History
A 35-year-old female presents with a 2-day history of a sore throat, malaise and a rash. The cutaneous eruption has spread rapidly over the two days to involve her trunk, limbs and face. Her eyes feel gritty and sore. Her lips have become crusted and painful. Three weeks previously her antiepileptic medication had been changed from topiramate to carbamazepine.
Examination
The cutaneous eruption consists of dusky erythema which is patchy on her face and limbs but more confluent over her trunk. Between 20 and 30 per cent of the skin surface is involved in the eruption. Blisters are seen centrally within erythematous, atypical, target-like lesions and there are purpuric macules. Nikolsky’s sign is positive (sloughing-off of the epidermis under lateral pressure). She has chelitis with erythema, crusting and bleeding. There are erosions and small areas of skin necrosis (Fig. 22.1). The nasal mucosa and genital tract are spared.
Questions
• What is the most likely cause of this rapid-onset skin eruption?
• What management plan would you instigate?
• Which healthcare professionals should ideally be involved in her care?
Medicine
This patient has developed Stevens–Johnson syndrome (SJS) secondary to carbamazepine. SJS is a severe, immune-complex mediated, drug hypersensitivity eruption that affects the skin and mucous membranes. Patients feel unwell and should be admitted to hospital for high-dependency care. SJS usually develops within two months of commencing a new medication. Patients characteristically develop a sore throat, eyes, lips and genitals as the mucosae become inflamed and eroded. Skin lesions affect 10–30 per cent of the skin surface with a variable morphology: erythematous macules, atypical target lesions with central blisters, dusky purpuric lesions, erosions and necrosis. As the skin becomes denuded there is a high risk of secondary bacterial infection. Patients with SJS need management in hospital by a multidisciplinary team including dermatologists, high-dependency doctors and nurses, ophthalmologists and oral physi-cians. This patient also required care from the neurologists to determine and monitor an alternative anti-epileptic medication. The management of SJS is essentially supportive. The culprit medication should be stopped immediately (if the culprit is unclear then all mediations should be stopped if possible). Patients require intensive nursing care with hourly applications of topical 50:50 white soft paraffin with liquid paraffin, non-adherent dressings to denuded areas and a non-adherent bodysuit to help reduce cutaneous pain and secondary infection. Patients should be kept in a warm room and fluid balance monitored. Chlorhexidine mouthwash, regular eye drops, and supportive nutrition may be required. Active treat-ments may include prednisolone, methyl-prednisolone, intravenous immunoglobulin and/or ciclosporin.
KEY POINTS
• SJS is a serious drug hypersensitivity reaction that requires high dependency care.
• Skin lesions are of variable appearance but develop rapidly with mucous membrane involvement.
• Management is supportive plus treatment with oral prednisolone or other immunosuppressants.
need an explanation for this answer? contact us directly to get an explanation for this answer