Quick Facts: Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)

Pulmonary-renal syndrome affects both the lungs and kidneys. This disorder causes bleeding into the lungs (diffuse alveolar hemorrhage) along with damage to the microscopic blood vessels in the kidney (glomerulonephritis). Which of the following is almost always the cause of this syndrome?

An autoimmune disorder (a condition in which the body mistakenly attacks its own tissues)
A genetic abnormality
An injury to the lung and/or kidney
Severe, chronic hypertension (high blood pressure)

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Stevens-Johnson Syndrome (SJS)

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are forms of the same life-threatening skin disease. SJS involves less of your body, and TEN involves more. They both cause a rash, skin peeling, and sores.

SJS and TEN are usually caused by a reaction to a medicine
Symptoms for both diseases include peeling skin, fever, body aches, a flat red rash, and blisters and sores on your mouth, eyes, and vagina
Treatment is in an intensive care unit in the hospital and includes fluids, medicines, and stopping any medicine that may have caused the disease

SJS and TEN are most often caused by:

A reaction to a medicine, most often sulfa and other antibiotics or drugs for seizures (anticonvulsants)

Less often, cases are caused by a bacterial infection or vaccination.

SJS and TEN are more common in people who have:

A bone marrow transplant
Lupus
HIV infection

Symptoms of SJS and TEN usually start 1 to 3 weeks after starting a medicine. The first symptoms include:

Fever
Headache
Cough
Inflamed, red eyes
Body aches

Later symptoms include:

Flat, red rash on your face, neck, and trunk, which spreads to the rest of your body
Blisters that form and peel easily
Blistering in your mouth, eyes, and vagina

In SJS, skin peels off small areas of your body. In TEN, the areas are larger and more of your skin is affected. The more skin that peels off, the more dangerous it is.

Doctors can tell you have SJS or TEN by asking you about your symptoms and looking at your skin. They may also do a skin biopsy (taking a little piece of tissue to look at under a microscope).

Doctors care for you in the hospital in an ICU (intensive care unit). Doctors will stop any medicines that might have caused your SJS or TEN.

Treatments include:

Fluids by vein
Plasma exchange (a process that lets doctors filter some substances, such as a medicine that's causing your reaction, out of your blood)
Immune globulin
Sometimes, medicine

Generic Name	Select Brand Names
Immune globulin	Gammagard S/D

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