Abstract

Toxic epidermal necrolysis (TEN), an uncommon but potentially life-threatening skin reaction, is frequently induced by drugs. The mucocutaneous reaction is characterised by bullous detachment of the epidermis and mucous membranes. We present a 9-month-old male with methylmalonic acidaemia, generalised hypotonia, and global developmental delay. He presented with a 3-day history of fever, cough, shortness of breath, and vomiting. Eruption appeared after 5 days of vancomycin treatment. The eruption involved almost 60% of the total body surface area and both eyes. He was successfully treated with intravenous immunoglobulin (IVIG), antibiotics, and appropriate wound management and made a full recovery with negligible sequelae despite the severity of his disease. Important components of successful treatment include early recognition, intensive care, prompt withdrawal of the causative agent, early administration of IVIG, appropriate fluid resuscitation, and control of infection. IVIG might be beneficial in the treatment of TEN; however, controlled studies are needed to evaluate IVIG compared to other modalities.

Highlights

  • Toxic epidermal necrolysis (TEN) is a severe drug-induced life-threatening disease characterised by fulminant, widespread blisters which become responsible for epidermal sloughing

  • Oral mucosal hyperaemia and erosions appeared. These lesions were diagnosed as vancomycin-induced toxic epidermal necrolysis

  • The diagnosis of TEN in our patient was based on its classic presentation: 1) prodromal phase with fever and upper respiratory tract symptoms; 2) ophthalmological and oral mucous membrane involvement; 3) typical skin lesions involving more than 10% of the total surface area of the skin with positive Nikolsky’s sign;2 4) exclusion of other differential diagnoses, including burns, pemphigus/ pemphigoid disease, erythema multiforme;3 5) superficial skin swabs and all septic work-ups were negative; the patient’s improvement after the withdrawal of vancomycin made toxic shock syndrome unlikely

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Summary

Introduction

Toxic epidermal necrolysis (TEN) is a severe drug-induced life-threatening disease characterised by fulminant, widespread blisters which become responsible for epidermal sloughing It is associated with high mortality and the majority of patients die from complications related to infection.[1] Supportive therapies and antiseptics are of paramount importance in the management of patients with. Granulocyte colony-stimulating factor (G-CSF) was given for neutropaenia (10 mcg/kg once daily for two days) and stopped when the neutropaenia resolved His ammonia level increased to 495 ummol/L, so sodium benzoate and sodium phenyl butyrate were given again as bolus, and continued as maintenance. These lesions were diagnosed as vancomycin-induced toxic epidermal necrolysis. An ophthalmologic examination showed the ocular mucosa was affected by erosions, corneal abrasions, and abnormally directed lashes

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