Layell syndrome: difficulties of diagnostics

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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.

About the authors

Zlata V. Davydova

St. Petersburg State Pediatric Medical University

Author for correspondence.
Email: zlata.davydova@rambler.ru

MD, PhD, Associate Professor, Department of Pathological Anatomy at the Rate of Forensic Medicine

Russian Federation, Saint Petersburg

Olga V. Sokolova

St. Petersburg State Pediatric Medical University

Email: last_hope@inbox.ru

MD, PhD, Associate Professor, Department of Pathological Anatomy at the Rate of Forensic Medicine

Russian Federation, Saint Petersburg

Alexey V. Sobolev

North-Western State Medical University named after I.I. Mechnikov

Email: microbiota@spbmapo.ru

PhD, Dr Med Sci, Professor, Department of Clinical Mycology, Allergology, Immunology

Russian Federation, Saint Petersburg

References

  1. Адаскевич В.П. Неотложные состояния в дерматологии. - СПб.: Ольга, 2000. [Adaskevich VP. Neotlozhnye sostoyaniya v dermatologii. Saint Petersburg: Ol’ga; 2000. (In Russ.)]
  2. Федосеева Г.Б. Частная аллергология. - СПб.: Норммед-Издат, 2001. [Fedoseeva GB. Chastnaya allergologiya. Saint Petersburg: Normmed-Izdat; 2001. (In Russ.)]
  3. Хаитов Р.М., Ильина Н.И. Иммунопатология и аллергология: Алгоритмы диагностики и лечения. - М.: ГЭОТАР-Медиа, 2003. [Khaitov RM, Il’ina NI. Immunopatologiya i allergologiya: Algoritmy diagnostiki i lecheniya. Moscow: GEOTAR Media; 2003. (In Russ.)]
  4. Chave TA, Mortimer NJ, Sladden MJ, et al. Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol. 2005;153(2):241-53. doi: 10.1111/j.1365-2133.2005.06721.x.
  5. Trent J, Halem M, French LE, Kerdel F. Toxic epidermal necrolysis and intravenous immunoglobulin: a review. Semin Cutan Med Surg. 2006;25(2):91-93. doi: 10.1016/j.sder.2006.04.004.
  6. Creamer D, Walsh SA, Dziewulski P, et al. UK guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016. J Plast Reconstr Aesthet Surg. 2016;69(6):e119-e153. doi: 10.1016/j.bjps.2016.01.034.

Copyright (c) 2018 Davydova Z.V., Sokolova O.V., Sobolev A.V.

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This work is licensed under a Creative Commons Attribution 4.0 International License.
 


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