Introduction
Rowell’s syndrome (RS) is an uncommon entity in which patients with systemic lupus erythematosus (SLE) rarely develop characteristic lesions similar to those of erythema multiforme (EM)-like skin lesions, in the presence of specific serological abnormalities (1–4). Firstly, the association between lupus and EM was described by Scholtz in 1922. In 1963, Professor Neville Rowell and his colleagues reported four female patients with discoid lupus erythematosus (DLE) and EM-like skin lesions (among 120 patients with DLE) (2,3). SLE is a chronic autoimmune‐mediated inflammatory disorder with multisystem and multiorgan manifestations, while EM is an acute, immune‐mediated condition linked to infection, medications, and autoimmune disorders without special autoantibodies, as seen in autoimmune disorders. EM is distinguished by evident target lesions on the skin, accompanied by erosion, blisters, or bullae of mucosal areas (such as the mouth, genitals, and eyes) (3,5).
To reach a diagnosis, meeting all major criteria along with one minor criterion is necessary. Major criteria include the presence of systemic or cutaneous lupus erythematosus (CLE), erythema multiform-like lesions, and antinuclear antibodies (ANA). Minor criteria include the presence of chilblains, anti-Ro, or anti-La antibodies or rheumatoid factor (RF) (4). While the precise etiopathogenesis of RS remains unclear, it is believed to be triggered by factors such as drugs, infections, ultraviolet exposure, cigarette smoking, and psychological stress (1,3). Eventually, RS is considered a rare but distinct entity in rheumatology, and systemic lupus erythematosus (SLE) presenting initially as EM-like lesions is quite uncommon (2).