Stevens-Johnson syndrome (patient information)
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Stevens-Johnson syndrome |
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Overview
Stevens-Johnson syndrome is a life-threatening condition affecting the skin. The syndrome is thought to be a hypersensitivity reaction affecting the skin and the mucous membranes
What are the symptoms of Stevens-Johnson syndrome?
Stevens-Johnson syndrome (SJS) usually begins with a fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Skin eruption occurs 1-3 days after the flu-like symptoms. Skin lesions start as red or reddish blue lesions on trunk and extremities and later increase in size and combine. Blisters, Erosions and Vesicles are alsoseen
Ulcers and other lesions also begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.
What causes Stevens-Johnson syndrome?
Stevens-Johnson Syndrome is caused by drug reactions in the majority of the cases although it is also seen in certain viral and bacterial infections, and certain malignancies. Most commonly implicated drugs include sulfa drugs, Allopurinol, Anti-Epileptics (Phenobarbital, Carbamazepine, Lamotrigine), Antibiotics (for example Penicillin), and NSAIDs. Infections that are associated with SJS may include HIV, Herpes Simplex Virus, Mumps, Mycoplasma, Mycobacteria, Hepatitis and Streptococcal Infection.
Who is at highest risk?
Common risk factors in the development of SJS include Low immunity, HIV infection, Active cancers (particularly Blood cancers), Genetic Predisposition ( HLA-B 1502 and HLA-B 5801 variants in particular) on exposure to anti-epileptic medications or Allupurinol. Other risk factors may include past or family history of SJS/TEN, Radiation therapy and Rapid use of medications.
Diagnosis
SJS is mainly diagnosed through the clinical skin findings. However, a skin biopsy is helpful in making the definitive diagnosis. Other tests are done to either rule out the superimposed bacterial infection, measure disease severity or detect the causative agent or organism. Tests include CBC, BMP, Blood cultures, ESR, CRP, IL-6, ELISA for virus detection among others.
When to seek urgent medical care?
Urgent medical Care should be sought in case of skin eruptions or rash along with flu-like symptoms following a drug use or otherwise.
Treatment options
Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment. Treatment with steroids is controversial since it might aggravate the condition or increase risk of secondary infections.
Other agents have been used, including cyclophosphamide and cyclosporine, among with Cyclosporin has exhibited some therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms.
Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics.
An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids leading to corneal vascularization and impaired vision, as well as a host of other ocular problems.
Also, an extensive physical therapy program ensues after the patient is discharged from the hospital.
Where to find medical care for Stevens-Johnson syndrome?
Directions to Hospitals Treating Condition
Prevention
- For people of Chinese, Indian or Sutheast Asian descent, consultation with your doctor is recommended before taking carbamazepine. This drug is used treat epilepsy, bipolar disorder and some other conditions. But people with a gene called HLA-B*1502 have an increased risk of Stevens-Johnson syndrome if they take this drug.
- If you've had Stevens-Johnson syndrome before and your doctor told you it was caused as a result of medication use, avoid using that drug again and others like it. The reason is to prevent the recurrence of disease, which is usually more severe than the first episode and can be fatal.
- Family members of people developing SJS after a drug use should also try to to avoid that drug because some forms of this condition have a genetic risk factor.
What to expect (Outlook/Prognosis)?
Prognosis of Stevens-Johnson syndrome is generally good. Most lesions heal rapidly within 1-3 weeks unless secondary bacterial infection of lesions occurs. Recovery can take longer, although it usually depends on the severity of symptoms and body surface area involved. Mortality rate is 1-5 percent in SJS and in TEN is 25-35 percent
Possible complications
Common complications of SJS/TEN include:
- Bacterial Infections leading to sepsis
- Eye Complications - Appropraite treatment of acute ocular complications can prevent long term complications
- Pulmonary - Pnemonia, Interstitial pneumonitis, ARDS (25 percent of patients have been reported to require mechanical ventilation)
- Skin - Scarring