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Stevens-Johnson Syndrome (SJS)
Stevens-Johnson Syndrome (SJS)
(SJS)
Ri 蔡明峰
Stevens-Johnson syndrome
severe expression of erythema multiforme
involves the skin and the mucous
membranes
oral, nasal, eye, vaginal, urethral, GI, and
lower respiratory tract mucous membranes
potential for severe morbidity and even
death.
Stevens-Johnson syndrome
Etiologic categories
Infection
Drug-induced
Malignancy-related
Collagen vascular disease
Idiopathic
25-50% of cases
Stevens-Johnson syndrome
Adults and the elderly
Drugs and malignancies induced are most
often
Childs
Related more often due to infections
Stevens-Johnson syndrome
Early spring and winter
Male-to-female ratio is 2:1
Caucasian predominance has been
reported
Mortality rate : 3-15% (or 30%)
second to fourth decade
have been reported as young as 3 months
Stevens-Johnson syndrome
Individuals appear to be more susceptible
to developing SJS
HLA-Bw44
A part of HLA-B12
HLA-DQB1*0601
Stevens-Johnson syndrome
Typical symptoms are as follows:
Cough productive of a thick purulent sputum
Headache
Malaise
Arthralgia
Stevens-Johnson syndrome
The following signs may be noted on exam:
Fever
Epistaxis
Tachycardia, hypotension
Conjunctivitis, corneal ulcerations
Erosive vulvovaginitis or balanitis
Altered level of consciousness, Seizures, coma
Ocular symptoms
Red eye Foreign body sensation
Tearing Decreased vision
Dry eye Burn sensation
Pain Photophobia
Itching Diplopia
History
Typically begins with a nonspecific upper
respiratory tract infection
1 to 14 days
fever, sore throat, chills, headache, vomiting,
diarrhea and malaise may be present
History
Mucocutaneous lesions develop abruptly
last 2-4 weeks.
typically are nonpruritic
may lead to mucosal scarring and loss of
function of the involved organ system
History
Esophagus involvement
Esophageal strictures
Tracheobronchial mucosa shedding
Respiratory failure
Ocular sequelae
Corneal ulceration
Anterior uveitis
Keratitis or panophthalmitis (3-10%blindness)
Vaginal stenosis and penile scarring
Renal complications (rare)
History
The rash can begin as macules that
develop into papules, vesicles, bullae,
urticarial plaques, or confluent erythema.
The center of these lesions may be vesicular,
purpuric, or necrotic.
The typical lesion has the appearance of a
target.
The target lesion exhibits central necrosis
surrounded by a rim of perivenular inflammation
Mucosal involvement may include
erythema, edema, sloughing, blistering,
ulceration, and necrosis.
History
Although lesions may occur anywhere, but the
rash may be confined to any one area of the
body
most often the trunk.
Lesions may become bullous and later rupture,
leaving denuded skin.
The skin becomes susceptible to secondary infection
Fever or localized worsening
Fever occur in up to 85% of cases
History
Recurrences may occur if the responsible
agent is not eliminated or reexposed
Drug etiologies include
Penicillins
Sulfas
Phenytoin (and related anticonvulsants)
Carbamazepine
Barbiturates
valdecoxib (COX-2 inhibitor)
Penicillin, sulfas, or phenytoin, had
previously been prescribed to more than
2/3 of all patients with SJS.
Hypersensitivity reaction to drugs has
been suggested that probably both
Immune hypersensitivity reactions
Mediated by toxic intermediates
Infectious diseases have been reported
herpes simplex virus (HSV)
Influenza
Mumps
cat-scratch fever (Bartonella henselae)
mycoplasma
lymphogranuloma venereum
Histoplasmosis
cholera
In children, Epstein-Barr virus and enteroviruses have
been identified
Elevated in serum level :
TNF-a
IL-2 receptor
IL-6
CRP
However, none of these serologic tests is
used routinely in diagnosing SJS
CBC may reveal
Normal WBC count or a nonspecific
leukocytosis
A severely elevated WBC count indicates a
superimposed bacterial infection
Typical histopathologic findings
Initially, dermal inflammatory cell infiltrate
superficial and mostly perivascular
Migration of lymphocytes along the
dermoepidermal junction
Keratinocytes undergo apoptosis
Full-thickness necrosis of epidermis
blister formation
Conjunctival biopsy
active ocular disease
subepithelial plasma cells & lymphocyte
infiltration
Lymphocytes are perivascular
The predominant is the helper T cell
Skin lesions are treated as thermal burns
Attention to
airway and hemodynamic stability
fluid status, wound/burn care
Electrolyte correction
pain control
Treatment of SJS is primarily supportive
and symptomatic
Offending drugs must be stopped
Underlying diseases and secondary infections
must be identified and treated
Oral lesions
mouthwashes
Topical anesthetics
Useful in reducing pain
Allowing the patient to take in fluids
Areas of denuded skin
Covered with compresses of saline
The systemic steroids use is controversial
Useful in high doses early in the reaction
But morbidity and mortality actually may
increase in association with steroid use
Because of increasing secondary infection rate
Some authors believe that they are
contraindicated