1/06/2009

Stevens Johnson syndrome (SJS): Symptoms & Treatment

OverviewStevens-Johnson Syndrome is a very serious condition commonly caused by an allergic reaction to drugs. Most common cause of Stevens-Johnson Syndrome is an allergic reaction on antibiotics or anti-convulsant, and pain relievers, especially sulfas, non-steroidal anti-inflammatory drugs (NSAIDs...



Overview





Stevens-Johnson Syndrome is a very serious condition commonly caused by an allergic reaction to drugs. Most common cause of Stevens-Johnson Syndrome is an allergic reaction on antibiotics or anti-convulsant, and pain relievers, especially sulfas, non-steroidal anti-inflammatory drugs (NSAIDs). Stevens-Johnson syndrome is a serious, sometimes fatal inflammatory disease. Everyone who uses those drugs and cocaine can be affected. Any drug is a potential cause. Vaccines, such as those for tuberculosis and polio, could also be cause of Stevens-Johnson Syndrome. People who have Stevens-Johnson Syndrome suffer from inflammation of the skin and mucous membranes. Many organs can become significantly affected during the disease process. Stevens-Johnson Syndrome is a serious disorder and in some cases, it can be fatal. Stevens-Johnson Syndrome is the most severe form of erythema multiforme, sometimes is called erythema multiforme major. The diagnosis of Stevens-Johnson Syndrome is usually based on the physical exam, skin and mucosal lesions are sign of disease. In some cases skin biopsy may be performed. Stevens-Johnson syndrome affects people any age. Most important thing for treatment is the recognition and proper diagnosis of the disorder.







Symptoms





Stevens-Johnson Syndrome is marked by the rapid attack of fever, skin lesion, and open sores on the mucous membranes of the eyes, mouth, nasal passage, lips, and genitals.





The disease begins with a nonspecific respiratory tract infection. Other symptoms are fever, sore throat, chills, headache, and malaise. Stevens-Johnson syndrome affects the mucous membranes of the oral cavity, eyes, nostril, and both the anal and genital regions. In some cases vomiting and diarrhea could occur. Mucocutaneous nonpruritic lesions are common in Stevens Johnson syndrome. Clusters last about 2 %26ndash; 4 weeks. In some cases people whit SJS may not be able to eat or drink, mucous membranes of the mouth can be affected.





Stevens-Johnson Syndrome is severe expression of the erythema multiforme. When someone has Stevens-Johnson Syndrome, erythema multiforme can be present and become noticeable like skin lesion. Erythema multiforme is an allergic condition marked by a rash on the skin and mucous membranes. Erythema multiforme can occur with many infections, collagen diseases, drug reactions, allergies, and pregnancy. The skin lesions may look like a target lesion or bubble-like.



Toxic Epidermal Necrolysis which involves multiple large blisters that coalesce also is present. Toxic Epidermal Necrolysis is followed by a sloughing of most of the skin and mucous membranes.








You should know that Stevens-Johnson Syndrome is a very serious condition. This disease involves many parts of the body and extensive lesion formation. Those lesions first affect the mucous membranes, and then affect the lungs, mouth, stomach, intestines eyes, and every major organ.



Most common signs include: fever, epistaxis, conjunctivitis, vulvovaginitis or balanitis, eizures and corneal ulcerations, erosive and coma. Pneumonia, pain in the joints, and prostration are common. A painful conjunctivitis may occur in the eye, and can lead to corneal scarring and loss of vision. Ophthalmologist should be involved for patients with conjunctivitis so that precautions can be taken to avoid permanent eye damage. In advanced stage of disease renal failure and pneumonia may occur. That can be fatal.





Treatment





The syndrome may be an allergic reaction to certain drugs, or it may follow pregnancy, herpes virus I, or other infection. It is rarely seen linked with cancer or with radiation therapy. Early diagnosis with the early recognition and withdrawal of all potential causative drugs is most important for a successful treatment. A dermatologist is the needed to establish the diagnosis. Some severe cases of Stevens Johnson Syndrome may require the involvement of a burn specialist or plastic surgery specialist. Depending on which organ is affected, consultations with a gastroenterologist, pulmologist, ophthalmologist, and nephrologist and other specialist may be helpful.








There is there is no effective treatment to stop the spread of Stevens Johnson Syndrome throughout the body. Primary treatment is supportive and symptomatic. Treatment includes management of Stevens Johnson Syndrome symptoms. Treatment includes bed rest, antibiotics for pneumonia, painkilling drugs, mouthwashes, and sedatives. Usually Stevens Johnson Syndrome has been caused by an allergic reaction to a drug or an infection. Also is important to find out which drug caused this disorder. Stop usage of the offending drug or treating the infection can have a positive influence on the progress of Stevens Johnson Syndrome. Drugs such as Bextra and antibiotics should be discontinued immediately.





Underlying infection should be recognized. Underlying diseases or infections which is causing Stevens Johnson Syndrome must be treated. Antibiotics are appropriate if superinfection is cause of Stevens Johnson Syndrome. Antibiotics also are used if bacterial disease, such as mycoplasma, is suspected to be the cause. When respiratory tract is affected (lung) treatment include: aerosols, bronchial aspiration and physical therapy. Some drugs are needed like heparin which is recommended for the duration of hospitalization. In some cases antacids reduce the incidence of gastric bleeding. Emotional and psychiatric support can be helpful.





Treatment whit corticosteroids are controversial. Some scientists think that corticosteroids provoke increased risk of infection, prolonged wound healing, masking of early signs of sepsis, severe gastrointestinal bleeding and increased mortality.








If someone has Stevens Johnson Syndrome, during disease usually suffers from massive fluid loss. So, treatment includes fluid replacement and electrolyte correction. Disease spreading can cause compromise of the cardiovascular and respiratory systems. So, one of the most important part of treatment is to ensure blood flow and blood pressure and airway stability. Pain management is also important part of treatment, because this disease is a very painful disorder. Intensive supportive care is important in severe cases. The patient must be transferred to an intensive care unit or a burn center. Skin lesions should be treated as burns. Denudated skin must be covered with compresses of saline or Burow solution. Careful daily control is necessary to monitor for secondary superinfections. Some specific treatment options can be applied to reduce the symptoms like mouthwash treatment for lesions in the mouth, and topical anesthesia to reduce pain. Saline compresses may be applied to the eyelids, lips, and nose.








You should know that there is no cure or treatment to stop the spreading of Stevens Johnson Syndrome. Some new researches have suggested that hemodialysis and immunoglobin, cyclophosphamide, and plasmapheresis, are effective treatments. You should know that those are not standard methods of care.





Patients must avoid any future exposure to drugs which caused Stevens-Johnson Syndrome. Recurrences are possible.