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A brief presentation in understanding of SJS.
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Understanding SJS and TEN
Presented byJacynta F Pepin (RN)
Stevens-Johnson Syndrome (SJS) andToxic 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
I SURVIVED SJS