Stevens-Johnson Syndrome is a serious medical condition commonly caused by an allergic reaction to drugs. The drugs associated with Stevens-Johnson Syndrome (SJS) are antibiotics (penicillin and sulfa's), anti-convulsants, and pain relievers. Prescription pain relievers, such as Bextra (valdecoxib), Celebrex (celecoxib) and Daypro, as well as over-the-counter pain relievers, such as Motrin, Ibuprofin, Children's Motrin and Advil, have reportedly been associated with Stevens-Johnson Syndrome. Cocaine has also recently been added to the list of drugs capable of eliciting Stevens-Johnson Syndrome. SJS can also occur in response to infections or illnesses. Twenty-five to 50% of all cases derive from unknown origin.
What is Stevens-Johnson Syndrome?
Stevens Johnson Syndrome patients suffer from inflammation of the skin and mucous membranes. Since mucous membranes are present in many organs throughout the body, such as the eyes, digestive system, lungs and respiratory system, many organs can become significantly affected during the disease process. SJS technically is an immune-complex-mediated hypersensitivity (allergic) condition. It is a severe expression of the condition known as erythema multiforme and is a lesser form of the condition known as toxic epidermal necrolysis. SJS is a serious disorder with potential for severe morbidity and in some cases, it can be fatal.
Erythema multiforme can be present and become noticeable in the form of a classic skin lesion, without displaying whole-body symptoms. This condition is normally associated with herpes simplex or mycoplasma infections. It occurs most often in children and young adults.
Stevens-Johnson Syndrome is a much more severe condition than erythema multiforme. SJS typically involves multiple areas of the body and extensive lesion formation. The lesions can extend to the mucous membranes, thus affecting the lungs, eyes, mouth, stomach, intestines and virtually every major organ.
Toxic Epidermal Necrolysis is the most severe condition associated with immune complex hypersensitivity. This condition involves multiple large blisters that coalesce, followed by a sloughing of most of the skin and mucous membranes.
Stevens-Johnson Syndrome Clinical Course
The disease process for Stevens-Johnson Syndrome typically begins with a nonspecific upper respiratory tract infection. Early Stevens Johnson Syndrome symptoms occur in the first 1- to 14-day period during which fever, sore throat, chills, headaches and malaise may be present. Vomiting and diarrhea are sometimes noted in the early symptoms. Skin and mucous membrane lesions can develop abruptly with clusters of outbreaks lasting from 2- to 4-weeks. Continued fever or localized worsening of the lesions suggests a superimposed infection; however, continued fever has been shown to occur in up to 85% of the cases. In some cases, the lesions of the mouth and mucous membranes are so severe, patients may not be able to eat or drink.
In up to 15% of the cases, patients with severe SJS die. In the severe cases, the lesions lead to significant scarring of the involved organs and loss of function of the organ systems. Restriction of the esophagus and of the respiratory tract can occur due to lesions and scaring. Ocular effects can include corneal ulcers and uveitis. Blindness can occur secondary to eye conditions caused by SJS. Scarring of the genital areas can occur, but renal complications are rare. SJS treatment is focused on the management of disease symptoms because there is no treatment (as of yet) able to stop the spread of the disease.
