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Understanding
SJS and TEN
Presented by
Jacynta F Pepin (RN)
Stevens-Johnson Syndrome (SJS) and
Toxic Epidermal Necrolysis (TEN)
 Life-threatening mucocutaneous diseases
 Within the spectrum of SCAR
• Resemble erythema multiforme majus (EMM)
• Mucosal involvement
• Epidermal necrosis
SJS and TEN
• SJS usually less severe
• Etiology, genetic susceptibility and
pathomechanism are same
• Mainly cause by drugs, infection or unknown
• Presents as medical emergency
Signs and Symptoms
Early sign:
• Fever
• Sore throat
• Cough
• Burning eyes
Signs and Symptoms
• Facial swelling
• Tongue swelling
• Hives
• Skin pain
• A red or purple skin rash that spreads within
hours to days
• Blisters on skin and mucous membranes
• Shedding (sloughing) of skin
Clinical Presentation
• Typical Targets with three concentric
zones
Clinical Presentation
• Confluent purpuric
macules and limited
areas of skin
detachment
• Nikolsky sign is
positive in SJS/TEN
Nikolsky Sign
• skin can be pushed
slightly aside by
pressure of fingers
• refer to the base of
the blister, and thus
to the level of
epidermal separation
Clinical Presentation
• Detachment of large epidermal sheets in
SJS/TEN overlap
Drug Causes
Infectious causes
• Herpes (herpes simplex or herpes zoster)
• Influenza
• HIV
• Diphtheria
• Typhoid
• Hepatitis
Risk Factor
• Almost equal in ratio men:female
• Mortality rate:
– SJS 10%
– SJS / TEN ovelap 30%
– TEN 50%
Therapeutic Consideration
• Treatment focuses on eliminating the
underlying cause, controlling symptoms and
minimizing complications.
• Recovery can take weeks to months,
depending on the severity of condition.
Topical Treatment
• Blister should be left in place
• Erosion: Chlorhexidine, octenisept,
polyhexanide
• High room temp
• Debride skin under GA and apply allograft
Supportive Treatment
• ICU / Burn Unit
• Fluid replacement 0.7ml/kg/%BSA affected
• Albumin 1ml/kg/%skin detachment
• Nutritional
Medications
• Analgesic
• Antihistamines
• Antibiotics, when needed
• Steroids (topical/oral)
• Intravenous corticosteroids
• Immunoglobulin intravenous (IVIG)
Complication
• Transdermal fluid loss- hypovolumia
• Electrolyte imbalance- katabolic metabolism
• Septicemia – usually induced from CVL
• Multiorgan failure
Summary
• SJS and TEN are considered as one disease
entity of different severity.
• SJS/TEN is mainly caused by drugs, but also by
infections and probably other risk factors not
yet identified.
Summary
• The cytolytic protein granulysin was identified
a marker for the severity of the disease based
on skin detachment.
• No treatment has been identified to be
capable of halting the progression of skin
detachment yet.
Summary
• supportive management is crucial to improve
the patient’s state.
• Despite all therapeutic efforts, mortality is
high and increases with disease severity,
patients’ age and underlying medical
conditions.
Thank You

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What is stevens johnson syndrome?

  • 1. Understanding SJS and TEN Presented by Jacynta F Pepin (RN)
  • 2. Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)  Life-threatening mucocutaneous diseases  Within the spectrum of SCAR • Resemble erythema multiforme majus (EMM) • Mucosal involvement • Epidermal necrosis
  • 3. SJS and TEN • SJS usually less severe • Etiology, genetic susceptibility and pathomechanism are same • Mainly cause by drugs, infection or unknown • Presents as medical emergency
  • 4. Signs and Symptoms Early sign: • Fever • Sore throat • Cough • Burning eyes
  • 5. Signs and Symptoms • Facial swelling • Tongue swelling • Hives • Skin pain • A red or purple skin rash that spreads within hours to days • Blisters on skin and mucous membranes • Shedding (sloughing) of skin
  • 6. Clinical Presentation • Typical Targets with three concentric zones
  • 7. Clinical Presentation • Confluent purpuric macules and limited areas of skin detachment • Nikolsky sign is positive in SJS/TEN
  • 8. Nikolsky Sign • skin can be pushed slightly aside by pressure of fingers • refer to the base of the blister, and thus to the level of epidermal separation
  • 9. Clinical Presentation • Detachment of large epidermal sheets in SJS/TEN overlap
  • 10.
  • 12. Infectious causes • Herpes (herpes simplex or herpes zoster) • Influenza • HIV • Diphtheria • Typhoid • Hepatitis
  • 13. Risk Factor • Almost equal in ratio men:female • Mortality rate: – SJS 10% – SJS / TEN ovelap 30% – TEN 50%
  • 14. Therapeutic Consideration • Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications. • Recovery can take weeks to months, depending on the severity of condition.
  • 15. Topical Treatment • Blister should be left in place • Erosion: Chlorhexidine, octenisept, polyhexanide • High room temp • Debride skin under GA and apply allograft
  • 16. Supportive Treatment • ICU / Burn Unit • Fluid replacement 0.7ml/kg/%BSA affected • Albumin 1ml/kg/%skin detachment • Nutritional
  • 17. Medications • Analgesic • Antihistamines • Antibiotics, when needed • Steroids (topical/oral) • Intravenous corticosteroids • Immunoglobulin intravenous (IVIG)
  • 18. Complication • Transdermal fluid loss- hypovolumia • Electrolyte imbalance- katabolic metabolism • Septicemia – usually induced from CVL • Multiorgan failure
  • 19. Summary • SJS and TEN are considered as one disease entity of different severity. • SJS/TEN is mainly caused by drugs, but also by infections and probably other risk factors not yet identified.
  • 20. Summary • The cytolytic protein granulysin was identified a marker for the severity of the disease based on skin detachment. • No treatment has been identified to be capable of halting the progression of skin detachment yet.
  • 21. Summary • supportive management is crucial to improve the patient’s state. • Despite all therapeutic efforts, mortality is high and increases with disease severity, patients’ age and underlying medical conditions.