Stevens-Johnson syndrome and toxic epidermal necrolysis: A systematic review of PubMed/MEDLINE case reports from 1980 to 2020

Liqin Wang; Sheril Varghese; Fatima Bassir; Ying-Chin Lo; Carlos A Ortega; Sonam Shah; Kimberly G Blumenthal; Elizabeth J Phillips; Li Zhou

doi:10.3389/fmed.2022.949520

Stevens-Johnson syndrome and toxic epidermal necrolysis: A systematic review of PubMed/MEDLINE case reports from 1980 to 2020

Front Med (Lausanne). 2022 Aug 24:9:949520. doi: 10.3389/fmed.2022.949520. eCollection 2022.

Authors

Liqin Wang¹, Sheril Varghese¹, Fatima Bassir¹, Ying-Chin Lo¹, Carlos A Ortega², Sonam Shah^{1

3}, Kimberly G Blumenthal⁴, Elizabeth J Phillips⁵, Li Zhou¹

Affiliations

¹ Division of General Internal Medicine and Primary Care, Department of Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United States.
² School of Medicine, Vanderbilt University, Nashville, TN, United States.
³ Harvard Medical School, Dana-Farber Cancer Institute, Boston, MA, United States.
⁴ Division of Rheumatology, Allergy, and Immunology, Harvard Medical School, Massachusetts General Hospital, Boston, MA, United States.
⁵ Division of Infectious Disease, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States.

Abstract

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening immunologic reactions. Prior studies using electronic health records, registries or reporting databases are often limited in sample size or lack clinical details. We reviewed diverse detailed case reports published over four decades.

Methods: Stevens-Johnson syndrome and toxic epidermal necrolysis-related case reports were identified from the MEDLINE database between 1980 and 2020. Each report was classified by severity (i.e., SJS, TEN, or SJS-TEN overlap) after being considered a "probable" or "definite" SJS/TEN case. The demographics, preconditions, culprit agents, clinical course, and mortality of the cases were analyzed across the disease severity.

Results: Among 1,059 "probable" or "definite" cases, there were 381 (36.0%) SJS, 602 (56.8%) TEN, and 76 (7.2%) SJS-TEN overlap cases, with a mortality rate of 6.3%, 24.4%, and 21.1%, respectively. Over one-third of cases had immunocompromised conditions preceding onset, including cancer (n = 194,18.3%), autoimmune diseases (n = 97, 9.2%), and human immunodeficiency virus (HIV) (n = 52, 4.9%). During the acute phase of the reaction, 843 (79.5%) cases reported mucous membrane involvement and 210 (19.8%) involved visceral organs. Most cases were drug-induced (n = 957, 90.3%). A total of 379 drug culprits were reported; the most frequently reported drug were antibiotics (n = 285, 26.9%), followed by anticonvulsants (n = 196, 18.5%), analgesics/anesthetics (n = 126, 11.9%), and antineoplastics (n = 120, 11.3%). 127 (12.0%) cases reported non-drug culprits, including infections (n = 68, 6.4%), of which 44 were associated with a mycoplasma pneumoniae infection and radiotherapy (n = 27, 2.5%).

Conclusion: An expansive list of potential causative agents were identified from a large set of literature-reported SJS/TEN cases, which warrant future investigation to understand risk factors and clinical manifestations of SJS/TEN in different populations.

Keywords: Stevens-Johnson syndrome; case report; drug-related side effects and adverse reactions; review literature; toxic epidermal necrolysis.

Publication types

Systematic Review