SJS and TEN are life-threatening mucocutaneous diseases within the spectrum of severe cutaneous adverse reactions (SCAR) that can be caused by drugs, infections, or unknown factors. They involve epidermal necrosis and sloughing of the skin and mucosal surfaces. SJS usually presents as less severe than TEN. While no treatment can halt skin detachment progression, supportive care including fluid replacement, nutrition, analgesics, and antibiotics when needed is crucial, though mortality increases with severity and underlying patient conditions.
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
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
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
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
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.