Lupus is the Latin word for wolf and was first used in the 10th century to describe erosive skin lesions that looked as though a wolf had eaten away the flesh. The term lupus erythemateux was first used in 1851 by Cazenave, a Frenchman who described an illness in a patient with manifestations occurring on the skin. It is not surprising the systemic lupus erythematosus (SLE) was first recognized as a skin disorder, because cutaneous manifestations constitute one of the most common clinical features of the disease. Further descriptions by Kaposi in 1872 and Osler in 1895 led to the concept of a multi-system disease, as it became recognized that patients developed complications in other organ systems.(1, 2)
Abnormal immunologic function and formation of antibodies against "self" antigens underlie the pathogenesis of SLE. The autoantibodies in this disease became apparent with the development of the lupus erythematosus cell test in 1948 and the fluorescent antinuclear antibody test in 1957.(1)
In 1971, the American Rheumatism Association developed and in 1982 revised criteria used to distinguish SLE from other collagen vascular diseases.(3) To classify as having SLE, four or more of the eleven criteria must be present. The criteria include:
malar rash
discoid rash
photosensitivity
oral ulcers
arthritis
serositis (pleuritis or pericarditis)
renal disorder
neurologic disorder (seizures or psychosis in the absence of offending drugs or known metabolic derangements)
hematologic disorders (hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia)
immunologic disorder (positive LE test or anti-DNA, or anti-Sm, or false positive syphilis test)
antinuclear antibody.
The etiology of abnormal antibody production and development of SLE is still unknown. Genetic, environmental, and hormonal factors may all have a role in loss of self-tolerance and expression of disease. A popular theory is that autoimmune diseases, such as SLE, develop in genetically susceptible individuals after exposure to a "triggering" agent, possibly something in the environment.(4)
