Clinical features

Clinical features typically develop 1-3 weeks after the drug or infection exposure (1).

There may be an initial prodromal phase which includes fever, arthralgia, malaise, headache, vomiting, diarrhoea, and myalgia (2). This is followed by severe mucocutaneous lesions which are bullous and ulcerating.

Skin lesions:

• Skin lesions may appear as erythematous macules or as target lesions (2)
• The typical target lesions contain 3 rings - a bright-pink or red inner ring, a lighter-pink outer ring, and a darker-pink outermost ring
• skin detachment can be seen following these lesions which is the dominant clinical feature of SJS
• skin lesions can be pruritic and painful (1)
• lesions are seen in the extremities initially but may spread to involve the trunk as well (3)

Mucosal lesions:

• Mucosal lesions are common and typically involve 2 or more sites such as oral, ocular, or genitourinary (1)

Sites of ulceration include:

• mouth and fauces - causes a painful stomatitis
• respiratory tract
• gastrointestinal tract - erosions in the oesopahgus may cause dysphagia (1)
• the urethra - thus a non-gonococcal urethritis
• ocular - conjunctivitis, keratitis and corneal scarring (1)
• the skin

Reference:

• 1. Ravin K.A et al. Mycoplasma pneumoniae and Atypical Stevens-Johnson Syndrome: A Case Series. Pediatrics 2007;119: e1002-e1005
• 2. Segal A.R et al. Cutaneous Reactions to Drugs in Children. Pediatrics 2007;120:e1082-e1096 3. Hackle J.G et al. Photo quiz - Rash in an adolescent. AFP 2004;70(3)
• 3. Hackle J.G et al. Photo quiz - Rash in an adolescent. AFP 2004;70(3)