Layell syndrome: difficulties of diagnostics

  • Zlata V. Davydova St. Petersburg State Pediatric Medical University
  • Olga V. Sokolova St. Petersburg State Pediatric Medical University
  • Alexey V. Sobolev North-Western State Medical University named after I.I. Mechnikov

Abstract

In this article, we want to present a complex case from our practice when a diagnostically uncertain patient has been treated for a long time from a respiratory disease, then she developed toxic epidermal necrolysis (Lyell’s syndrome), which had an atypical beginning, whose complications were the immediate cause of the patient’s death. Toxic epidermal necrolysis is an acute, severe, life-threatening disease characterized by a widespread bullous lesion of the skin and mucous membranes, its peculiarity is the appearance of thin-walled, easily tearing bubbles, in the place of which extensive necrotic fields resemble severe burns of 2-3 degrees, in combination with severe intoxication and impaired functions of all organs. The development of the disease is most often associated with taking medications, possibly with epidermotropism. The information reflecting the modern view on the etiology, pathogenesis, diagnosis of Lyell’s syndrome, as well as the morphological picture of the disease are presented. On a concrete example, objective reasons are analyzed from practice, which led to a belated diagnosis of epidermal necrolysis, formation of sepsis and multiple organ failure. The data that are relevant for the practice of forensic medical examinations are presented, which allow us to formulate objective and scientifically substantiated conclusions about the existence of cause-effect relationships between the defects in the provision of medical care and the onset of death of the patient.