Lupus erythematosus (LE) is a clinical syndrome that has multiple, but largely unknown causes. It exhibits an extremely broad spectrum of symptoms, and it can range in severity from being potentially fatal within a few weeks to eliciting minor indolent symptoms which, prior to immunologic testing, are virtually undiagnosable. When limited to the skin, it is called discoid lupus erythematosus (DLE); when the viscera are symptomatically affected, it is termed systemic lupus erythematosus (SLE). The inciting causes activate immunologic mechanisms that mediate the pathological, predominantly inflammatory, tissue responses.

History

Medical use of the term lupus has been traced to the fifteenth century, when it designated a variety of cancer. The term was reintroduced by London physician Robert Willan in 1808 to designate cutaneous tuberculosis, particularly when it affected the face. Cutaneous tuberculosis eventually received the synonym lupus vulgaris. In 1851 P. L. Alphée Cazenave of Paris used the term lupus erythemateaux to describe the condition that came to be called discoid lupus erythematosus (DLE) by Vienna’s Moriz Kaposi in 1872 (Jarcho 1957). During 1866–70, Kaposi diagnosed this disease in 22 patients and concluded that it was more common and more severe in women. All 3 deaths occurred among his 15 female patients. Although one of these had pulmonary tuberculosis, and cutaneous tuberculosis was common, Kaposi believed that DLE is not related to tuberculosis. Such a causal relationship, however, came to be advocated, particularly by French dermatologists, and remained under discussion until the 1930s. During the 5 years in which Kaposi saw 22 cases of DLE, 279 cases of lupus vulgaris were seen in the same department (Kaposi 1872).