Stevens-Johnson Syndrome Flashcards

1
Q

What is 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 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
  • Sulfasalazine
  • 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
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14
Q

What bacteria can cause SJS?

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

What protozoa can cause SJS?

A
  • Malaria

- Trichomoniasis

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16
Q

What other than infection and medications can cause SJS?

A

Immunisations

17
Q

What immunisations can cause SJS?

A
  • Measles

- Hep B

18
Q

What are the risk factors for SJS?

A
  • HIV/AIDS
  • SLE
  • Genetic factors
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 and malaise
20
Q

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

A

It suddenly develop Mucocutaneous lesions

21
Q

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

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
What symptoms may present if there is Genitourinary involvement in SJS?
Dysuria or inability to pass urine
26
What symptoms may present if there is Ocular involvement in SJS?
- Painful red eyes - Purulent conjunctivitis - Photophobia - Blepharitis
27
How is SJS diagnosed?
On the basis of Clinical Classification and Histopathology of Skin Biopsy
28
What will Histopathology show in SJS?
The Bullae are sub-epidermal and there may be epidermal cell necrolysis?
29
What investigations are important in SJS to assess severity and level of Dehydration?
- Electrolytes - Glucose - Bicarbonate
30
What are the DDx for SJS?
- Bullous pemphygoid - Chemical or thermal burns - Erythroderma - Exfoliative dermatitis - Maculopapular drug rashes - Staphylococcal scalded skin syndrome