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Historical approach to scleroderma
Historical Approach to Scleroderma UPENDRA DE SILVA, MD LAWRENCE CHARLES PARISH,
MD
cleroderma seems to be a comparatively easy dermatologic entity to trace through medical history; yet, it differs little from a multitude of other diseases for which the nomenclature and the classification have been repeatedly muddled.’ The current terminology, including morphea, scleroderma, and progressive systemic sclerosis, can sometimes lead to confusion (Table 1).2 The condition under consideration has clinical manifestations that can be recorded by patients and nonprofessional observers.3 Physicians in ancient times viewed patients who may have had the characteristic hardening of the skin, but there are often not sufficient details to confirm the diagnosis. Hippocrates, for example, records patients in whom “the skin is stretched, and parched and hard, the disease terminates without sweat.” 4 More information can be found in several reviews of the history of scleroderma.2,5-* Scattered reports from the seventeenth and early eighteenth centuries are suggestive of sclerodenna but illustrate the existing confusion. A 1731 presentation before the Royal Society in London may have been scleroderma or more possibly scleredema:
S
A Country Labourer, living not far from Euston-Hull in Suffolk, shewed a Boy (his Son) about Four teen Years of Age, having a cuticular Distemper, of a different Kind from any hitherto mentioned in the Histories of Diseases. His Skin (if it might be so called) seemed rather like a dusky coloured thick Cafe exactly fitting every Part of his Body, made of a rugged Bark, or Hide, with Bristles in some Places, which Case covering the whole excepting the Face, the Palms of the Hands, and the Soles of the Feet, caused an
From the Department of Dermatology and the Jefferson Centerfor Znternational Dermatology, Jefferson Medical College, Thomas Jefferson Uniuersity, Philadelphia, Pennsylvania. Address correspondence to Lawrence Charles Parish,MD, 1819 1. F. Kennedy Boulevard, Philadelphia, PA 19103.
0 1994 by Elsevier Science Inc.
l
0738-081x/94/$7.00
Appearance cloathed.9
as if those Parts alone were naked,
and the rest
Scleroderma - The Term By the first part of the nineteenth century, physicians were describing clinical entities that would become part of modem medicine. In 1836, Giovambattista Fantonetti (1791- 1861), a prominent physician in Milan, published the first patient whose disease was called scleroderma. Writing in his own journal, Effemetidi della Scienze Mediche, he described Antonia Alessandri, a 30-year-old peasant, who was found to have diffuse edema and large pustules on her body and legs. Within a short period, her skin had become hard and brown.
When
a “cooling
medicine”
failed
to alleviate
her
problems, she was hospitalized in Pavia. Her skin was described as being dark, tense, and firm as leather, except for her face and the nipples of her breasts. She could hardly move her hands, arms, or legs. In fact, “it was difficult to stick a needle into the skin.” Purging, ingesting dulcamara (bittersweet), and bathing in cicuta water (hemlock) softened her skin, but the right leg was still “hard as wood,” that is, until the intervention of leeches. Two weeks later, mercurial ointment was prescribed. After 3 months, “all parts of the skin was soft and of the natural color, with the exception of that of the right leg.” With her rather miraculous cure, the question arises about the diagnosis, scleroderma or scleredema. lo Although Fantonetti was the first to use the term scleroderma to designate a disease in an adult, his patient was unlikely to have had the disease known by this name. She recovered from the clinical manifestations within 3 weeks of the onset of the induration. The Greek word skleros became appropriate to describe the transient hardness or induration; hence, the varied nomenclature that developed in the nineteenth century.5 A French physician, Elie
201
202
DE
SILVA
AND
Clinics in Dermatology 1994:22:202-205
PARISH
Table 1. Synonymsfor Scleroderma Term
Author
Sclereme des adultes Skleroderma Sclerema adultorum, Sclereme des adultes Ichthyosis cornea Chorionitis, Sclerostenose cutanee Erytheme chronique Scleroderma adultorum Cutis tensa chronica Sclereme simple Keloid of Addison Scleroma Sclerosis cutanea simplex Hautsklerom Skleriasis, Sklerodermia Cicatrisierendes hautsclerem Sclerodermia Elephantiasis sclerosa Dermatosklerosis, Sclerosis dermatos, Morphoea Sclerodermie des extremites Progressive systemic sclerosis
Year
Nationality
Alibert Fantonetti Thiri al
1817 1836 1845
French Italian French
Startin Forget
1846 1847
English French
Grisolle Briick
Am%? Rasmussen Wilson
1847 1847 1854 1854 1854 1861 1861 1862 1863 1864 1864 1867 1869
French German German French English German German German German German German Danish English
Hardy Goetz
1877 1945
French South African
Gillette Addison Foerster Kiihler Mosler Virchow Wemicke
Adapted from Benedek and Rodnan.’
Gintrac (1791-1877), 1847. (see Table 1).
revived
the term scle’rodermie in
Scleroderma - The Disease If the Duchy of Savoia did not win the honors for the first description of scleroderma, the Kingdom of the Two Sicilies did. In 1752, Carlo Curzio presented a 17-year-old woman who had been admitted to the Royal Hospital in Naples. She was complaining of diffuse hardness of her skin throughout her body. Examination revealed the firmness to vary. The neck, forehead, and eyelids were very firm (she could not completely open or close her eyes), while the extremities were not firm enough to interfere with their use. Curzio prescribed phlebotomies, vapor baths, and quicksilver. The treatment was successful enough that 11 months later her skin was pliable and soft once more.” The governor of the Royal Hospital in Naples was so intrigued by the successful treatment of Patrizia Galiera that he wrote to the Royal Academy of Sciences in Paris. In 1755, the report was published in Paris. Subsequently, the pioneer dermatologists Robert Willan (1757 - 1812) and Jean Alibert (1768 - 1837) would refer to the disease in their texts.* Carlo Curzio’s accomplishments were memorialized by the establishment of the Carlo Curzio Societyfor the Study of Connective Tissue Diseases at the time of the First International Meeting on Eosinophilic Fusciitis, Naples, Italy, May 9, 1981 (see Figure 5).15 l
Like other diseases, scleroderma was constantly being rediscovered throughout the nineteenth century, but it was a definite entity, as shown by the moulages still present in the Mu&e de l’H6pital Saint Louis in Paris (Figures l-4). The first American account was given by Abraham B. Arnold of Baltimore. Calling the disease scleroderma, he described a 52-year-old man, dyspeptic in nature, who developed a cough during the winter. This was soon followed by hardness of his hands and feet. The fingers became hard, giving the hands a rounded appearance. They were cold and clammy. He found that hydrotherpeutics caused resolution of the hardness of the arms but not of the hands or fingers. Even administration of potassium and iodide made no difference. His 1869 report included two other similarly afflicted patients.12 More physicians began to recognize the disease and its devastation. By the late nineteenth century, the systemic manifestations of scleroderma had become known. Sir William Osler (1849 - 19 19) noted: In its more aggravated forms diffuse scleroderma is one of the most terrible of all human ills. Like Tithonus, to “wither slowly,” and like him to be “beaten down and marred and wasted” until one is literally a mummy, encased in an evershrinking, slowly contracting skin of steel, is a fate not pictured in any tragedy, ancient or modem.13
Scleroderma has continued to be the preferred term to incorportate the cutaneous changes of a generalized disease. In 1945, Robert Goetz in Cape Town, South Africa proposed that the term be discarded and the more descriptive progressive systemic sclerosis be used to reflect the systemic nature of the malady.14 The term “sclerosis” already being in use, it appears that “progressive systemic sclerosis” would describe the condition adequately until such time as the etiology has been established. Scleroderma is then only of the symptoms or signs of progressive systemic sclerosis.
Morphea The localized form of scleroderma was apparent as early as 1854. In a presentation to the Royal Medical and Chirurgical Society in London, Thomas Addison (1793 - 1860) called attention to a localized firmness found in several patients. One of his patients was a 30-year-old woman. The integument had a hard, drawn, tight look on the limb being extended; there might be felt through nearly the whole length of both arm and leg a rigid band which gave to the touch the impression of some inelastic substance tightly strained under the
integument.“j
Addison designated his findings had already introduced the term ologic literature to mean crab’s Alibert as a type of hypertrophic
as true keloids. Alibert chtloi’de into the dermatclaw; thus, the keloid of scarring and the keloid
Clinics in Dermatology 1994;12:201-205
Figure 1
Figure 3
HISTORICAL
DE SILVA AND PARISH APPROACH TO SCLERODERMA
203
Figure 2
Figure 4
Figures l-4 Moulages from the Muse’ede I’HGpital St. Louis in Palis, depicting various aspectsof scleroderma as seen in the nineteenth centu y. of Addison as morphea. ’ Erasmus Wilson (1809- 1884) of London appears to have introduced the term morpheu, which is derived from the Greekformu, meaning a spot on the skin.” When Henry Radcliffe Cracker (1845 - 1909) of London examined a patient with morphea, he obtained a specimen for histopathologic correlation. Initially, he could only recognize a lack of alteration in the epidermis.
As the disease progressed, the corium demonstrated atrophy of the papillae and an increase in the connective tissue. He concluded that the skin in morphea is white and opaque, while the skin in scleroderma is pale or yellowish. Finally, Cracker agreed with Fagge’s impression that morphea is a superficial and localized form of scleroderma, suggesting the term diffused and circumscribed scleroderma.lB
204
DE
SILVA
AND
Clinics in Dermatology 1994;12:201- 205
PARISH
in the latter situation later.19
and for the extremities
only to be attacked
Scleredema Adultorum Curzio
may have recognized
the first patient
with
sclere-
dema adultorum, but attention for this disease seemed to have lagged for some time. C. Hilton Fagge (1838-1883) described a 40-year-old woman who was admitted to Guy’s Hospital in London. The hardness and induration extended from the right side of the face to the neck, trunk, and arms within 2 months. Fagge termed the disease scleriasis and credited H.-E Thirial (1810- 1863) as describing similar afflictions in Paris; however, he objected to Thirial’s name, sclereme des adultes, as the disease could occur in children.*O Not only did the nomenclature expand, but the confusion resulting from the mixing up of diseases and their manifestations continued. The disease did not come into its own until 1902, when Abraham Buschke (1868-1943) presented his patient to the Berlin Dermatological Society.*l Henry Piffard (1842-1910)
Figure
5. Plaque (b) commemorating Carlo Curzio in Naples,
Italy.
of New
York,
in observing
a 49-year-old
blacksmith with hardening of the shoulder, had previously realized that the man’s scleriasis differed from scleroderma, as the condition cleared within 3 months, but the contemporary term would be created by Buschke (Table 2).** Table 2. Svnonumsfor Scleredema
Sclerema Neonatorum This affliction of infants reached epidemic proportions in eighteenth-century France. By 1788, an average of 600 infants were admitted to I’Hopital des Enfants-trouves and 400 infants to I’Hotel-Dieu. The diagnosis of “enfans geles” or “enjuns durs” was tantamount to death. Michael Underwood (173 7 - 1820), a London pediatrician, called attention to the disease, later known as Underwood’s disease, in his Treatise on the Diseases of Children, published in 1784 and recognized as the first pediatric textbook in English. Moritz Kaposi (Kohn) (1837-1902), in the five-volume text edited by himself and his father-in-law, Ferdinand von Hebra (18 16 - 1880), discussed sclerema neonatorum, stating: By this designation, which is already much in vogue, we wish to which distinguish by name the disease of early childhood, differs clinically and anatomically from the sclerodema of adults. . . The disease makes its appearance first of all, almost invariably, on the lower extremities, and extends from thence upwards on to the abdomen. As the disease advances, it appears on the upper extremities and on the face. It is rare for it to begin
Term
Author
SclQeme des adultes Acute scleriasis Scleriasis Sclerodermie oedemateuse Scleroedema Sclerohypodermie ou sclerofascie Straitmodennie Scleroedeme benin de l’adulte Scleroedema (sclerema) adultorum Progressive benign subcutaneous induration Benign sclereme of aponeuroses Scleroedema adultorum (Buschke) Benign scleremia Scleroderma adultorum Buschke Diffuse benign sclerodermiform cellulitis
Year
Nationality
Fagge Piff ard Hardy Buschke Blaschko
1845 1869 1876 1877 1902 1902
French English American French German German
Baginsky Nob1
1903 1912
German French
Hoffmann
1923
German
Sellei
1928
Hungarian
1930
French
Ehrmann, Briinauer Merenlander, Zand Maschkilleisson
1931
German
1935
French
1935
Russian
Touraine, Gale, Soulignac
1937
French
Thirial
Audry,
de
Adapted from Benedek and Rodnnn.’
Gadrat
Clinics in Dermatology 1994;22:201-205
Conclusions Scleroderma is a well-defined disease today, one that is recognized for having both cutaneous and systemic manifestations. Recent speculation has focused on whether this connective tissue disease is induced by a Borrelium or is related to an occupational hazard, as in the case of the famous painter Paul Klee (1879 - 1940) in whom lead poisoning may have been a factor.23
References 1. Parish LC. An historical approach to the nomenclature of rheumatoid arthritis. Arthrit Rheum 1963;6:138-58. 2. Rodnan HP, Jablonska S, Medsger TA. Classification and nomenclature of progressive systemic sclerosis (scleroderma). Clin Rheum Dis 1979;5:1- 13. 3. Borroni G. Does a pre-Raphaelite painting by Ford Madox Brown depict a case of systemic sclerosis? Am J Dermatopath01 1985;7:353-56. 4. David M. A case of scleroderma mentioned by Hippocrates in his aphorisms. Koroth 1981;8:61-63. 5. Benedek TG, Rodnan GP. The early history and nomenclature of scleroderma and of its differentiation from sclerema neonatorum and scleroedema. Sem Arthr Rheum 1982; 12:52-67. 6. Rodnan GP, Benedek TG. An historical account of the study of progressive systemic sclerosis (diffuse scleroderma). Ann Int Med 1962;57:305-19. 7. Moynahan EJ. Addison’s place in dermatology. Guy’s Hosp Rep 1960;109:262-68. 8. Fagge CH. Keloid, scleriasis, morphoea. Guy’s Hosp Rep 1868;13:255-81. 9. Machin J. An extract from the minutes of the Royal Society, March 16, 1731, containing an uncommon case of a dietempered skin. Philos Trans Roy Sot Lond 1731;37:299302.
HISTORICAL
DE SILVA AND PARISH APPROACH TO SCLERODERMA
205
10. Fantonetti G. Case of general induration of the skin. Dublin J Med Sci 1838;13:158-59. 11. Curzio C. Discussioni anatomico-pratiche di un raro, estravagante morbo cutaneo in una giovane Donna felicemente curato in quest0 grande Ospedale degl’IncurabiIi. Napoli: Giovanni di Simone, 1753: 1 - 13. 12. Arnold AB. Three cases of scleroderma; with remarks. Am J Med Sci 1869;58:89-91. 13. Osler W. On diffuse scleroderma; With special reference to diagnosis, and to the use of the thyroid-gland extract. J Cutan & G-U Dis 1898;16:49-67,127-34. 14. Goetz RH. Pathology of progressive systemic sclerosis (generalized scleroderma) with special reference to changes in the viscera. Clin Proc 1945;4:337-92. 15. Giordano M. Introduction. Connect Tiss Dis 1982;1:9-12. 16. Kaposi M. Scleroderma adultorum. In: Hebra F, Kaposi M, editors. On Disease of the Skin Including the Exanthemata. London: The New Sydenham Society, 1874:104-24. vol III. 17. Wilson E. On striae et maculae atrophicae cutis, or false cicatrices of the skin. J Cut Med 1867-68;1:140-47, 18. Cracker HR. Diseases of the skin. Trans Path Sot Lond 1880;31:315-22. 19. Kaposi M. Sclerema neonatorum. In: Hebra F, Kaposi M., editors. On Disease of the Skin Including the Exanthemata. London: The New Syndenham Society, 1874:124-29. vol III. 20. Fagge CH. Diseases, etc., of the skin and its appendages. Trans Path01 Sot Lond 1868;20:403-09. 21. Buschke A. Ueber scleroedem. Berl Klin Wochenschr 1902;39:955 -57. 22. Piffard HG. An Elementary Treatise on Diseases of the Skin, for the Use of Students and Practitioners. New York: Macmillan, 1876:366-68. 23. Pedersen LM, Permin H. Rheumatic disease, heavy-metal pigments, and the great masters. Lancet 1988;i:1267-69.
MD
cleroderma seems to be a comparatively easy dermatologic entity to trace through medical history; yet, it differs little from a multitude of other diseases for which the nomenclature and the classification have been repeatedly muddled.’ The current terminology, including morphea, scleroderma, and progressive systemic sclerosis, can sometimes lead to confusion (Table 1).2 The condition under consideration has clinical manifestations that can be recorded by patients and nonprofessional observers.3 Physicians in ancient times viewed patients who may have had the characteristic hardening of the skin, but there are often not sufficient details to confirm the diagnosis. Hippocrates, for example, records patients in whom “the skin is stretched, and parched and hard, the disease terminates without sweat.” 4 More information can be found in several reviews of the history of scleroderma.2,5-* Scattered reports from the seventeenth and early eighteenth centuries are suggestive of sclerodenna but illustrate the existing confusion. A 1731 presentation before the Royal Society in London may have been scleroderma or more possibly scleredema:
S
A Country Labourer, living not far from Euston-Hull in Suffolk, shewed a Boy (his Son) about Four teen Years of Age, having a cuticular Distemper, of a different Kind from any hitherto mentioned in the Histories of Diseases. His Skin (if it might be so called) seemed rather like a dusky coloured thick Cafe exactly fitting every Part of his Body, made of a rugged Bark, or Hide, with Bristles in some Places, which Case covering the whole excepting the Face, the Palms of the Hands, and the Soles of the Feet, caused an
From the Department of Dermatology and the Jefferson Centerfor Znternational Dermatology, Jefferson Medical College, Thomas Jefferson Uniuersity, Philadelphia, Pennsylvania. Address correspondence to Lawrence Charles Parish,MD, 1819 1. F. Kennedy Boulevard, Philadelphia, PA 19103.
0 1994 by Elsevier Science Inc.
l
0738-081x/94/$7.00
Appearance cloathed.9
as if those Parts alone were naked,
and the rest
Scleroderma - The Term By the first part of the nineteenth century, physicians were describing clinical entities that would become part of modem medicine. In 1836, Giovambattista Fantonetti (1791- 1861), a prominent physician in Milan, published the first patient whose disease was called scleroderma. Writing in his own journal, Effemetidi della Scienze Mediche, he described Antonia Alessandri, a 30-year-old peasant, who was found to have diffuse edema and large pustules on her body and legs. Within a short period, her skin had become hard and brown.
When
a “cooling
medicine”
failed
to alleviate
her
problems, she was hospitalized in Pavia. Her skin was described as being dark, tense, and firm as leather, except for her face and the nipples of her breasts. She could hardly move her hands, arms, or legs. In fact, “it was difficult to stick a needle into the skin.” Purging, ingesting dulcamara (bittersweet), and bathing in cicuta water (hemlock) softened her skin, but the right leg was still “hard as wood,” that is, until the intervention of leeches. Two weeks later, mercurial ointment was prescribed. After 3 months, “all parts of the skin was soft and of the natural color, with the exception of that of the right leg.” With her rather miraculous cure, the question arises about the diagnosis, scleroderma or scleredema. lo Although Fantonetti was the first to use the term scleroderma to designate a disease in an adult, his patient was unlikely to have had the disease known by this name. She recovered from the clinical manifestations within 3 weeks of the onset of the induration. The Greek word skleros became appropriate to describe the transient hardness or induration; hence, the varied nomenclature that developed in the nineteenth century.5 A French physician, Elie
201
202
DE
SILVA
AND
Clinics in Dermatology 1994:22:202-205
PARISH
Table 1. Synonymsfor Scleroderma Term
Author
Sclereme des adultes Skleroderma Sclerema adultorum, Sclereme des adultes Ichthyosis cornea Chorionitis, Sclerostenose cutanee Erytheme chronique Scleroderma adultorum Cutis tensa chronica Sclereme simple Keloid of Addison Scleroma Sclerosis cutanea simplex Hautsklerom Skleriasis, Sklerodermia Cicatrisierendes hautsclerem Sclerodermia Elephantiasis sclerosa Dermatosklerosis, Sclerosis dermatos, Morphoea Sclerodermie des extremites Progressive systemic sclerosis
Year
Nationality
Alibert Fantonetti Thiri al
1817 1836 1845
French Italian French
Startin Forget
1846 1847
English French
Grisolle Briick
Am%? Rasmussen Wilson
1847 1847 1854 1854 1854 1861 1861 1862 1863 1864 1864 1867 1869
French German German French English German German German German German German Danish English
Hardy Goetz
1877 1945
French South African
Gillette Addison Foerster Kiihler Mosler Virchow Wemicke
Adapted from Benedek and Rodnan.’
Gintrac (1791-1877), 1847. (see Table 1).
revived
the term scle’rodermie in
Scleroderma - The Disease If the Duchy of Savoia did not win the honors for the first description of scleroderma, the Kingdom of the Two Sicilies did. In 1752, Carlo Curzio presented a 17-year-old woman who had been admitted to the Royal Hospital in Naples. She was complaining of diffuse hardness of her skin throughout her body. Examination revealed the firmness to vary. The neck, forehead, and eyelids were very firm (she could not completely open or close her eyes), while the extremities were not firm enough to interfere with their use. Curzio prescribed phlebotomies, vapor baths, and quicksilver. The treatment was successful enough that 11 months later her skin was pliable and soft once more.” The governor of the Royal Hospital in Naples was so intrigued by the successful treatment of Patrizia Galiera that he wrote to the Royal Academy of Sciences in Paris. In 1755, the report was published in Paris. Subsequently, the pioneer dermatologists Robert Willan (1757 - 1812) and Jean Alibert (1768 - 1837) would refer to the disease in their texts.* Carlo Curzio’s accomplishments were memorialized by the establishment of the Carlo Curzio Societyfor the Study of Connective Tissue Diseases at the time of the First International Meeting on Eosinophilic Fusciitis, Naples, Italy, May 9, 1981 (see Figure 5).15 l
Like other diseases, scleroderma was constantly being rediscovered throughout the nineteenth century, but it was a definite entity, as shown by the moulages still present in the Mu&e de l’H6pital Saint Louis in Paris (Figures l-4). The first American account was given by Abraham B. Arnold of Baltimore. Calling the disease scleroderma, he described a 52-year-old man, dyspeptic in nature, who developed a cough during the winter. This was soon followed by hardness of his hands and feet. The fingers became hard, giving the hands a rounded appearance. They were cold and clammy. He found that hydrotherpeutics caused resolution of the hardness of the arms but not of the hands or fingers. Even administration of potassium and iodide made no difference. His 1869 report included two other similarly afflicted patients.12 More physicians began to recognize the disease and its devastation. By the late nineteenth century, the systemic manifestations of scleroderma had become known. Sir William Osler (1849 - 19 19) noted: In its more aggravated forms diffuse scleroderma is one of the most terrible of all human ills. Like Tithonus, to “wither slowly,” and like him to be “beaten down and marred and wasted” until one is literally a mummy, encased in an evershrinking, slowly contracting skin of steel, is a fate not pictured in any tragedy, ancient or modem.13
Scleroderma has continued to be the preferred term to incorportate the cutaneous changes of a generalized disease. In 1945, Robert Goetz in Cape Town, South Africa proposed that the term be discarded and the more descriptive progressive systemic sclerosis be used to reflect the systemic nature of the malady.14 The term “sclerosis” already being in use, it appears that “progressive systemic sclerosis” would describe the condition adequately until such time as the etiology has been established. Scleroderma is then only of the symptoms or signs of progressive systemic sclerosis.
Morphea The localized form of scleroderma was apparent as early as 1854. In a presentation to the Royal Medical and Chirurgical Society in London, Thomas Addison (1793 - 1860) called attention to a localized firmness found in several patients. One of his patients was a 30-year-old woman. The integument had a hard, drawn, tight look on the limb being extended; there might be felt through nearly the whole length of both arm and leg a rigid band which gave to the touch the impression of some inelastic substance tightly strained under the
integument.“j
Addison designated his findings had already introduced the term ologic literature to mean crab’s Alibert as a type of hypertrophic
as true keloids. Alibert chtloi’de into the dermatclaw; thus, the keloid of scarring and the keloid
Clinics in Dermatology 1994;12:201-205
Figure 1
Figure 3
HISTORICAL
DE SILVA AND PARISH APPROACH TO SCLERODERMA
203
Figure 2
Figure 4
Figures l-4 Moulages from the Muse’ede I’HGpital St. Louis in Palis, depicting various aspectsof scleroderma as seen in the nineteenth centu y. of Addison as morphea. ’ Erasmus Wilson (1809- 1884) of London appears to have introduced the term morpheu, which is derived from the Greekformu, meaning a spot on the skin.” When Henry Radcliffe Cracker (1845 - 1909) of London examined a patient with morphea, he obtained a specimen for histopathologic correlation. Initially, he could only recognize a lack of alteration in the epidermis.
As the disease progressed, the corium demonstrated atrophy of the papillae and an increase in the connective tissue. He concluded that the skin in morphea is white and opaque, while the skin in scleroderma is pale or yellowish. Finally, Cracker agreed with Fagge’s impression that morphea is a superficial and localized form of scleroderma, suggesting the term diffused and circumscribed scleroderma.lB
204
DE
SILVA
AND
Clinics in Dermatology 1994;12:201- 205
PARISH
in the latter situation later.19
and for the extremities
only to be attacked
Scleredema Adultorum Curzio
may have recognized
the first patient
with
sclere-
dema adultorum, but attention for this disease seemed to have lagged for some time. C. Hilton Fagge (1838-1883) described a 40-year-old woman who was admitted to Guy’s Hospital in London. The hardness and induration extended from the right side of the face to the neck, trunk, and arms within 2 months. Fagge termed the disease scleriasis and credited H.-E Thirial (1810- 1863) as describing similar afflictions in Paris; however, he objected to Thirial’s name, sclereme des adultes, as the disease could occur in children.*O Not only did the nomenclature expand, but the confusion resulting from the mixing up of diseases and their manifestations continued. The disease did not come into its own until 1902, when Abraham Buschke (1868-1943) presented his patient to the Berlin Dermatological Society.*l Henry Piffard (1842-1910)
Figure
5. Plaque (b) commemorating Carlo Curzio in Naples,
Italy.
of New
York,
in observing
a 49-year-old
blacksmith with hardening of the shoulder, had previously realized that the man’s scleriasis differed from scleroderma, as the condition cleared within 3 months, but the contemporary term would be created by Buschke (Table 2).** Table 2. Svnonumsfor Scleredema
Sclerema Neonatorum This affliction of infants reached epidemic proportions in eighteenth-century France. By 1788, an average of 600 infants were admitted to I’Hopital des Enfants-trouves and 400 infants to I’Hotel-Dieu. The diagnosis of “enfans geles” or “enjuns durs” was tantamount to death. Michael Underwood (173 7 - 1820), a London pediatrician, called attention to the disease, later known as Underwood’s disease, in his Treatise on the Diseases of Children, published in 1784 and recognized as the first pediatric textbook in English. Moritz Kaposi (Kohn) (1837-1902), in the five-volume text edited by himself and his father-in-law, Ferdinand von Hebra (18 16 - 1880), discussed sclerema neonatorum, stating: By this designation, which is already much in vogue, we wish to which distinguish by name the disease of early childhood, differs clinically and anatomically from the sclerodema of adults. . . The disease makes its appearance first of all, almost invariably, on the lower extremities, and extends from thence upwards on to the abdomen. As the disease advances, it appears on the upper extremities and on the face. It is rare for it to begin
Term
Author
SclQeme des adultes Acute scleriasis Scleriasis Sclerodermie oedemateuse Scleroedema Sclerohypodermie ou sclerofascie Straitmodennie Scleroedeme benin de l’adulte Scleroedema (sclerema) adultorum Progressive benign subcutaneous induration Benign sclereme of aponeuroses Scleroedema adultorum (Buschke) Benign scleremia Scleroderma adultorum Buschke Diffuse benign sclerodermiform cellulitis
Year
Nationality
Fagge Piff ard Hardy Buschke Blaschko
1845 1869 1876 1877 1902 1902
French English American French German German
Baginsky Nob1
1903 1912
German French
Hoffmann
1923
German
Sellei
1928
Hungarian
1930
French
Ehrmann, Briinauer Merenlander, Zand Maschkilleisson
1931
German
1935
French
1935
Russian
Touraine, Gale, Soulignac
1937
French
Thirial
Audry,
de
Adapted from Benedek and Rodnnn.’
Gadrat
Clinics in Dermatology 1994;22:201-205
Conclusions Scleroderma is a well-defined disease today, one that is recognized for having both cutaneous and systemic manifestations. Recent speculation has focused on whether this connective tissue disease is induced by a Borrelium or is related to an occupational hazard, as in the case of the famous painter Paul Klee (1879 - 1940) in whom lead poisoning may have been a factor.23
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