A rare case of a patient with systemic lupus erythematosus who presented with Rowell syndrome responding to treatment with hydroxychloroquine and prednisone with 1 year of relapse-free survival on belimumab.

Rowell syndrome, first described in 1963 by Rowell et al., is an infrequently reported and unique syndrome occurring in patients with systemic lupus erythematosus (SLE). This syndrome characteristically presents with erythema-multiforme-like lesions as well as other specific immunologic and histopathological manifestations. Since Rowell's original description, diagnostic criteria have been proposed and modified to better describe the syndrome. We describe a 32-year-old African American female patient with a previous history of SLE who presented with dermatologic, immunologic, and histopathological manifestations that fit the modified diagnostic criteria for Rowell syndrome.