What is sjs disease

The HLA complex helps the immune system distinguish the body's own proteins from proteins made by foreign invaders such as viruses and bacteria.

The HLA-B gene has many different normal variations, allowing each person's immune system to react to a wide range of foreign proteins. In a process that is not well understood, the drug causes immune cells called cytotoxic T cells and natural killer NK cells to release a substance called granulysin that destroys cells in the skin and mucous membranes.

In particular, these skin reactions have occurred in people with an unusual form of pneumonia caused by infection with Mycoplasma pneumoniae and in people with viral infections, including cytomegalovirus. Researchers suspect that a combination of infections and drugs could contribute to the disease in some individuals.

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Contact a health care provider if you have questions about your health. From Genetics Home Reference. Research Studies from ClinicalTrials. Epub Mar 5. HLA associations and clinical implications in T-cell mediated drug hypersensitivity reactions: an updated review. J Immunol Res. Epub May 8. The incidence of the condition among people with HIV is about times greater than among people in the general population.

Having cancer. Having a weakened immune system. Treatment Treatment. When the diagnosis is confirmed, the extent of the disease should be determined quickly so that the most appropriate place for treatment can be decided. Several studies show that the chance of recovery is better for those moved promptly to a burn care unit or intensive care unit. Treatment should ideally be managed by a team of doctors experienced in treating the condition.

Eye inflammation can worsen quickly within a few days, so daily eye evaluations by an ophthalmologist and aggressive treatment are needed. Treatment depends on the extent of eye involvement and may involve any of several strategies either alone or in combination, including: [4] [6] Saline rinses to clean the eyes and eyelids. Lubrication multiple times per day with preservative-free eye drops or ointments including for those with no apparent eye involvement. Eye medicines with topical corticosteroids and broad-spectrum antibiotics.

Amniotic membrane transplantation AMT to try to prevent vision loss and complications involving the mucous membranes. Some people need multiple procedures. The amniotic membrane is the innermost layer of the placenta, and can been used as a graft or dressing to aid in repairing the surface of the eyes and promote healing. Beyond supportive care, various therapies have been tried by doctors, including systemic corticosteroids, intravenous immune globulin IVIG , cyclosporine , plasmapheresis , and anti-tumor necrosis factor TNF monoclonal antibodies.

However, with the exception of thalidomide which was found to be harmful , none have been adequately studied in randomized trials. There is, however, increasing evidence that cyclosporine may slow the progression of the condition.

Prognosis Prognosis. The long-term outlook and chance of recovery varies from person to person. Regrowth of the affected skin typically occurs in two to three weeks, but recovery can take weeks to months, depending on the severity of symptoms.

Feelings of overwhelming tiredness may persist for months. Depression may also develop. For some, complications may develop within weeks to months of an acute episode, and there may be long-term complications involving the skin and affected mucous membranes, which can severely impact quality of life. However recurrent episodes due to different medications or infections have also been reported. The most common causes of death include sepsis , acute respiratory distress syndrome , and multiple organ failure.

Find a Specialist Find a Specialist. Healthcare Resources To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. You can also learn more about genetic consultations from MedlinePlus Genetics. Research Research. Clinical Research Resources ClinicalTrials. Click on the link to go to ClinicalTrials. Please note: Studies listed on the ClinicalTrials.

We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study. Learn More Learn More. Where to Start MayoClinic. This website is maintained by the National Library of Medicine.

The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. In-Depth Information Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free. Stevens-Johnson syndrome Toxic Epidermal Necrolysis TEN The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.

This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. Orphanet is a European reference portal for information on rare diseases and orphan drugs.

Access to this database is free of charge. Click on the link to view a sample search on this topic. Have a question? References References. High WA. Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis. Stevens-Johnson syndrome. Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae.

Kang MH. Hanyang Med Rev. Foster CS. SJS affects around people in every million, so it certainly passes the rarity test.

At Isabel, which started as a charity after the misdiagnosis of a rare disease, we try to shed a light on rare diseases, particularly those that require timely diagnosis, and particularly those that often affect children. Stevens-Johnson syndrome, named after the two doctors who first described the disease in , is an autoimmune disorder that manifests as a painful rash on the skin, mucous membranes, and genitals.

The disease is often caused by an allergic reaction to certain medications. It can be an over-reaction of the immune system to viral or bacterial infections, and this is more common in children and young adults. Any infection, from the simple flu virus to viral meningitis, can cause an adverse immune response and SJS.

Likewise, any drug could at all could cause an allergic reaction, although some are more common than others. The most common drugs to cause SJS are:. SJS is an acute emergency disease, meaning it develops over a matter of hours or days and immediate medical attention should be sought.

If left untreated, the skin rash intensifies and blisters, causing painful sores when they burst. The mucous membranes, which run through the digestive tract from the mouth to the anus, also become blistered and ulcerated, making digestion and fluid intake extremely difficult. Eventually, the skin dies and begins to peel off, at which point the body is at a high risk of sepsis and other life threatening complications.

SJS is serious enough as it is, but when the reaction is more severe it is then called toxic epidermal necrolysis TEN. They are the same disease, and are differentiated by the level of damage to the skin. The causes and treatment are much the same, which we will go into later on in the blogpost. The beginning stages of SJS are somewhat hard to spot as they resemble those of the flu, with a temperature, sore throat and fatigue.