SJS/TEN Syndrome

Question 1

What is the rash associated with SJS?

Answer 1

• Erythematous rash that develops initially in clusters of macules
• Widespread over the face and all 4 limbs, began at the trunk
• The rash progresses to blisters
• Mucosal involvement is prominent and severe
• Rash is at it’s most prominent at day 4 of symptoms

Question 2

What is the rash associated with TEN syndrome?

Answer 2

• The blisters of SJS merge to form sheets of skin detachment (called desquamation) exposing red, oozing dermis
• Positive Nikolsky sign (Epidermal layer easily sloughs off when pressure is applied)

Question 3

What is the difference between SJS and TEN?

Answer 3

• They are variants of the same condition
• SJS covers <10% of TBSA
• TEN has >30% TBSA

TBSA - Total body surface area

Question 4

What are causes of these syndromes?

Answer 4

Medications

• Antibiotics (trimethoprim/sulfamethoxazole, sulphonamides)
• Anti epileptic drugs (carbamazepine, phenobarbital, phenytoin)
• Lots of medications

Infections -

• Mycoplasma pneumoniae
• Herpes, EBV, CMV

Other medical conditions -

• SLE
• HIV/AIDS
• Radiotherapy

Genetic predisposition

Question 5

What are clinical features of SJS/TENS?

Answer 5

• Prodromal flu like illness for several days
• Fever >39
• Sore throat, odynophagia, dysphagia
• Arthralgia, malaise
• Painful skin rash that starts at the trunk and extends rapidly over hours to days onto the face and limbs with blistering
• Mucosal ulceration of eyes, lips, mouth, genitals

Question 6

What investigations would you undertake?

Answer 6

• Clinical findings
• Skin biopsy taken at transition point of blistering to assess level of desquamation

Blood tests -

• Anaemia in all cases
• Leucopenia, lymphopenia is common
• Mildly deranged LFT’S
• U+E’s
• Blood culture to rule out Staph infection
• aBG to assess respiratory compromise

Others -
- CXR to rule out pneumonia

Question 7

What is the SCORTEN scoring system?

Answer 7

• Predicts mortality of patients with SJS/TEN

- Calculated within 24 hours of admission

Question 8

What is the management?

Answer 8

- Similar to second degree burns
Immediate - 
- Withdraw causative agent 
- ABCDE
- To burns centre/ICU
- Skin cleaned using sterile water, and non adherent dressings used 
- Greasy emollient used 
- Eye care, oral hygiene, fluid balance, analgesia

Question 9

What are complications of SJS/TEN?

Answer 9

• Dehydration
• Infection
• Hypothermia
• Ocular complications - conjunctivitis, tear duct loss, corneal ulcerations, anterior unveitis
• Acute liver injury, renal failure
• Shock
• Coagulopathy