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4: Y4 - Dermatology [3] > [4] Stevens-Johnson Syndrome > Flashcards

[4] Stevens-Johnson Syndrome Flashcards

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Dermatology Board Prep flashcards
1
Q

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

A

An immune complex mediated hypersensitivity disorder

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2
Q

What does SJS form a spectrum with?

A

Toxic epidermal necrolysis (TEN)

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3
Q

Which is less severe, SJS or TEN?

A

SJS

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4
Q

When is the disease considered SJS not TEN?

A

Under 10% skin surface involvement

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5
Q

What is SJS/TEN in terms of skin involvement?

A

10-30%

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6
Q

How much skin is involved in TEN?

A

> 30%

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7
Q

What sort of hypersensitivity reaction is SJS?

A

Type 4

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8
Q

What happens in the type 4 hypersensitivity reaction of SJS?

A

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

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9
Q

What percentage of SJS cases are caused by medication?

A

75%

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10
Q

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

A

25%

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11
Q

What are some examples of medications that can cause SJS?

A
  • Allopurinol
  • Carbamazepine
  • Sulfonamides
  • Antiviral agents such as nevirapine and abacavir
  • Anticonvulsants
  • NSAIDs
  • Salicylates
  • Sertraline
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12
Q

What types of infections can cause SJS?

A
  • Viral
  • Bacterial
  • Fungal
  • Protozoal
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13
Q

What viral infections can cause SJS?

A
  • HSV
  • EBV
  • Enteroviruses
  • Others
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14
Q

What bacteria can cause SJS?

A
  • Group A β-haemolytic strep
  • Diphtheria
  • Mycobacteria
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15
Q

What protozoa can cause SJS?

A
  • Malaria

- Trichominiasis

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16
Q

What other than infection and medications can cause SJS?

A

Immunisations

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17
Q

What immunisations can cause SJS?

A
  • Measles

- Hep B

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18
Q

What are the risk factors for SJS?

A
  • HIV/AIDS
  • SLE
  • Genetic factors
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19
Q

How does SJS often start its presentation?

A

With a non-specific URTI that can be associated with ;

  • Fever
  • Sore throats
  • Headache
  • Arthralgia
  • Vomiting and diarrhoea
  • Malaise
20
Q

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

A

To suddenly developing mucocutaneous lesions

21
Q

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

A

2-4 weeks

22
Q

Are SJS lesions pruritic?

A

Not generally

23
Q

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

A

As severe oromucosal ulceration

24
Q

How can respiratory involvement present in SJS?

A

As a cough productive of thick, purulent sputum

25
Q

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

A

Dysuria or inability to pass urine

26
Q

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

A
  • Painful red eye
  • Purulent conjunctivitis
  • Photophobia
  • Blepharitis
27
Q

How is SJS diagnosed?

A

On the basis of clinical classification and histopathology of skin biopsy

28
Q

What will histopathology show in SJS?

A

The bullae are subepidermal and there may be epidermal cell necrolysis

29
Q

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

A
  • Electrolytes
  • Glucose
  • Bicarbonate
30
Q

What are the differentials for SJS?

A
  • Bullous pemphygoid
  • Chemical or thermal burns
  • Erythroderma
  • Exfoliative dermatitis
  • Maculopapular drug rashes
  • Staphylococcal scalded skin syndrome
31
Q

Is SJS worrying?

A

Yes, its a dermatological emergency 🚨

32
Q

When managing SJS what should be done if possible?

A

Identify the causative agent and withdraw immediately

33
Q

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

A

SCORTEN system

34
Q

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

A

3 or more

35
Q

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

A

Burns

36
Q

What supportive therapy is used in treating SJS?

A
  • Maintain airway
  • Maintain haemodynamic stability
  • Correct fluid and electrolyte loss IV
  • Pain relief
37
Q

What can occur at lesion sites in SJS?

A

Secondary infection

38
Q

How can oral lesions be managed in SJS?

A

Mouthwashes of topical anaesthetics to reduce pain and allow fluid intake

39
Q

How should ocular SJS be managed?

A

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

40
Q

What are the potential complications of SJS?

A
  • 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
41
Q

What are the potential GI complications of SJS?

A
  • Ulceration
  • Necrolysis
  • Strictures
  • Perforation
42
Q

What are the potential eye complications of SJS?

A
  • Corneal ulceration
  • Anterior uveitis
  • Sight impairment
43
Q

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

A
  • Hyperhidrosis
  • Hair loss
  • Heat and cold sensitivity
  • Scarring
  • Irregular pigmentation
44
Q

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

A

Vaginal, urethral and anal strictures

45
Q

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

A
  • Photophobia

- Sight impairment

4: Y4 - Dermatology [3] (26 decks)

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