Stevens-Johnson syndrome

background

Stevens-Johnson syndrome (SJS) is a type of allergic reaction that usually occurs in response to medications. It is a severe type of erythema multiforme, which is a skin disease characterized by skin eruptions and blisters. SJS is a less severe form of another disease called toxic epidermal necrolysis (TENS).
Pediatricians, A.M. Stevens and S.C. Johnson, discovered the disease in 1922 when they treated two young male patients with skin lesions, fever, inflamed mucosa and conjunctivitis (pinkeye).
SJS is a systemic disorder that is potentially life threatening, especially if left untreated. About 3-15% of patients with severe SJS die. In severe cases, the lesions can cause significant scarring of the involved organs, which often leads to loss of function of the organ systems. For instance, restriction of the esophagus and of the respiratory tract can occur due to lesions and scaring.
SJS typically involves many areas of the body and extensive lesion formation. The lesions can extend to the mucous membranes and affect any organ, including the lungs, eyes, mouth, stomach and intestines. The disease can cause severe damage to the mouth, nose, eye, vagina, urethra, gastrointestinal tract and/or lower respiratory tract.
It is estimated that about one million Americans are diagnosed with SJS each year.
SJS is an immune-complex-mediated hypersensitivity disorder that may be caused by medications, viral infections and malignancies (cancers). In up to half of cases, no specific etiology has been identified.
The ratio of male to female occurrence is two to one. Since the causes of SJS are not well understood, it is unclear why it is more common among males.
Currently, there is no treatment for the specific disease. Instead, treatment focuses on alleviating the symptoms. In general, the condition lasts about two to six weeks.

Related Terms

Adenoviral conjunctivitis, allergic, allergic reaction, allergic response, antibodies, blister, bullous systemic lupus erythematosus, drug allergy, drug-induced, drug reaction, erythroderma ichthyosiform congenital, erythema multiforme, erythema multiforme major, idiopathic, immune-complex-mediated, immune defense system, immune system, immunoglobulin, immunosuppressant, intravenous immunoglobulin therapy, IVIG, lesion, mucocutaneous, pinkeye, rash, skin disease, skin disease, skin disorder, skin lesion, Stevens Johnson syndrome, systemic disorder.

adverse effect vs. allergic reaction

Not all adverse reactions to drugs are allergies. In fact, less than 10 percent of adverse drug reactions are allergic because most drug side effects are not related to the immune system. Instead, the drug directly affects various organs throughout the body.
For instance, a nasal decongestant constricts the capillaries in the nose in order to reduce nasal swelling and open the airway passages. However, a side effect is that it also constricts the capillaries in other regions of the body, which may lead to an increased heart rate, headache, dizziness or increased blood pressure. Since a decongestant is not specific to a single part of the body or one single action, many people could experience side effects. Therefore, it is a non-allergic reaction because the body's immune system is not involved.
Other causes of adverse reactions include interactions between two or more drugs, overdose or the body's inability to break the drug down completely as a result of liver or kidney damage.
Allergic reactions can be classified into four immunopathologic categories using various classification systems. These classifications are based on the immune system's response to the allergen, not on the severity of the reaction.
SJS is technically an immune-complex-mediated hypersensitivity (allergic) condition. Therefore, SJS is a type III allergic reaction. This category involves the formation of an antigen-antibody immune complex, which deposits on blood vessel walls and activates cell components known as complements. This causes a serum-sickness like syndrome, involving fever, swelling, skin rash and enlargement of the lymph nodes in about three to eight hours. It may be caused by a variety of allergens, including penicillins, sulfonamides, intravenous (IV) contrast media and hydantoins.

support for patients

The Steven Johnson Syndrome Foundation is an online resource for SJS patients and their families. The organization aims to provide support services, information about SJS treatments and therapies to both healthcare professionals and the public. The goal of the organization is to promote awareness about the disease so that a quick diagnosis can be made and quality treatment can begin.
More information can be found at the Steven Johnson Syndrome Foundation website, www.sjsupport.org.