Antibodies to Ro/SSA are found in patients with subacute cutaneous lupus erythematosus (SCLE), complement deficiency lupus erythematosus, systemic lupus erythematosus (SLE), neonatal lupus erythematosus, and Sjögren syndrome (SS). Most studies describing the cutaneous pathology associated with anti-Ro antibodies have been of patients with SCLE. Over a 42-month period, we encountered skin biopsy specimens from 23 anti-Ro-positive patients who did not have SCLE: 15 had SLE variably manifesting as SCLE-like rashes; malar erythema; a dermatomyositis-like rash; vascular disease involving cutaneous, cardiac, peripheral, and central nervous systems; restrictive pulmonary disease; periorbital edema; and myositis. Two patients had primary Sjögren syndrome, one had primary antiphospholipid antibody syndrome, and two had rheumatoid arthritis; all five had clinical evidence of cutaneous vasculopathy encompassing livedo, perniosis, and palpable purpura. Three additional patients presented with folliculocentric purpura without other stigmata to permit classification as a specific connective tissue disease. In the SLE patients, biopsy specimens of photodistributed eruptions showed an interface dermatitis accompanied by superficial vascular plexus density reduction. Vasculopathic reactions in patients with and without SLE comprised neutrophilic, lymphocytic, or pauciinflammatory thrombogenic subtypes. Although at times a marker of SCLE, the identification of anti-Ro antibodies may isolate a subset of patients at higher risk of multiorgan vasculopathy, myositis, and progressive pulmonary disease. We postulate that many of the features seen in these patients reflect the sequelae of antibody mediated endothelial cell injury.