Stevens Johnson Syndrome Flashcards
What is Stevens Johnson syndrome (SJS)?
It is defined as a severe systemic reaction affecting the skin and mucosa, which can lead to epidermal detachment and mucosal loss
What is the pathophysiology of Stevens Johnson syndrome?
There is an immune-complex mediated hypersensitivity reaction, which is most commonly related to drug administration
What dermatological condition is Stevens Johnson syndrome a variant of? How do we differentiate between these conditions?
Toxic epidermal necrolysis (TENS)
It can be differentiated by the degree of skin and mucous membrane involvement
In Stevens Johnson syndrome, there is < 10% total body surface area (TBSA) involvement
In toxic epidermal necrolysism there is > 30% total body surface area (TBSA) involvement
What are the five risk factors associated with Stevens Johnson syndrome?
Drug Administration
Family History
Infection
Radiotherapy
HIV/AIDs
What nine drugs are associated with Stevens Johnson syndrome?
Trimethoprim
Sulfamethoxazole
Penicillin
Carbamazepine
Phenobarbital
Phenytoin
Lamotrigine
Valproate Acid
NSAIDs
What genes are associated with Stevens Johnson syndrome?
HLA Alleles
What four infections are associated with Stevens Johnson syndrome?
Mycoplasma Pneumonia
Herpes Virus
Epstein Barr Virus
Cytomegalovirus
What are the ten clinical features of Stevens Johnson syndrome?
Prodromal Flu-Like Illness
Fever > 39C
Sore Throat
Dysphagia
Conjunctivitis
Arthralgia
Painful Dusky Erythematous Rash
Epidermal Detachment
Nikolsky Sign Positive
Mucosal Ulceration
Describe the rash associated with Stevens Johnson syndrome
‘Target Lesions’
The rash starts in the trunk region, as clusters of macules
It then extends rapidly over hours to days onto the face and limbs, in which the macules begin to blister
What is Nilkolsky’s sign?
It is when the epidermal layer easily sloughs off when pressure is applied to the affected area
Which ten regions of the body tend to be affected by mucosal ulceration in Stevens Johnson syndrome?
Eyes
Lips
Mouth
Oesophagus
Gastrointestinal Tract
Kidneys
Liver
Anus
Genital Area
Urethra
What investigation is used to diagnose Stevens Johnson syndrome?
Skin Biopsy
Where should skin biopsies be conducted when investigating Stevens Johnson syndrome? Why?
It should be taken at the transition point of the blistering
This allows assessment of the level of desquamation
What are the three histological features of Stevens Johnson syndrome?
Keratinocyte Necrosis
Full-Thickness Epidermal Necrosis
Minimal Inflammation
Where should Stevens Johnson syndrome patients be managed?
Secondary Care
