Toxic epidermal necrolysis: a paradigm of critical illness

Abstract

Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.

Clinical and pathogenic aspects of the severe cutaneous adverse reaction epidermal necrolysis (EN)

“…EN starts with a prodromal phase of typically 48–72 h, presenting with specific systemic symptoms such as fever and cough 5 . Subsequently, erythematous macules and atypical target lesions develop and spread rapidly within a few days.…”

“…Most frequently, the oropharynx, eyes, genitals and anus are affected, but also the nose, oesophagus, trachea and bronchi can be involved, which can lead to respiratory problems. The severity of mucosal involvement is not correlated with the area of skin affected 5 . Re‐epithelialization usually takes 1–3 weeks 5 .…”

“…The severity of mucosal involvement is not correlated with the area of skin affected 5 . Re‐epithelialization usually takes 1–3 weeks 5 . However, the acute phase is often followed by sequelae as described later on 11 …”

“…The adverse reaction is rare with an incidence estimated at 2–7 cases per million persons per year. Of these, 0.4–1.9 cases per million persons per year are diagnosed with TEN 2,5 . There is no consensus about adequate, specific treatment strategies, and mortality rates are high (23% at 6 weeks, 34% at 1 year) 6 .…”

Bullous epidermolysis after allogeneic stem cells transplantation – non-typical clinical presentation of severe graft versus host disease

“…Разрешение очагов на фоне терапии с использованием технологии экстракорпорального фотофереза характерной для обоих патологических состояний. В частности, апоптоз кератиноцитов, некроз клеток и вакуольная дистрофия эпидермоцитов -изменения, характерные при РТПХ, -характерны и для ТЭН [5].…”