Intravenous immunoglobulin in the treatment of childhood Stevens Johnson syndrome

Abstract

A 12-year-old boy of Polynesian background, who had been previously well, presented with swollen blistering lesions involving his lips and oral mucosa which then progressed to involve his limbs and genitalia over 3 days. He was unable to eat and drink and had difficulty passing urine because of pain. A diagnosis of Stevens Johnson syndrome (SJS) was made. The question of whether intravenous immunoglobulin (IVIG) would be effective in treating this condition was raised. SJS is a severe mucocutaneous reaction characterised by destruction of epithelial cells resulting in widespread blisters with involvement of at least two mucosal membranes. It has a clinical course of up to 6 weeks with a high morbidity and mortality of 5–10%. The current management of SJS is primarily supportive. It involves careful wound care, close monitoring of fluid and electrolyte balance, ophthalmological review and eye care, infection control, pain control and nutritional support. Any inciting drug is discontinued, and causative organism is treated – the most common being Mycoplasma pneumoniae. The use of IVIG for SJS has been controversial. IVIG has been used with reported beneficial effects in various case reports in treating SJS. In 1998, Viard et al. reported that IVIG inhibited Fas-mediated keratinocyte apoptosis in vitro – a key mechanism in the pathogenesis of SJS. This added in vitro evidence to clinical experience.