Connective Tissue Disorders

Connective tissue disorders (CTDs) are a heterogeneous group of conditions that share autoimmune pathogenesis associated with an abnormal function or structure of the elements of the connective tissue. Major CTDs include lupus erythematosus, scleroderma, dermatomyositis, and Sjogren syndrome. Dapsone is an alternative option as well as thalidomide in some European countries. The refractory disease has been treated with retinoids, methotrexate, mofetil or mycophenolate acid, cyclosporine, and azathioprine with variable results. Systemic lupus erythematosus can affect virtually any organ of the body. The generation of numerous types of autoantibodies causes several combinations of clinical signs and laboratory abnormalities. Active extensive morphea or generalized morphea are treated with methotrexate, combination therapy of systemic steroids and methotrexate, or with phototherapy with ultraviolet A1 or narrowband UVB. Capillary abnormalities of the proximal nail fold of the hands can be visualized with dermatoscopy or capilleroscopy. Patients with SjS present with variable and insidious symptoms of difficult classification.