About johnson-stevens disease

What is johnson-stevens disease?

Until recently the relationship of Stevens-Johnson syndrome to other severe blistering disorders was a matter of some debate. Now a consensus seems to be evolving that describes SJS as a rare disorder involving lesions of the mucous membranes along with small blisters on the reddish or purplish, flat, thickened patches of skin. As a result, SJS is now distinguished as a separate disorder from erythema multiforme major (EMM). SJS is now considered to be a less severe variant of toxic epidermal necrolysis (TEN).

SJS and TEN appear to be characterized by identical clinical signs and symptoms, identical treatment approach and identical prognosis. Patients diagnosed with TEN can present with symptoms ranging from 10% skin involvement and severe threat to the patient's sight to a presentation involving 90% of the skin but only a modest threat to the patient's sight.

SJS (and TEN) is an inflammatory disorder of the skin triggered by an allergic reaction to certain drugs including antibiotics, such as some sulfonamides, tetracycline, amoxicillin, and ampicillin. In some cases, nonsteroidal anti-inflammatory medications and anticonvulsants, such as Tegretol and phenobarbital have also been implicated. Over-the-counter medications may act as triggers as well. In some cases, it is also possible that the disorder may be traced to a reaction to an infection.

One report suggests that the term SJS be limited to cases in which less than 10% of the total body surface area is involved. The authors suggest that the term TEN be limited to cases in which 30% or more of the total body surface area is involved. The term SJS/TEN Overlap is used to describe patients in whom between 10-30% of the total body surface area involved.

What are the symptoms for johnson-stevens disease?

Red rash or red blotches on your skin symptom was found in the johnson-stevens disease condition

As the condition develops, other signs and symptoms include:

  • Unexplained widespread skin pain
  • A red or purple Rash that spreads
  • Blisters on your skin and the mucous membranes of the mouth, nose, eyes and genitals
  • Shedding of skin within days after Blisters form

What are the causes for johnson-stevens disease?

Stevens-Johnson syndrome is a rare and unpredictable illness. Your health care provider may not be able to identify its exact cause, but usually the condition is triggered by medication, an infection or both. You may react to medication while you're using it or up to two weeks after you've stopped using it.

Drugs that can cause Stevens-Johnson syndrome include:

  • Anti-gout medications, such as allopurinol
  • Medications to treat seizures and mental illness (anticonvulsants and antipsychotics)
  • Antibacterial sulfonamides (including sulfasalazine)
  • Nevirapine (Viramune, Viramune XR)
  • Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve)

Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV.

What are the treatments for johnson-stevens disease?

Treating Stevens-Johnson syndrome requires hospitalization, possibly in an intensive care unit or a burn unit.

Stopping nonessential medications

The first and most important step in treating Stevens-Johnson syndrome is to stop taking any medications that may be causing it. If you're taking more than one drug, it may be hard to tell which drug is causing the problem. So your health care provider may have you stop taking all nonessential medications.

Supportive care

Supportive care you're likely to receive while in the hospital includes:

  • Fluid replacement and nutrition. Because skin loss can result in significant loss of fluid from your body, replacing fluids is an important part of treatment. You may receive fluids and nutrients through a tube inserted in the nose and guided to the stomach (nasogastric tube).
  • Wound care. Cool, wet compresses might help soothe blisters while they heal. Your health care team may gently remove dead skin and put petroleum jelly (Vaseline) or a medicated dressing over affected areas.
  • Eye care. You may also need care from an eye specialist (ophthalmologist).


Medications used in the treatment of Stevens-Johnson syndrome include:

  • Pain medication to reduce discomfort.
  • Medication to reduce inflammation of the eyes and mucous membranes (topical steroids).
  • Antibiotics to control infection, when needed.
  • Other oral or injected (systemic) medications, such as corticosteroids and intravenous immune globulin. Studies show that the drugs cyclosporine (Neoral, Sandimmune) and etanercept (Enbrel) are helpful in treating this disease.

If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, new skin may begin to grow within several days. In severe cases, full recovery may take several months.

What are the risk factors for johnson-stevens disease?

Factors that increase your risk of developing Stevens-Johnson syndrome include:

  • An HIV infection. Among people with HIV, the incidence of Stevens-Johnson syndrome is about 100 times greater than among the general population.
  • A weakened immune system. The immune system can be affected by an organ transplant, HIV/AIDS and autoimmune diseases.
  • Cancer. People with cancer, particularly blood cancer, are at increased risk of Stevens-Johnson syndrome.
  • A history of Stevens-Johnson syndrome. If you've had a medication-related form of this condition, you are at risk of a recurrence if you use that drug again.
  • A family history of Stevens-Johnson syndrome. If an immediate blood relative has had Stevens-Johnson syndrome, you may be at increased risk of getting it too.
  • Genetic factors. Having certain genetic variations puts you at increased risk of Stevens-Johnson syndrome, especially if you're also taking drugs for seizures, gout or mental illness.

Is there a cure/medications for johnson-stevens disease?

Johnson –stevens disease is a very rare syndrome with potentially fatal skin reactions in which loss of skin occurs and mucosal membranes accompanied by systemic symptoms is shown in some case. For these conditions, in 80% of cases, the reactions from medications are responsible.

This condition requires intensive care or burn care unit in hospitalization,

  • If a person is taking unessential medications, then it needs to be stopped by tracking the cause from which medicine it triggers.
  • Fluid replacement is required because of skin loss; the body may lose some extra fluids
  • Dressing over the affected area and using petroleum jelly is one of the ways to cure this condition.
  • Temperature control
  • In some cases, oxygen or ventilators are required

Systemic corticosteroids are often used in a high dosage for the first two or three days. Granulocyte colony-stimulating factor (G-CSF) is also beneficial for a patient with neutropenia.
Other drugs that can be useful are as follows:

  • Systemic corticosteroids
  • Ciclosporin
  • TNF-alpha inhibitors
  • N-acetylcysteine
  • Intravenous immunoglobulins

These drugs are given under the observations of doctors because of their side effects and their roles are controversial.

Skin pain,Fever,Body aches,Red rash or red blotches on your skin,Cough,Blisters and sores on the skin and on mucus membranes of the mouth, throat, eyes, genitals and anus,Peeling skin,Drooling (because closing the mouth is painful)
Very serious skin peeling conditions,Painful rash that spreads,Blisters
Stopping the problem medication,Replacing electrolytes,Applying skin dressings,Providing pain medications and antibiotics,IV immunoglobulin,Cyclosporine,IV steroids,Amniotic membrane grafts (for your eyes)

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