Stevens–Johnson syndrome

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Stevens–Johnson syndrome
Synonyms	SJS
Pronounce	N/A
Specialty	N/A
Symptoms	Fever, sore throat, fatigue, skin rash, blisters, mucous membrane involvement
Complications	Dehydration, sepsis, organ failure, blindness
Onset	Sudden
Duration	Weeks to months
Types	N/A
Causes	Infection, medication
Risks	HIV/AIDS, systemic lupus erythematosus, cancer
Diagnosis	Clinical diagnosis, skin biopsy
Differential diagnosis	Toxic epidermal necrolysis, erythema multiforme, pemphigus vulgaris
Prevention	N/A
Treatment	Hospitalization, supportive care, corticosteroids, intravenous immunoglobulins
Medication	N/A
Prognosis	Variable, can be life-threatening
Frequency	Rare
Deaths	5-10% mortality rate

Stevens–Johnson syndrome (SJS) is a rare and severe skin reaction often triggered by medications or infections. It is part of a spectrum of disease that includes toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN), with SJS being the less severe form. Erythema multiforme (EM) is typically considered a separate condition. SJS is characterized by the onset of flu-like symptoms, followed by the development of blistering and peeling skin and mucous membranes, which can lead to serious complications.

Etiology

The exact cause of SJS is not fully understood, but it is thought to be an immune complex hypersensitivity reaction. This could be triggered by certain medications, infections (such as herpes simplex, HIV, and Hepatitis, among others), or possibly malignancies. It is important to note that not everyone exposed to these triggers will develop SJS, suggesting a genetic predisposition.

Symptoms

Early symptoms of SJS can mimic those of a common flu, including fever, sore throat, fatigue, and body aches. A few days later, the individual may develop painful, red or purplish skin that blisters and peels, particularly in the mouth and eyes, but potentially anywhere on the body. This skin sloughing can lead to raw and bleeding areas, exposing the person to potential infection.

Diagnosis

Diagnosis of SJS is based on the characteristic symptoms, a physical examination, and the individual's medical history. The doctor may also order skin biopsy and cultures to rule out other causes of the skin lesions. In some cases, a blood test may be performed to assess the individual's overall health and organ function, as SJS can cause complications that affect the lungs, heart, and kidneys.

Treatment

There is no specific cure for SJS; instead, treatment focuses on eliminating the underlying cause, controlling symptoms, and minimizing complications. The person will usually need to be hospitalized and, in severe cases, treated in an intensive care unit or a burn unit. Treatment may include discontinuing the medication that caused the reaction, providing fluids and electrolytes to prevent dehydration, and administering pain medication. If an infection is present, antibiotics or antiviral medications may be prescribed.

Complications

Complications of SJS can be severe and may include dehydration, sepsis, pneumonia, and multiple organ failure. Long-term complications can include permanent skin damage, scarring, changes in skin coloration, and damage to the eyes, which can result in blindness.

See Also

• Toxic epidermal necrolysis
• Erythema multiforme
• Drug reaction with eosinophilia and systemic symptoms

References

• Habif, T.P. (2015). "Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis". In Clinical Dermatology (6th ed.). Elsevier Health Sciences.
• Revuz J, Penso D, Roujeau JC, Guillaume JC, Payne CR, Wechsler J, Touraine R (1987). "Toxic epidermal necrolysis. Clinical findings and prognosis factors in 87 patients". Archives of Dermatology, 123(9):1160–5.
• Assier H, Bastuji-Garin S, Revuz J, Roujeau JC (1995). "Erythema multiforme with mucous membrane involvement and Stevens-Johnson syndrome are clinically different disorders with distinct causes". Archives of Dermatology, 131(5):539–43.
• Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC; SCAR Study Group. (2002). "Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study". Archives of Dermatology, 138(8):1019–24.
• Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS (2005). "Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature". Annals of Allergy, Asthma & Immunology, 94(4):419–36; quiz 436–8, 456.

External Links

• PubMed Health – Stevens-Johnson Syndrome
• Mayo Clinic - Stevens-Johnson Syndrome

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NIH genetic and rare disease info

• NIH info on Stevens–Johnson syndrome

Stevens–Johnson syndrome is a rare disease.