Rowell syndrome

Rowell syndrome is a rare and controversial clinical entity characterized by the coexistence of systemic lupus erythematosus (SLE) or discoid lupus erythematosus (DLE) with erythema multiforme-like lesions, along with a distinctive immunological profile. The syndrome was first described by Rowell, Beck, and Anderson in 1963. It primarily affects the skin and immune system. Patients typically present with the characteristic features of lupus erythematosus — such as photosensitivity, malar rash, joint pain, and systemic inflammation — alongside targetoid or erythema multiforme-like skin lesions that may be widespread. The immunological hallmark includes a speckled pattern of antinuclear antibodies (ANA), the presence of anti-Ro (SS-A) antibodies, and a positive rheumatoid factor, though not all reported cases meet these original criteria. The existence of Rowell syndrome as a distinct entity remains debated in the dermatological and rheumatological literature, with some experts arguing that the erythema multiforme-like lesions simply represent a cutaneous manifestation of lupus erythematosus rather than a separate syndrome. The condition predominantly affects women, consistent with the female predominance seen in lupus. Treatment is generally directed at the underlying lupus erythematosus and may include systemic corticosteroids, antimalarials such as hydroxychloroquine, immunosuppressive agents, and supportive skin care. The erythema multiforme-like lesions often respond to treatment of the underlying lupus. Prognosis depends largely on the severity of the associated lupus and the degree of systemic involvement. Due to its rarity and the ongoing debate regarding its classification, there are no standardized treatment guidelines specific to Rowell syndrome.

Common questions about Rowell syndrome