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Stevens-Johnsons Syndrome

Stevens-Johnsons Syndrome

Erythema multiforme on the hands (source: www.nlm.nih.gov)

National Library of Medicine

Stevens-Johnsons Syndrome, or Erythema multiforme, is a skin disorder resulting from an allergic reaction.


Causes, Incidence, and Risk Factors


Erythema multiforme is a type of hypersensitivity (allergic) reaction that occurs in response to medications, infections, or illness. Medications associated with erythema multiforme include sulfonamides, penicillins, barbiturates, and phenytoin. Associated infections include herpes simplex and mycoplasma infections.

The exact cause is unknown. The disorder is believed to involve damage to the blood vessels of the skin with subsequent damage to skin tissues. Approximately 90% of erythema multiforme cases are associated with herpes simplex or mycoplasma infections. The disorder occurs primarily in children and young adults.

Erythema multiforme may become noticeable with a classic skin lesion, with or without systemic (whole body) symptoms. In Stevens-Johnson syndrome, the systemic symptoms are severe and the lesions are extensive, involving multiple body areas, especially the mucous membranes. Toxic epidermal necrolysis (TEN syndrome, or Lyell’s syndrome) involves multiple large blisters (bullae) that coalesce, followed by sloughing of all or most of the skin and mucous membranes.


Symptoms


Multiple skin lesions:

  • With sudden onset, which may recur
  • That may spread
  • That may appear as nodule, papule, or macule
  • Central lesion surrounded by concentric rings of paleness and redness, also called “target”, “iris”, or “bull’s eye”
  • May have vesicles and bullae (blisters of various sizes)
  • Located on the legs, arms, palms, hands, or feet
  • May involve the face or lips
  • Trunk is usually not involved
  • Usually symmetrical

Other symptoms may include:

  • Itching of the skin may be present
  • Fever
  • General ill feeling
  • Joint aches
  • Vision abnormalities
  • Dry eyes
  • Bloodshot eyes
  • Eye pain
  • Eye burning, itching and discharge
  • Mouth sores

Signs and Tests


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

There may be a positive Nikolsky’s sign.

A skin lesion biopsy and microscopic examination may be helpful to differentiate erythema multiforme from other disorders. Erythema multiforme may show tissue death and other changes. Microscopic examination of the tissue may also show antibody deposits.


Treatment


Treatment goals include control of the underlying causes or illnesses, treatment of the symptoms, and prevention of infection. Suspected medications should be discontinued.

Treatment of mild symptoms may include:

  • Moist compresses applied to skin lesions
  • Medications such as antihistamines to control itching
  • Over-the-counter medications (such as acetaminophen) to reduce fever and discomfort
  • Topical anesthetics (especially for mouth lesions) to ease discomfort that interferes with eating and drinking

Treatment of severe symptoms may include:

  • Hospitalization and treatment in an intensive care or burn care unit for severe cases, Stevens-Johnson syndrome, and toxic epidermal necrolysis
  • Systemic corticosteroids to control inflammation
  • Intravenous immunoglobulins (IVIG) to stop the process
  • Antibiotics to control secondary skin infections

Good hygiene and isolation from others may be required to prevent secondary infections.

Extensive skin involvement may cause the loss of large quantities of body fluids, causing shock in addition to the risk of infection. Intensive care with support of body systems may be required.

Skin grafting may be helpful in cases in which large areas of the body are affected.

In cases that are caused by the herpes virus, daily antiviral medications may be prescribed to prevent recurrences of erythema multiforme.


Expectations


Mild forms of erythema multiforme usually resolve without difficulty in 2 – 6 weeks, but they may recur. More severe forms may be difficult to treat. Stevens-Johnson syndrome and toxic epidermal necrolysis are associated with high death rates.


Complications

  • Permanent skin damage and scarring
  • Occasionally, lesions on internal organs causing: Pneumonitis (lung inflammation), Myocarditis (heart inflammation), Nephritis (kidney inflammation), and Hepatitis (liver inflammation)
  • Secondary skin infection (cellulitis)
  • Systemic infection, sepsis
  • Loss of body fluids, shock

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  • Photo_user_blank_big

    elisemoren

    almost 7 years ago

    2 comments

    Hi!! I am a 21 year old girl from Norway. I got Stevens-Johnsons syndrom in August 07. I don`t remember much, because I was in a coma most of the time, and when I was awake, I got morphine. But the doctors cut my hair.. I did also loose all of my nails, just some of them are back... I have big weight issues, too skinny!!! I lost over 60 % of my skin( how I did survive is amazing), I thought I would never have the face I had before I got sick, but suprisingly enough, my face has healed the best!! My privates were touched by this too, I have had 2 plastic operations to fix it, and 1 eye operation, and guess what I feel great now!! It was a really tough time for me afterwords, depressed and stuff, but I came back:) God gave me a second chance:D

  • Rasta_max50

    rickland_92

    over 6 years ago

    4 comments

    This disease really gives patient's suffering..I had given care to pediatric patients diagnosed with such. The sad thing is, all we could do is supportive care. So sorry to hear of your beloved child ms.monica.

  • Photo_user_blank_big

    monicalucia

    over 7 years ago

    2 comments

    thanks for the article...what is most surprising is that the md who are the experts in this field had never seen the sjs so early on....if the did it could have saved alot of pain for her...nonetheless because of it we have learned much and are sharing same

  • Photo_user_blank_big

    FinallyNurse

    over 7 years ago

    4 comments

    I am currently caring for an elderly lady with this disease. I am soooo sorry to hear about your daughter. I have a 5 year old and I can't even begin to imagine!

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