Stevens-Johnson syndrome (SJS)

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Stevens-Johnson syndrome (SJS)

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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.

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Stevens-Johnson syndrome

• Etiologic categories
• Infection
• Drug-induced
• Malignancy-related
• Collagen vascular disease
• Idiopathic
• 25-50 of cases

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Stevens-Johnson syndrome

• Adults and the elderly
• Drugs and malignancies induced are most often
• Childs
• Related more often due to infections

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Stevens-Johnson syndrome

• Early spring and winter
• Male-to-female ratio is 21
• Caucasian predominance has been reported
• Mortality rate 3-15 (or 30)
• second to fourth decade
• have been reported as young as 3 months

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Stevens-Johnson syndrome

• Individuals appear to be more susceptible to
  developing SJS
• HLA-Bw44
• A part of HLA-B12
• HLA-DQB10601

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Stevens-Johnson syndrome

• Typical symptoms are as follows
• Cough productive of a thick purulent sputum
• Headache
• Malaise
• Arthralgia

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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

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• Ocular symptoms
• Red eye
• Tearing
• Dry eye
• Pain
• Itching

• Foreign body sensation
• Decreased vision
• Burn sensation
• Photophobia
• Diplopia

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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

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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

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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)

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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

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• Mucosal involvement may include erythema, edema,
  sloughing, blistering, ulceration, and necrosis.

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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

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History

• Recurrences may occur if the responsible agent is
  not eliminated or reexposed

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• Drug etiologies include
• Penicillins
• Sulfas
• Phenytoin (and related anticonvulsants)
• Carbamazepine
• Barbiturates
• valdecoxib (COX-2 inhibitor)

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• Penicillin, sulfas, or phenytoin, had previously
  been prescribed to more than 2/3 of all patients
  with SJS.

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• Hypersensitivity reaction to drugs has been
  suggested that probably both
• Immune hypersensitivity reactions
• Mediated by toxic intermediates

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• 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

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• Elevated in serum level
• TNF-a
• IL-2 receptor
• IL-6
• CRP
• However, none of these serologic tests is used
  routinely in diagnosing SJS

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• CBC may reveal
• Normal WBC count or a nonspecific leukocytosis
• A severely elevated WBC count indicates a
  superimposed bacterial infection

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• 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

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• Conjunctival biopsy
• active ocular disease
• subepithelial plasma cells lymphocyte
  infiltration
• Lymphocytes are perivascular
• The predominant is the helper T cell

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• 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

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• 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

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• 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