What is stevens johnson syndrome?

A brief presentation in understanding of SJS.

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 P resents 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 t hree c oncentric zones

7. Clinical Presentation Confluent p urpuric m acules and limited a reas 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 e pidermal s heets in SJS/TEN overlap

10. Drug Causes

11. Infectious causes Herpes (herpes simplex or herpes zoster) Influenza HIV Diphtheria Typhoid Hepatitis

12. Risk Factor Almost equal in ratio men:female Mortality rate: SJS 10% SJS / TEN ovelap 30% TEN 50%

13. 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 .

14. Topical Treatment Blister should be left in place Erosion: Chlorhexidine , octenisept , polyhexanide High room temp Debride skin under GA and apply allograft

15. Supportive Treatment ICU / Burn Unit Fluid replacement 0.7ml/kg/%BSA affected Albumin 1ml/kg/%skin detachment Nutritional

16. Medications Analgesic Antihistamines Antibiotics, when needed S teroids (topical/oral) Intravenous corticosteroids I mmunoglobulin intravenous (IVIG)

17. Complication Transdermal fluid loss- hypovolumia Electrolyte imbalance- katabolic metabolism Septicemia – usually induced from CVL Multiorgan failure

18. S ummary 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.

19. Summary The cytolytic protein granulysin was identified a marker for the severity of the disease based on skin detachment. N o treatment has been identified to be capable of halting the progression of skin detachment yet.

20. 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.

21. Thank You I survived sjs