Stevens-Johnson syndrome

(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