A Brief History of Scleroderma

by Walter Coyle, Scleroderma Foundation New England Chapter Assistant Treasurer and former National Board member (originally published in "Scleroderma News," 1988, Vol. 8, No. 2)

While scleroderma may not be very well known to the average person, investigation reveals it has been around for a long time.

Classical References
Cases of skin disease similar to scleroderma may be found in the writings of Hippocrates as far back as 460–370 B.C. Other ancients, including Oribasius (325–403 A.D.) and Paulus Agineta (625–690 A.D.), also wrote on the subject. It is difficult for us to know if these were truly examples of scleroderma because the descriptions were inexact.

The First Modern Reference
The first truly definite description of the disease was written by Carlo Curzio in a monograph published in Naples in 1753. This account produced considerable interest in Paris and London medical circles.

The account concerns a young woman of 17 named Patrizia Galiera, who was admitted to the hospital and assigned to Dr. Curzio. Her symptoms as described by the doctor involved hardness of the skin (differing in degree from place to place), tightness around the mouth, and hardness around the neck. He noted loss of warmth in the skin but no other problem in pulse, respiration, or digestion.

Much of the report contains details of the treatment, which included warm milk and vapor baths, bleeding from the foot, and small doses of quicksilver. Happily (according to the report), after 11 months the skin became soft and flexible, and all natural functions were restored!

Curzio's observations were published in French in 1755 and aroused considerable interest. The early dermatological texts of R. William in London (1808) and his student, J. L. Alibert, in Paris (1818) referred to Curzio's observation.

Localized Scleroderma Is Described
Later, Alibert claimed to be the first to recognize the disease, calling it "Sclermia Circumscripia," and describing two cases of what was probably linear scleroderma.

Scleroderma in the 19th Century
There seems to be little mention of scleroderma in medical literature in the intervening years until 1847. In that year, M. Grisoll in Paris and C.P. Forget in Strasbourg rediscovered the disease and opened new areas of discussion and review.

As previously mentioned, Alibert initially described localized scleroderma, but T. Addison in 1854 took care to differentiate other types of the disease from the Alibert description.

In 1857, Erasmus Wilson identified "morphea" and "en coup de sabre," which terms resulted when he described the lesions of one of his patients as "resembling the scar of a sword wound." Incidentally, Sir Arthur Conan Doyle, creator of Sherlock Holmes, used localized scleroderma as the theme of his story "The Adventure of the Blanched Soldier."

The First Association of Scleroderma with Raynaud's Disease

Abnormal vasoconstriction with scleroderma was first documented by M. Raynaud. Raynaud described a 30-year old farmer who, in 1863, began to have numbness in his arms during winter and noted gradual development of hardness in his hands and darkening of his skin. Today we call similar cases by the name of Raynaud's disease or syndrome.

Finally, P. Horteloup wrote a comprehensive monologue in 1865 and the term "sclerodermie" was generally accepted after much discussion. Also in 1865, G. Lewin and J. Heller published an extensive review of the disease in Berlin.

The first American report appeared in the "American Journal of Medicine" in 1869 and described a history of three patients.

Later, in 1899, T. K. Monro reviewed 180 cases of Raynaud's disease and reported a marked tendency to scleroderma in 13 cases.

By 1900, more than 500 cases of scleroderma had been reported and discussed in the medical literature.

The 20th Century
During the early 1900s, more and more studies took place and researchers began to study the relationship of scleroderma to other diseases and the term collagen disease developed. However, in 1953, P. Klemperer in the "American Journal of Pathology" warned that we should be careful not to use the term collagen disease as a catch-all for any puzzling malady.

Conclusion
Today, interest in scleroderma grows and more data continues to be collected. Some of the studies now being supported by the Scleroderma Foundation will undoubtedly be part of the future history of scleroderma.

Mr. Coyle is Treasurer of the Scleroderma Foundation, and a member of its Board of Directors. The author excerpted some material for the article from a paper written by G.P. Rodnan, M.D., and T.G. Benedek, M.D., in the August 1962 "Annals of Internal Medicine."

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