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

Abstract

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. We sought to define the features of EM and SJS/TEN in the setting of LE. The worldwide literature on the topic was systematically collected and reviewed. 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. The study was based on retrospective data and the number of reported cases identified was relatively small. RS might be included as a chronic CLE subtype within the spectrum of LE-specific skin disease.