Ten out of Ten: Toxic Epidermal Necrolysis

Abstract

Introduction

Toxic epidermal necrolysis (TEN) is a severe, potentially fatal mucocutaneous reaction involving 30% or more body surface area (BSA) with a high mortality rate. Stevens-Johnson syndrome (SJS) is the less severe form, involving less than 10% BSA. SJS/TEN overlaps involve 10-30% BSA.

Case report

A 59 years old gentleman presented to the emergency department complaining of fever, generalized rashes, painful oral erosions, and productive cough. His co-morbid include diabetes mellitus and hypertension. His medications include insulin, bisoprolol, cardiprin, and phenytoin which was commenced 3 months ago after being admitted for meningoencephalitis. Examination shows interspersed areas of desquamation, blisters, and atypical target lesions located primarily in the trunk, upper limbs, and genital area. There are also bilateral purulent conjunctivitis and erythematous oral mucosa erosions. Chest X-ray shows right lower zone consolidation. The patient was admitted and started on antibiotics, hydrocortisone, and ciclosporin. The patient shows an excellent response to treatment, and he was allowed home after 2 weeks of admission with topical emollients and steroids.

Discussion

Multiple etiologies are associated with TEN, with drugs such as allopurinol, antiepileptics, and sulfa-group antibiotics topping the list. The earliest cutaneous lesions are atypical targets and purpuric lesions, which progress to vesicles and bullae with diffuse skin desquamation, often with mucosa and ocular involvement. Hypovolemic shock and electrolyte imbalance may occur in severe cases with extensive skin detachments leading to massive fluid loss. Moreover, complications such as secondary infections, pneumonia, interstitial pneumonitis, acute respiratory distress syndrome, gastrointestinal ulceration, and perforations are frequent. SCORTEN score is used in determining prognosis and severity, and transferring to an intensive or burn unit is recommended if score ≥2. Identification and withdrawal of causative agents are the crucial first steps in management. This is followed by stringent supportive care, similar to managing burn patients: Wound care, fluid and electrolyte management, nutritional support, ocular care, temperature management, pain control, and monitoring for complications, requiring a multidisciplinary team in treatment. The role of systemic corticosteroids in TEN/SJS remains uncertain; however, multiple studies show cyclosporin may be beneficial. The mortality rate for TEN is more than 30%.