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).