paeds derm Flashcards
what is stevens johnson syndrome
a severe systemic reaction affecting the skin and mucosa that is almost always caused by drug reactions
Defined as severe skin detachment with mucocutaneous complications
Aetiology of stevens johnson syndrome
infection: URTI, pharyngitis, otitis media, herpes
Vaccinations: small pox
Medications:
- anticonvulsants
- antibiotics
- antifungals
- NSAIDs
- penicillin
- allopurinol
- oral contraceptive pill
- sulphonamides
patients with what disease are at an increased risk of SJS
patients with HIV and active cancer
pathophysiology of stevens johnson syndrome
immune reaction to foreign antigens- immune complex mediated
what can stevens johnson develop into ?
toxic epidermal necrolysis
How does stevens johnson syndrome present?
rash- maculopapular with target lesions, can develop into vesicles and bullae
Nikolsky’s sign- the epidermal layer sloughs easily off when pressure is applied
erosions and ulcerations of eyes, lips, mouth, pharynx, oesophagus, GI tract
Systemic symptoms- fever, arthralgia
differentials for stevens johnsons syndrome
staphyloccocal scalded skin
toxic shock syndrome
erythema multiforme
burns
What is the definitive test for stevens johnsons syndrome
skin biopsy - shows keratinocyte apoptosis with detachment of the epidermal layer of the skin from the dermal layer
1st line management of stevens johnsons syndrome
hospital admission and removal of the causative agent
treatment of stevens johnson syndrome
- hospital admission and removal of the causative agent
- VTE prophylaxis
- surgical wound care
- analgesia
- fluid and electrolyte management
- nutritional support
- PPI
May consider IV immunoglobulins
what percent of the skin is involved in stevens johnson syndrome
less than 10%
what percent of the skin is involved in toxic epidermal necrolitis
at least 30%
mortality rate of stevens johnsons syndrome
10%
what can cause mortality in stevens johnsons syndrome (3)
dehydration
infection
disseminated intravascular coagulation
