Steven-Johnson Syndrome Flashcards
1
Q
What is Steven- Johnson Syndrome?
A
- This is mucocutaneous necrosis with at least 2 mucosal sites involved
- Rare, acute, serious, potentially fatal skin reaction in which there are sheet- like skin and mucosal loss
- Nearly always caused by medications
2
Q
Who gets SJS/ TEN?
A
- Can affect anyone on medication
-Affect all age groups and all races - Associated with HIV
- Genetic factors are important
3
Q
What are the clinical features of SJS/ TEN?
A
- develops within the first week of antibiotic therapy
- Prodromal illness for several days before - a flu like illness
- Symptoms: Fever, sore throat, runny nose and cough, sore red eyes, general aches and pains
-Skin lesions: macules - fat, red, diffuse erythema, target oil (erythema Multiformat), blisters (flaccid) - blisters merge and form sheets of skin detachment, exposing red and oozing dermis
4
Q
What is the Nikolsky Sign?
A
- Positive in areas of skin redness.
- This means that blisters and erosions appear when the skin is rubbed gently
5
Q
What investigations/ diagnosis is required for SJS/TEN?
A
- Skin biopsy
-Histopathology = keratinocyte necrosis
6
Q
What might you see with bloods on SJS/TEN?
A
- Anaemia
- Leucopenia
- Neutropenia
- Eosinophilia
- Raised Liver Enzymes = Hepatitis
- Mild Proteinuria
7
Q
What is SCORTEN?
A
- Scorten is an illness severity score that has been developed to predict the mortality in SJS and TEN
- One point for :
- age > 40
- presence of malignancy
- heart rate > 120
- epidermal detachment > 10%
- serum urea >10mmol
- serum glucose >14 mmol
- serum bicarbonate <20 mmol
8
Q
What are some of the causes of SJS?
A
- Penicillin
- Sulphonamides
- Lamotrigine, Carbamazepine, Phenytoin
- Allopurinol
- NSAIDs
- OCP
9
Q
What is the management for SJS?
A
- Stop the offending drug
- Hospital Admission to ITU
- Pain relief
- Fluid and Nutritional replacement (IV and NG routes)
- Temperature maintenance
- Use antiseptics
- Dressing such as gauze with pertrolatum
- Frequent eye drops
- Mouthwashes
