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Stevens Johnson Syndrome (SJS)

Stevens-Johnson syndrome (SJS) & toxic epidermal necrolysis (TEN) are mucocutaneous drug-induced or idiopathic reaction patterns characterized by skin tenderness & erythematic of skin & mucosa, followed by extensive cutaneous & mucosal exfoliulltion & are potentially life-threatening due to multi system involvement. SJS has been classified as a severe expression of erythematic multiforme (EM), & is sometimes referred to as erythematic multiforme major .

The Stevens Johnson Syndrome Foundation said they have seen a rise in the disease involving children & ibuprofen use during 2004, but the disease is still not well known.

reason of Stevens Johnson Syndrome

Unknown but consistent with immullogic mechanisms, i.e., cell-mediated catatonic reaction against epidermal cells. Epidermis infiltrated by activated lymphocytes, mainly CD8 cells, & macrophages. Cytokines produced by activated mononuclear cells & keratinocytes probably contribute to local cell death, fever & malaise. SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng .

Signs & symptoms of Stevens Johnson Syndrome

Prodrome Fever, influenza-like symptoms 1 to 3 days prior to mucocutaneous lesions, Mild to moderate skin tenderness, conjunctival burning or itching.

Skin Symptoms Skin pain, burning sensation tenderness, parenthesis.

Mucous Membrane Symptoms Mouth lesions are painful, tender.

General Symptoms Impaired alimentation photophobia, painful micturition, anxiety.

As Stevens Johnson Syndrome progresses the skin literally sloughs off like a full thickness burn called Toxic Epidermal Necrolysis. In about 10 to 30 percent of cases, Stevens Johnson Syndrome results in blinding the patient or killing them. The blinding is the result of the entire surface of the eye scarring over, causing the vision to be lost.

Diagnosis

The diagnosis is primarily based on the appearance of the skin lesion & its typical symmetrical distribution, especially if there is a history of risk factors or associated diseases.

Treatment of Stevens Johnson Syndrome

People with Stevens-Johnson syndrome or toxic epidermal necro lysis are hospitalized. Any drugs suspected of causing the disorder are immediately discontinued. When possible, these people are treated in a burn unit & given scrupulous care to avoid infection. If the person survives, the skin grows back on its own, & unlike burns, skin grafts are not needed. Fluids & salts, which are lost through the damaged skin, are replaced intravenously.

Use of corticosteroids to treat the disorder is controversial: Some doctors believe that giving large doses within the first few days is beneficial; others believe that corticosteroids should not be used. These drugs suppress the immune system, which increases the potential for serious infection. If infection develops, doctors give antibiotics immediately.

prohibition of Stevens Johnson Syndrome

• Early diagnosis & withdrawal of suspected drugs are very important.
• Patients are best cared for in a burn or intensive care unit.
• The patient must be aware of the likely offending drug & that other drugs of the same class can cross react. These drugs must never be readministered. Patient should wear a medical alert bracelet.