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You are here: Home / Articles / A Historical Look at the Characterization of Lupus as a Systemic Disease

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

October 18, 2018 • By Ruth Jessen Hickman, MD

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Dr. Salmon notes, “He mentions coma and stupor, he notes atrophy of the cortical substance and edema of the meninges. In his descriptions, we have rash, arthritis, pleurisy and central nervous system involvement, as well as constitutional signs such as fever—that’s a pretty good number of features listed in the ACR criteria for classification of lupus.”

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Dr. Kaposi provided a bleak description of the disease’s most acute manifestations. “At the same time, the patients concerned had the symptoms of an intense, generalized, feverish disease. They laid on the back, had a hot, dry, cracked tongue, general prostration, disturbed consciousness. Over the course of two to three weeks, coma … and death occurred under increasing brain disorder. Among 15 female cases of lupus erythematosus, we saw this picture five times, and three times death occurred with the symptoms described.”

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The difference in disease course of the two forms of rash earlier described by Dr. Hebra is detailed by Dr. Kaposi: a form of lupus that is cutaneous only (lupus erythematosus discoides) and a form of lupus that occurs with systemic and sometimes life-threatening symptoms (designated as lupus erythematosus discretus et aggregatus).

Lupus often presents with a butterfly rash.

Lupus often presents with a butterfly rash.

Dr. Kaposi noted: “The lupus erythematosus discoides runs regularly and continuously in a chronic fashion, and usually without any serious complication. … Lupus erythematosus aggregates, on the other hand, also show a chronic affect, and alone more often than either from the beginning, or at least during the later course, acute eruptions occurred. Accordingly, all those intense local and deleterious general manifestations of the disease, which I have described as accompanying the acute eruption of the lupus erythematosus previously, predominantly, or almost exclusively belong to that second form of the lupus erythematosus, and this is accordingly the prognostically more serious one to watch.”

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The description and characterization of rash types maps fairly well onto modern distinctions between patients who have cutaneous discoid disease only and those who have SLE. As Dr. Salmon notes, “Most patients who only have discoid disease never develop systemic lupus, whereas patients with more serious skin manifestations—subacute cutaneous lupus or vasculitis—tend to be the ones who have more systemic disease.”

Advances in Understanding

The renowned internist and educator Sir William Osler described SLE and further advanced the idea that skin disease (including lupus erythematosus) can be a sign of significant systemic illness, and that systemic manifestations may occur in some cases even without skin disease.6 By the turn of the century, SLE had become well recognized as a systemic disease, though little was known about its etiology.4 The discovery of the LE cell by Malcolm Hargrave, MD, in 1948 emphasized the systemic nature of the disease and ushered in our modern understanding of SLE.5

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Filed Under: Conditions, SLE (Lupus) Tagged With: Classification Criteria, Dr. Moriz Kaposi, History, Lost & FoundIssue: October 2018

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About Ruth Jessen Hickman, MD

Ruth Jessen Hickman, MD, was born and raised in eastern Kentucky, where she first cultivated her love of literature, writing and personal narratives. She attended Kenyon college, where she received a Bachelor of Arts in philosophy, summa cum laude. She worked with individuals with psychiatric conditions and later in a neuroscience lab at the University of Illinois, Chicago, before graduating from Indiana University Medical School in 2011. Instead of pursuing clinical medicine, Ruth opted to build on her strength of clearly explaining medical topics though a career as a freelance medical writer, writing both for lay people and for health professionals. She writes across the biomedical sciences, but holds strong interests in rheumatology, neurology, autoimmune diseases, genetics, and the intersection of broader social, cultural and emotional contexts with biomedical topics. Ruth now lives in Bloomington, Ind., with her husband, son and cat. She can be contacted via her website at ruthjessenhickman.com.

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