Stevens-Johnson Syndrome

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Stevens-Johnson Syndrome

• A Brief Summary

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Source

• Uptodate Articles

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Introduction

• Stevens-Johnson syndrome (SJS) and toxic
  epidermal necrolysis (TEN) are severe
  idiosyncratic reactions.
• They are most commonly triggered by medications,
  which are characterized by fever and
  mucocutaneous lesions leading to necrosis and
  sloughing of the epidermis.
• SJS and TEN are distinguished chiefly by severity
  and percentage of body surface involved.

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Stevens-Johnson syndrome

• SJS is the less severe condition, in which skin
  sloughing is limited to less than 10 percent of
  the body surface.
• It is characterized by a prodrome of malaise and
  fever, followed by the rapid onset of
  erythematous or purpuric macules and plaque.
• The skin lesions progress to epidermal necrosis
  and sloughing.
• Mucosal membranes are affected in 92 to 100
  percent of patients, usually at two or more
  distinct sites (ocular, oral, and genital)

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Toxic epidermal necrolysis

• Toxic epidermal necrolysis (TEN), or Lyell's
  syndrome, involves sloughing of greater than 30
  percent of the body surface area.
• TEN also begins with a prodrome of fever and
  malaise, although temperatures are typically
  higher than those seen with SJS, often exceeding
  39 degrees Celsius.
• Mucous membranes are involved in nearly all
  cases.

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TEN (continued)

• The skin lesions are widely distributed
  erythematous macules and patches, although about
  50 percent of cases begin with diffuse erythema.
• The skin lesions progress to full-thickness
  epidermal necrosis leads.
• The ultimate appearance of the skin has been
  likened to that of extensive thermal injury.

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SJS/TEN overlap syndrome

•  SJS/TEN overlap syndrome describes patients with
  involvement of greater than 10 percent, but less
  than 30 percent of body surface area.

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Etiology

• Medications are the leading trigger of SJS and
  TEN in both adults and children.
• In adults, medications cause 30 to 50 percent of
  cases of SJS and up to 80 percent of cases of
  TEN.
• Infections are the next most common trigger of
  adult SJS (up to 15 percent).
• Rare causes of SJS and TEN include vaccinations,
  systemic diseases, chemical exposure, herbal
  medicines, and foods.

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Medications

• The following groups of agents are most commonly
  implicated
• Anti-gout agents (especially allopurinol)
• Antibiotics (sulfonamides gtgt penicillins gt
  cephalosporins)
• Antipsychotics and anti-epileptics
  (including carbamazepine, dilantin,lamotrigine,
  and phenobarbital)
• Analgesics and non-steroidal anti-inflammatory
  agents (especially piroxicam)

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HISTORY AND CLINICAL PRESENTATION

• Drug exposure commonly precedes the onset of
  symptoms by one to three weeks (average 14 days)
  in medication-related cases.
• Reexposure may result in onset of symptoms in as
  little as 48 hours.

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Prodrome 

• SJS and TEN typically have a prodrome of fever
  and influenza-like symptoms one to three days
  before the development of mucocutaneous lesions.
• Fever is usually higher with TEN, and often
  exceeds 39 degrees Celsius.
• Skin tenderness, photophobia, and conjunctival
  itching or burning may be early symptoms in both
  conditions.

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Clinical presentation

• The following signs and symptoms, when present
  early in the course of a drug reaction or
  illness, should alert clinicians to the
  possibility of SJS/TEN
• Confluent erythema (erythroderma)
• Facial edema or central facial involvement
• Skin pain
• Palpable purpura
• Skin necrosis
• Blisters and/or epidermal detachment
• Mucous membrane erosions and crusting
• Swelling of tongue

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Clinical presentation

• There may be multiorgan involvement.
• In the absence of complications, the disorder
  generally resolves sufficiently that the patient
  can be discharged from the hospital in two to
  four weeks.

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diagnosis

• the diagnosis of SJS or TEN would be appropriate
  in a patient with
• A suggestive history of antecedent drug exposure
  or illness
• A prodrome of acute-onset febrile illness and
  malaise
• Erythematous macules, targetoid lesions, or
  diffuse erythema progressing to vesicles and
  bullae
• Necrosis and sloughing of the epidermis (of
  varying degrees)

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diagnosis

• The diagnosis of SJS or TEN is clinical.
  Histologic findings on skin biopsy are
  supportive, but not independently diagnostic.
• The differential diagnosis includes
• erythema multiforme
• other types of severe medication reactions
• severe reactions to bacterial toxins (eg, toxic
  shock syndrome
• staphylococcal scalded skin syndrome)
• Kawasaki disease.

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Management

• Early recognition and immediate withdrawal of any
  potentially causative agents are critical first
  steps in the management of SJS/TEN.
• Multiple specialists should be involved in the
  care of patients with SJS/TEN when possible,
  including experts in
• critical care
• plastic surgery
• Dermatology
• infectious disease
• Ophthalmology
• nutrition

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To prevent possible sepsis

• Sepsis is the major cause of death. Sterile
  handling, infection control measures, topical
  antibiotic agents, and surveillance cultures of
  possible sites of superinfection are important
  components of prevention. 

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Supportive care gt adjunctive therapies

• Supportive care should be the primary focus of
  management of SJS/TEN.
• Beyond this, there is insufficient evidence to
  establish the benefit of any adjunctive
  therapies.
• Systemic glucocorticoids and intravenous
  gammaglobulin (IVIG) are commonly used at many
  centers, although not all.
• Other treatments plasmapheresis, thalidomide.

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Prognosis

• The mortality of SJS is 1 to 3 percent, while the
  mortality of TEN ranges from 25 to 35 percent.
• Predictors of mortality include older age at
  onset and greater extent of skin involvement.
• Long-term sequelae of the skin and eyes are
  common among survivors.

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End

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