Stevens–Johnson syndrome
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Stevens–Johnson syndrome (SJS) is a type of severe skin reaction.[1] Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN) overlap, they are considered febrile mucocutaneous drug reactions and probably part of the same spectrum of disease, with SJS being less severe.[1][5][3] Erythema multiforme (EM) is generally considered a separate condition.[6] Early symptoms of SJS include fever and flu-like symptoms.[1] A few days later, the skin begins to blister and peel, forming painful raw areas.[1] Mucous membranes, such as the mouth, are also typically involved.[1] Complications include dehydration, sepsis, pneumonia and multiple organ failure.[1]
Stevens–Johnson syndrome | |
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Man with characteristic skin lesions of Stevens–Johnson syndrome | |
Specialty | Dermatology |
Symptoms | Fever, skin blisters, skin peeling, painful skin, red eyes[1] |
Complications | Dehydration, sepsis, pneumonia, multiple organ failure.[1] |
Usual onset | Age < 30[2] |
Causes | Certain medications, certain infections, unknown[2][1] |
Risk factors | HIV/AIDS, systemic lupus erythematosus, genetics[1] |
Diagnostic method | <10% of the skin involved, skin biopsy[2] |
Differential diagnosis | Chickenpox, staphylococcal epidermolysis, staphylococcal scalded skin syndrome, autoimmune bullous disease, Smallpox[3] |
Treatment | Hospitalization, stopping the cause[2] |
Medication | Pain medication, antihistamines, antibiotics, corticosteroids, intravenous immunoglobulins[2] |
Prognosis | Mortality ~7.5%[1][4] |
Frequency | 1–2 per million per year (together with TEN)[1] |
The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine.[1] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus, or the cause may remain unknown.[2][1] Risk factors include HIV/AIDS and systemic lupus erythematosus.[1]
The diagnosis of Stevens–Johnson syndrome is based on involvement of less than 10% of the skin.[2] It is known as TEN when more than 30% of the skin is involved and considered an intermediate form when 10–30% is involved.[3] SJS/TEN reactions are believed to follow a type IV hypersensitivity mechanism.[7] It is also included with drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), acute generalized exanthematous pustulosis (AGEP) and toxic epidermal necrolysis in a group of conditions known severe cutaneous adverse reactions (SCARs).[8]
Treatment typically takes place in hospital such as in a burn unit or intensive care unit.[2] Efforts may include stopping the cause, pain medication, antihistamines, antibiotics, intravenous immunoglobulins or corticosteroids.[2] Together with TEN, SJS affects 1 to 2 people per million per year.[1] Typical onset is under the age of 30.[2] Skin usually regrows over two to three weeks; however, complete recovery can take months.[2] Overall, the risk of death with SJS is 5 to 10%.[1][4]