History of systemic lupus erythematosus with an emphasis on certain recent major issues

The age of dermatology

The Latin word lupus, meaning wolf, was in the medical literature prior to the 1200s to describe skin lesions that devour flesh. Because of the inability to distinguish herpes, leprosy, and cancer as the cause of the skin lesions devouring the flesh, the term lupus was then applied to skin lesions nonspecifically. Rogerius (1230) has been credited to dissociate the skin lesions of lupus from that of herpes. The term was applied mostly to lesions on the lower extremities and on the face. Robert Willan, a British physician published his Manual on Skin Diseases with color illustrations based on his astute clinical observations. He was able to identify distinct clinical presentations of lupus skin diseases. Most of the early description of lupus skin diseases were lupus vulgaris.

Lupus erythematosus was first described as Erythema centrifugum by Laurent T Biett (1781–1840), a prominent French dermatologist. This was reported in 1833 by his pupil Cazenave (1802–77) “….a remarkable variety of this disease under the name of Erythema centrifugum. It is often of very rare occurrence, and appears most frequently in young people, especially in females, whose health is otherwise excellent….” Cazenave in his article published in 1851 renamed Erythema centrifugum as lupus erthemteux (LE). Prior to this, Ferdinand von Hebra (1816–80), a Viennese physician used the term of butterfly rash in 1848 to describe one of the two types of lupus erythematosus.

Regarding the etiology of lupus skin disease, it was remarkable that Jonathan Hutchinson (1828–1913), a British surgeon with multiple talents described photosensitivity in 1879 may be a cause of lupus by stating “ … Erythematous lupus is very rarely seen in those parts of the surface which is constantly protected by clothes. It is always made worse by exposure to the wind and cold… Sunburn of the nose is a common exciting cause.” In 1880, he described the lesion of lupus marginatus that resembles subacute lupus erythematosus described by Sontheimer et al. in 1979.

During the 19th century there was much debate about the pathogenesis of lupus skin diseases. It was assumed to be due to infections because most lupus patients came from poor neighborhoods with crowded living arrangements. With the discovery of tubercle bacillus by Robert Koch in 1882, several reports of growing tubercle bacillus from lupus lesions were published leading to popularity of the theory. Despite the finding by Walther Pick in 1901 that only 15 out of 29 patients with LE were positive for a tuberculin skin test with positive TB history, the tuberculous etiology of LE remained a favorite theory of LE pathogenesis. Most investigators argued for or against this theory based on clinical statistics, a practice still used by contemporary investigators in lupus research. The tuberculous etiology of LE led to the use of gold and other heavy metals such as bismuth as therapeutic agents for decades.