical lesion has the appearance of a target. The target is considered pathognomonic.
Lesions may become bullous and later rupture, leaving denuded skin. The skin becomes susceptible to secondary infection.
Urticarial lesions typically are not pruritic.
Infection may be responsible for the scarring associated with morbidity.
Although lesions may occur anywhere, the palms, soles, dorsum of hands, and extensor surfaces are most commonly affected.
The rash may be confined to any one area of the body, most often the trunk.
Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis.
The following signs may be noted on examination:
Altered level of consciousness
Corneal ulcerations
Erosive vulvovaginitis or balanitis

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