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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.
Br J Dermatol. 2012 Mar; 166(3):575-600.BJ

Abstract

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.

Authors+Show Affiliations

Department of Dermatology, Venereology and Allergology, University Hospital Leipzig, Philipp-Rosenthal-Str. 23-25, 04103 Leipzig, Germany.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

22014091

Citation

Ziemer, M, et al. "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." The British Journal of Dermatology, vol. 166, no. 3, 2012, pp. 575-600.
Ziemer M, Kardaun SH, Liss Y, et al. 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. Br J Dermatol. 2012;166(3):575-600.
Ziemer, M., Kardaun, S. H., Liss, Y., & Mockenhaupt, M. (2012). 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. The British Journal of Dermatology, 166(3), 575-600. https://doi.org/10.1111/j.1365-2133.2011.10705.x
Ziemer M, et al. 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. Br J Dermatol. 2012;166(3):575-600. PubMed PMID: 22014091.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Ziemer,M, AU - Kardaun,S H, AU - Liss,Y, AU - Mockenhaupt,M, PY - 2011/10/22/entrez PY - 2011/10/22/pubmed PY - 2012/4/28/medline SP - 575 EP - 600 JF - The British journal of dermatology JO - Br J Dermatol VL - 166 IS - 3 N2 - 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. SN - 1365-2133 UR - https://www.unboundmedicine.com/medline/citation/22014091/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_ L2 - https://doi.org/10.1111/j.1365-2133.2011.10705.x DB - PRIME DP - Unbound Medicine ER -