Stevens–Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature

Authors

  • M. Ziemer,

    1. Department of Dermatology, Venereology and Allergology, University Hospital Leipzig, Philipp-Rosenthal-Str. 23–25, 04103 Leipzig, Germany
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  • S.H. Kardaun,

    1. Department of Dermatology, Reference Centre for Cutaneous Adverse Reactions, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9713 GZ Groningen, the Netherlands
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  • Y. Liss,

    1. Dokumentationszentrum schwerer Hautreaktionen (dZh), Department of Dermatology, University Freiburg – Medical Center, Freiburg, Germany
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  • M. Mockenhaupt

    1. Dokumentationszentrum schwerer Hautreaktionen (dZh), Department of Dermatology, University Freiburg – Medical Center, Freiburg, Germany
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  • Funding sources
    M.Z. and S.H.K., none; Y.L. and M.M., the German Registry was funded by the following institutions: Ministry for Research and Technology (Bundesministerium für Forschung und Technologie; BMFT), grant no. 0701 564/4 (1990–1995); Federal Institute for Drugs and Medical Devices (Bundesinstitut für Arzneimittel und Medizinprodukte; BfArM), grant no. GS 1-68502-200 (1995–1998) and grant no. Z121.01-68502-208 (2001–2002); European Commission, RegiSCAR-project in the 5th Framework, grant no. QLRT-2002-01738 (2002–2005); and a consortium of pharmaceutical companies (Ciba Geigy/Novartis; Hoffmann-La-Roche; Hoechst AG/Hoechst Marion Roussel/Aventis; Sanofi Winthrop/Sanofi Synthelabo; Bayer AG/Bayer Vital; Glaxo Wellcome/GlaxoSmithKline; Schering AG; Goedecke Parke Davis; Pfizer GmbH; Cilag GmbH; Dr Willmar Schwabe; Boehringer Ingelheim; MSD Sharp & Dohme; Merck U.S.A.; Cephalon Inc.; Tibotec B.V.). M.M. received the Else Kröner Memorial Stipendium for support of clinical research through Else Kröner-Fresenius-Foundation (2008–2009).

  • Conflicts of interest
    None declared.

  • M.Z. and S.H.K. contributed equally to this study.

Sylvia H. Kardaun or Mirjana Ziemer.
E-mail: s.h.kardaun@derm.umcg.nl; mirjana.ziemer@medizin.uni-leipzig.de

Summary

Background  Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions with high morbidity and mortality. Some expressions of lupus erythematosus (LE) may cause enormous difficulties in differentiating them from SJS and TEN by showing large areas of sheet-like epidermal necrosis.

Objective  To evaluate clinically and histopathologically probable or definite cases of SJS/TEN with a history of systemic or other LE [(S)LE].

Methods  This was a retrospective analysis of validated cases of SJS/TEN with a history of (S)LE, based on a large population-based national registry.

Results  Among 1366 patients with SJS/TEN, 17 with a sufficiently documented history of (S)LE and representative histological material could be identified, suggesting a considerable over-representation of LE in patients with SJS/TEN. Eight of these showed clinically and/or histopathologically some LE-characteristic features interfering with the diagnosis of SJS/TEN. Differentiation could be elaborated on clinical and histopathological grounds: four patients were classified as SJS/TEN with a preceding (S)LE exacerbation and/or LE-typical histopathological features, and four as ‘TEN-like’ (S)LE.

Conclusion  Most patients with SJS/TEN and a history of (S)LE demonstrate clinical and histopathological properties allowing clear differentiation. However, occasionally acute cutaneous manifestations of (S)LE and SJS/TEN can be phenotypically similar, caused by extensive epidermal necrosis. Although no feature by itself is conclusive, a combination of recent (S)LE exacerbation, evident photodistribution, annular lesions and absent or only mild focal erosive mucosal involvement may favour LE over SJS/TEN clinically. Histopathologically, in particular, junctional vacuolar alteration, and the presence of solitary necrotic keratinocytes at lower epidermal levels, combined with moderate to dense periadnexal and perivascular lymphocytic infiltrates with a variable presence of melanophages, and mucin point to a LE-related origin.

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