Stevens-Johnson Syndrome

Question 1

What is Steven’s-Johnson Syndrome (SJS)?

Answer 1

An immune complex mediated hypersensitivity disorder

Question 2

What does SJS form a spectrum with?

Answer 2

Toxic epidermal necrolysis (TEN)

Question 3

Which is less severe, SJS or TEN?

Answer 3

SJS

Question 4

When is the disease considered SJS not TEN?

Answer 4

Under 10% skin surface involvement

Question 5

What is SJS/TEN in terms of skin involvement?

Answer 5

10-30%

Question 6

How much skin is involved in TEN?

Answer 6

> 30%

Question 7

What sort of hypersensitivity reaction is SJS?

Answer 7

Type 4

Question 8

What happens in the type 4 hypersensitivity reaction of SJS?

Answer 8

A drug or its metabolite stimulate cytotoxic T cells and T helper cells to initiate autoimmune reactions that attack self-tissues

Question 9

What percentage of SJS cases are caused by medication?

Answer 9

75%

Question 10

What percentage of SJS cases are caused by infection and other causes?

Answer 10

25%

Question 11

What are some examples of medications that can cause SJS?

Answer 11

• Allopurinol
• Carbamazepine
• Sulfonamides
• Antiviral agents such as nevirapine and abacavir
• Anticonvulsants
• NSAIDs
• Salicylates
• Sertraline

Question 12

What types of infections can cause SJS?

Answer 12

• Viral
• Bacterial
• Fungal
• Protozoal

Question 13

What viral infections can cause SJS?

Answer 13

• HSV
• EBV
• Enteroviruses
• Others

Question 14

What bacteria can cause SJS?

Answer 14

• Group A β-haemolytic strep
• Diphtheria
• Mycobacteria

Question 15

What protozoa can cause SJS?

Answer 15

• Malaria

- Trichominiasis

Question 16

What other than infection and medications can cause SJS?

Answer 16

Immunisations

Question 17

What immunisations can cause SJS?

Answer 17

• Measles

- Hep B

Question 18

What are the risk factors for SJS?

Answer 18

• HIV/AIDS
• SLE
• Genetic factors

Question 19

How does SJS often start its presentation?

Answer 19

With a non-specific URTI that can be associated with fever, sore throats, headache, arthralgia, vomiting and diarrhoea, and malaise

Question 20

After URTI symptoms in early SJS, how does presentation progress?

Answer 20

To suddenly developing mucocutaneous lesions

Question 21

How long do the outbreaks of mucocutaneous lesions in SJS typically last?

Answer 21

2-4 weeks

Question 22

Are SJS lesions pruritic?

Answer 22

Not generally

Question 23

How do the mucocutaneous lesions of SJS present in the mouth?

Answer 23

As severe oromucosal ulceration

Question 24

How can respiratory involvement present in SJS?

Answer 24

As a cough productive of thick, purulent sputum

Question 25

What symptoms may present if there is genitourinary involvement in SJS?

Answer 25

Dysuria or inability to pass urine

Question 26

What symptoms may present if there is ocular involvement in SJS?

Answer 26

- Painful red eye - Purulent conjunctivitis - Photophobia - Blepharitis

Question 27

How is SJS diagnosed?

Answer 27

On the basis of clinical classification and histopathology of skin biopsy

Question 28

What will histopathology show in SJS?

Answer 28

The bullae are subepidermal and there may be epidermal cell necrolysis

Question 29

What investigations are important in SJS to assess severity and level of dehydration?

Answer 29

- Electrolytes - Glucose - Bicarbonate

Question 30

What are the differentials for SJS?

Answer 30

- Bullous pemphygoid - Chemical or thermal burns - Erythroderma - Exfoliative dermatitis - Maculopapular drug rashes - Staphylococcal scalded skin syndrome

Question 31

Is SJS worrying?

Answer 31

Yes, its a dermatological emergency 🚨

Question 32

When managing SJS what should be done if possible?

Answer 32

Identify the causative agent and withdraw immediately

Question 33

What system can be used to rapidly assess prognosis of SJS?

Answer 33

SCORTEN system

Question 34

What score on the SCORTEN system for assessing SJS indicates need for ITU treatment?

Answer 34

3 or more

Question 35

What are the skin lesions in SJS managed the same as?

Answer 35

Burns

Question 36

What supportive therapy is used in treating SJS?

Answer 36

- Maintain airway - Maintain haemodynamic stability - Correct fluid and electrolyte loss IV - Pain relief

Question 37

What can occur at lesion sites in SJS?

Answer 37

Secondary infection

Question 38

How can oral lesions be managed in SJS?

Answer 38

Mouthwashes of topical anaesthetics to reduce pain and allow fluid intake

Question 39

How should ocular SJS be managed?

Answer 39

Frequent ophthalmology assessment and frequent eye drops including antibiotics and steroids where required

Question 40

What are the potential complications of SJS?

Answer 40

- Dehydration - Malnutrition - Shock and multi-organ failure - Thromboembolism and DIC - Secondary infection - Scarring - GI complications - Eye complications - Mucosal shedding of airways leading to respiratory failure

Question 41

What are the potential GI complications of SJS?

Answer 41

- Ulceration - Necrolysis - Strictures - Perforation

Question 42

What are the potential eye complications of SJS?

Answer 42

- Corneal ulceration - Anterior uveitis - Sight impairment

Question 43

What are the long term skin sequelae that some survivors of SJS experience?

Answer 43

- Hyperhidrosis - Hair loss - Heat and cold sensitivity - Scarring - Irregular pigmentation

Question 44

What are the long term mucous membrane sequelae that som survivors of SJS experience?

Answer 44

Vaginal, urethral and anal strictures

Question 45

What are the ocular sequelae that some survivors of SJS experience?

Answer 45

- Photophobia | - Sight impairment