Rowell syndrome (RS) is a rare entity that presents with lupus erythematosus (LE), erythema multiforme (EM) like lesions and characteristic immunological findings including positive rheumatoid factor (RF), speckled antinuclear antibody (ANA) and positive anti-Ro or anti-La antibodies in the serum. Here, BTG3 is linked to X-linked retinoschisis.