Original article
Erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis associated with lupus erythematosus

https://doi.org/10.1016/j.jaad.2011.10.012Get rights and content

Background

The occurrence of erythema multiforme (EM)-like lesions in association with lupus erythematosus (LE) is often referred to as “Rowell syndrome” (RS). However, the existence of RS, or at least its nosographic independence from LE, is questioned. The association of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) with LE is also controversial.

Objective

We sought to define the features of EM and SJS/TEN in the setting of LE.

Methods

The worldwide literature on the topic was systematically collected and reviewed.

Results

A total of 132 citations were found, from which 95 cases of EM-like lesions and 47 of SJS/TEN associated with LE were retrieved. Our analysis identified a subgroup defined as “subacute cutaneous LE (CLE)/acute CLE with EM-like lesions” and highlighted that this and subacute CLE/acute CLE with TEN-like lesions are variants of already known CLE subpatterns. On the other hand, RS can be considered an independent chronic CLE subtype characterized by the distinctive co-occurrence of chronic CLE and EM-like lesions and frequent, albeit mild, systemic involvement.

Limitations

The study was based on retrospective data and the number of reported cases identified was relatively small.

Conclusion

RS might be included as a chronic CLE subtype within the spectrum of LE-specific skin disease.

Section snippets

Methods

We searched the available literature by entering the terms “Rowell’s syndrome,” “Rowell syndrome,” “EM AND lupus,” “Stevens-Johnson AND lupus,” “TEN AND lupus,” and “Lyell AND lupus” into the biomedical search engines EMBASE, Google scholar beta, ISI Web of knowledge, PubMed, and SCOPUS. African Index Medicus; Biosis Previews and Biological Abstracts; Conference Papers Index; Index Medicus for South East Asia Region; KoreaMed; Latin American, Caribbean Health Sciences Literature database;

Results

A total of 132 citations were found as of April 30, 2010, from which we retrieved 101 full-text articles, 23 abstracts, and 8 titles (references available upon request to the corresponding author). Three reports were excluded because of redundancy; 24 further cases were excluded because the description and/or photographs of the skin lesions were not consistent with EM or SJS/TEN.

Discussion

The appearance of skin lesions mimicking EM and SJS/TEN in patients affected by LE seems to be rare and documented only by isolated case reports and small case series.

Conclusion

RS is a rare and often misdiagnosed condition. On the basis of this review, we conclude that RS might be included as an autonomous CCLE type within the spectrum of LE-specific skin disease.

We hope that the proposal of new diagnostic criteria for RS and the framing of clinical variants resembling RS will fuel new interest and debate among the scientific community and increase the recognition and research on the proteiform manifestations of CLE.

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Dr Torchia was funded in part by a fellowship awarded by the Società Italiana di Dermatologia e Malattie Sessualmente Trasmesse.

Disclosure: Dr Kerdel is on the advisory board and served as a speaker for Genetech, Astellas, Abbott, Centocor, and Amgen; he has also received research grants from the latter 3 companies. Drs Torchia and Romanelli have no conflicts of interest to declare.

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