Rowell syndrome, first described by Rowell et al. in 1963, is a rare entity characterized by the combination of lupus erythematosus and erythema multiforme (EM)-like lesions with a characteristic immunological pattern that includes a positive rheumatoid factor, speckled antinuclear antibodies (ANA), positive anti-Ro/SSA antibodies, and, positive anti-La/SSB antibodies [1]. The gene discussed is CALR; the disease is lupus erythematosus.