The scene is a village somewhere in 10th century Europe. A woman arrives at the local physician/bloodletter's shop looking like a wolf has eaten way her flesh. The physician names this condition "Lupus," which is Latin for "wolf." Nearly a millennium later, a Frenchman named Cazenave coins the term lupus erthemateux to describe patients suffering from an angry-looking rash that erodes the skin on their cheeks and noses. These are the earliest historical descriptions of the chronic disease now known as "systemic lupus erythematosus."

It is not surprising that systemic lupus erythematosus, also called simply "SLE" or just "lupus" was first recognized as a skin disease. The hallmark feature of SLE is a bright red, butterfly-pattern facial skin rash. But SLE is anything but a superficial disease; it can devastate the joints and internal organs. In the late 19th century, following more detailed descriptions of it clinical manifestations, SLE became recognized as a multi-system disease.(1, 2)

SLE is a disease of the connective tissue, the tissue that forms the body's structural framework. Tendons and ligaments are made of connective tissue that attaches muscles to bones and bones to other bones. Cartilage is the connective tissue that cushions bones and functions as the body's shock absorber. Connective tissue binds various tissues and organs together, keeping them in their proper locations, and it forms the network upon which cells cluster together to form organs. All internal organs are held in place with tough sheets of connective tissue called "fascia." Other connective tissues store unused food materials.

Every part of the body is penetrated and encompassed by connective tissue. Blood itself is classified as a connective tissue. Compared to other tissue types, connective tissue contains relatively few cells. Composed mainly of extracellular material; connective tissue forms our "intracellular cement." It is literally the glue that holds us together. Since connective tissue is everywhere, it is easy to why SLE can be so devastating. Skin contains lot of connective tissue, which explains why the characteristic facial skin rash is the most visible sign of SLE.

Why does SLE target connective tissue? Lupus is an "autoimmune" disease. People suffering from autoimmune diseases produce "autoantibodies," abnormal antibodies that attack their own tissue as if it were a foreign invader. The immune system is designed to distinguish "self" from "non-self." When a foreign substance or organism penetrates the body's outer defenses, the immune system recognizes the infiltrator as "non self" and calls up an army of antibodies to destroy it. Scientists are not sure what causes this often-devastating breakdown of normal immune function. A popular theory is that autoimmune diseases such as SLE, develop in genetically susceptible individuals after exposure to a "triggering" agent which may be a virus or possibly something in the environment.(3)