Steven Johnson Syndrome

Stevens-Johnson syndrome (SJS) is an immune-complex–mediated hypersensitivity complex that typically involves the skin and the mucous membranes. While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, gastrointestinal, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis. Stevens-Johnson syndrome is a serious systemic disorder with the potential for severe morbidity and even death.
The syndrome was first described in 1922, when the American pediatricians Albert Mason Stevens and Frank Chambliss Johnson reported the cases of 2 boys aged 7 and 8 years with "an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis." Both cases had been misdiagnosed by primary care physicians as hemorrhagic measles.
Erythema multiforme (EM), originally described by von Hebra in 1866, was part of the differential diagnosis in both cases but was excluded because of the "character of skin lesions, the lack of subjective symptoms, the prolonged high fever, and the terminal heavy crusting." Despite the presence of leukopenia in both cases, Stevens and Johnson in their initial report suspected an infectious disease of unknown etiology as the cause.
Typically, Stevens-Johnson syndrome (SJS) 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 are occasionally noted as part of the prodrome.
Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions are typically 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 Stevens-Johnson syndrome or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.
Typical prodromal symptoms are as follows:

• Cough productive of a thick purulent sputum
• Headache
• Malaise
• Arthralgia

Patients may complain of a burning rash that begins symmetrically on the face and the upper part of the torso. This may be accompanied by ocular symptoms.
In addition to the skin, lesions in Stevens-Johnson syndrome may involve the following parts of the body:

• Oral mucosa
• Esophagus
• Pharynx
• Larynx
• Anus
• Trachea
• Vagina
• Urethra

Ocular symptoms include the following:

• Red eye
• Tearing
• Dry eye
• Pain
• Blepharospasm
• Itching
• Grittiness
• Heavy eyelid
• Foreign body sensation
• Decreased vision
• Burn sensation
• Photophobia
• Diplopia