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." |
