A Historical Look at the Characterization of Lupus as a Systemic Disease

Dr. Kaposi was both Dr. Hebra’s student and his son-in-law. Called by some the “father of German dermatology,” Dr. Kaposi is known for a number of achievements, including his work on Kaposi sarcoma and xeroderma pigmentosum.¹⁰ But among Dr. Kaposi’s most important contributions is his characterization of lupus.

In 1869, Dr. Kaposi published his first paper on the topic, in Archiv für Dermatologie und Syphilis. He briefly recounted the history of the term, speculated on etiology and discussed symptomatic treatment.² In his much more extensive 42-page paper published in 1872, Dr. Kaposi included 11 detailed case studies, with extensive characterizations of symptoms, disease course and microscopy information consolidated from additional patients.¹

Dr. Kaposi could present no explanation of the disease etiology, but considered the disease inflammatory in nature, based on its histopathology. He also noted that “most of the diseased are found in persons of middle age,” and “the female sex will be affected by the disease much more often than the male.” This was the first description of the systemic nature of the disease and its severe constitutional and visceral manifestations.²

For Dr. Kaposi to group together patients with such varied manifestations as arthritis, pleurisy & coma, all of whom also had cutaneous lupus, was bold & prescient.

Commentary

Dr. Salmon

Jane Salmon, MD, is a professor of medicine at the Hospital for Special Surgery and the director of the Lupus and APS Center of Excellence in New York City. “Earlier physicians had done wonderful work and wrote eloquent descriptions distinguishing lupus erythematosus from tuberculosis and other entities,” Dr. Salmon notes. “But it was Dr. Kaposi who identified lupus as a disease of young women that was systemic and life threatening. For this reason, I think this paper is really interesting.”

In his 1872 paper, which summarized and built on previous descriptions, Dr. Kaposi acknowledged others’ contributions to characterizing the disease. He wrote, “As a new experience, it is to be added that: The lupus erythematosus may also appear and progress under the appearance of a widespread or subacute, feverish eruption and then, often affected with severe local and general symptoms of illness of the entire organism which can, indeed, endanger and destroy its life.”

Dr. Salmon notes that at the time, medical scholarship heavily relied on descriptive case series. In his extensive descriptions, Dr. Kaposi said, “Edematosis and thickening, glandular, painful swelling of the skin and tissues around the joints of the middle hand fingers, toes and also of the larger joints of the knee, of the elbow,” and also mentioned “ripping, tearing, deep bone pains, especially in the main bones, especially the tibia, forearm and carpal bones.”