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Stevens Johnson syndrome

Stevens–Johnson syndrome
Synonyms Erythema multiforme major
Stevens-johnson-syndrome.jpg
Man with Stevens–Johnson syndrome
Specialty Dermatology
Symptoms Fever, skin blisters, skin peeling, painful skin, red eyes
Complications Dehydration, sepsis, pneumonia, multiple organ failure.
Usual onset Age < 30
Causes Certain medications, certain infections, unknown
Risk factors HIV/AIDS, systemic lupus erythematosus, genetics
Diagnostic method <10% of the skin involved, skin biopsy
Similar conditions Chickenpox, staphylococcal epidermolysis, staphylococcal scalded skin syndrome, autoimmune bullous disease
Treatment Hospitalization, stopping the cause
Medication Pain medication, antihistamines, antibiotics, corticosteroids, intravenous immunoglobulins
Prognosis Mortality 10%
Frequency 1–2 per million per year (together with TEN)
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Classification
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Stevens–Johnson syndrome (SJS) is a type of severe skin reaction. Together with toxic epidermal necrolysis (TEN) it forms a spectrum of disease, with SJS being less severe. Early symptoms include fever and flu-like symptoms. A few days later the skin begins to blister and peel forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia, and multiple organ failure.

The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics, and nevirapine. Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus or the cause may remain unknown. Risk factors include HIV/AIDS and systemic lupus erythematosus. The diagnosis is based on involvement of less than 10% of the skin. It is known as TEN when more than 30% of the skin is involved and an intermediate form with 10 to 30% involvement.Erythema multiforme (EM) is generally considered a separate conditions.

Treatment typically takes place in hospital such as in a burn unit or intensive care unit. Efforts may include stopping the cause, pain medication, antihistamines, antibiotics, intravenous immunoglobulins, or corticosteroids. Together with TEN it affects 1 to 2 people per million per year. It is twice as common in males as females. Typical onset is under the age of 30. Skin usually regrows over two to three weeks; however, complete recovery can take months.


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