Stevens-Johnson syndrome (SJS) - PowerPoint PPT Presentation

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Stevens-Johnson syndrome (SJS)

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Stevens-Johnson syndrome. severe expression of erythema multiforme ... cat-scratch fever (Bartonella henselae) mycoplasma. lymphogranuloma venereum. Histoplasmosis ... – PowerPoint PPT presentation

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Title: Stevens-Johnson syndrome (SJS)


1
Stevens-Johnson syndrome (SJS)
  • Ri ???

2
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.

3
Stevens-Johnson syndrome
  • Etiologic categories
  • Infection
  • Drug-induced
  • Malignancy-related
  • Collagen vascular disease
  • Idiopathic
  • 25-50 of cases

4
Stevens-Johnson syndrome
  • Adults and the elderly
  • Drugs and malignancies induced are most often
  • Childs
  • Related more often due to infections

5
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

6
Stevens-Johnson syndrome
  • Individuals appear to be more susceptible to
    developing SJS
  • HLA-Bw44
  • A part of HLA-B12
  • HLA-DQB10601

7
Stevens-Johnson syndrome
  • Typical symptoms are as follows
  • Cough productive of a thick purulent sputum
  • Headache
  • Malaise
  • Arthralgia

8
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

9
  • Ocular symptoms
  • Red eye
  • Tearing
  • Dry eye
  • Pain
  • Itching
  • Foreign body sensation
  • Decreased vision
  • Burn sensation
  • Photophobia
  • Diplopia

10
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

11
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

12
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)

13
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

14
  • Mucosal involvement may include erythema, edema,
    sloughing, blistering, ulceration, and necrosis.

15
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

16
History
  • Recurrences may occur if the responsible agent is
    not eliminated or reexposed

17
  • Drug etiologies include
  • Penicillins
  • Sulfas
  • Phenytoin (and related anticonvulsants)
  • Carbamazepine
  • Barbiturates
  • valdecoxib (COX-2 inhibitor)

18
  • Penicillin, sulfas, or phenytoin, had previously
    been prescribed to more than 2/3 of all patients
    with SJS.

19
  • Hypersensitivity reaction to drugs has been
    suggested that probably both
  • Immune hypersensitivity reactions
  • Mediated by toxic intermediates

20
  • 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

21
  • Elevated in serum level
  • TNF-a
  • IL-2 receptor
  • IL-6
  • CRP
  • However, none of these serologic tests is used
    routinely in diagnosing SJS

22
  • CBC may reveal
  • Normal WBC count or a nonspecific leukocytosis
  • A severely elevated WBC count indicates a
    superimposed bacterial infection

23
  • 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

24
  • Conjunctival biopsy
  • active ocular disease
  • subepithelial plasma cells lymphocyte
    infiltration
  • Lymphocytes are perivascular
  • The predominant is the helper T cell

25
  • 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

26
  • 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

27
  • 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
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