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Stevens-Johnson Syndrome
A Brief Summary
Source
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Uptodate Articles
Introduction
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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.
Stevens-Johnson syndrome
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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)
Toxic epidermal necrolysis
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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.
TEN (continued)
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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 fullthickness epidermal necrosis leads.
The ultimate appearance of the skin
has been likened to that of extensive
thermal injury.
SJS/TEN overlap
syndrome
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SJS/TEN overlap syndrome describes
patients with involvement of greater
than 10 percent, but less than 30
percent of body surface area.
Etiology
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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,
Medications
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The following groups of agents are
most commonly implicated :
Anti-gout agents
(especially allopurinol)
Antibiotics (sulfonamides >>
penicillins > cephalosporins)
Antipsychotics and anti-epileptics
(including carbamazepine,
dilantin,lamotrigine,
and phenobarbital)
Analgesics and non-steroidal antiinflammatory agents (especially
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
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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.
Clinical presentation
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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
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
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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)
diagnosis
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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.
Management
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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
To prevent possible sepsis
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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.
Supportive care > adjunctive therapies
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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.
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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