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Dr.

Arthur Conan Doyle and the Case of Congenital Syphilis


Arthur M. Silverstein, Christine Ruggere

Perspectives in Biology and Medicine, Volume 49, Number 2, Spring 2006,


pp. 209-219 (Article)

Published by Johns Hopkins University Press


DOI: https://doi.org/10.1353/pbm.2006.0033

For additional information about this article


https://muse.jhu.edu/article/196007

Access provided by University of South Dakota (21 Nov 2018 13:12 GMT)
Dr. Arthur Conan Doyle
and the Case of
Congenital Syphilis

Arthur M. Silverstein and Christine Ruggere

ABSTRACT In 1894, Dr. Arthur Conan Doyle wrote “The Third Generation,” a
short story involving the transmission of congenital syphilis from generation to gener-
ation. Analysts of his writings have interpreted the pathogenetic mechanism involved
in modern terms: infection of mother by father and then transplacental infection of the
fetus. However, a review of the contemporary literature and the history of the concepts
of congenital and “hereditary” syphilis demonstrates that the late 19th-century under-
standing of the process involved a Lamarckian transmission of paternal infection, via the
sperm at the moment of conception. It was undoubtedly this concept that Doyle
learned in medical school in the late 1870s and that provided the background to his
story.

N 1894, DR. ARTHUR CONAN DOYLE (M.B. Edinburgh, 1881; M.D. Edin-
I burgh, 1885) wrote a short story entitled “The Third Generation.” In it, a
frightened young Londoner, Sir Francis Norton, consults Dr. Horace Selby one
evening. Selby is a specialist in a branch of medicine not often mentioned in
polite circles, but whose “European reputation” and whose practice permit
him—indeed encourage him—to live secluded and apart from the usual medical
centers.The worried young baronet shows Selby a serpiginous skin lesion on his
shin that had appeared suddenly that morning.
Selby seems to understand immediately what is at hand, and then wonders

Institute of the History of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
Correspondence: Dr. A. M. Silverstein, 38 Fells Road, Falmouth, MA 02540.
E-mail: arts@jhmi.edu.

Perspectives in Biology and Medicine, volume 49, number 2 (spring 2006):209–19


© 2006 by The Johns Hopkins University Press

209
Arthur M. Silverstein and Christine Ruggere

aloud whether Norton might have anything about which he is ashamed. “So
help me God, doctor,” cried Norton, “I have nothing in my life with which to
reproach myself.” To confirm his diagnosis, the doctor examines the patient’s
teeth and eyes, and “a glow of pleasure came over his large, expressive face.” He
had seen Hutchinson’s teeth (peg-like and notched incisors in the upper jaw)
and an interstitial keratitis (inflammation of the middle layers of the cornea), two
of the three components of “Hutchinson’s triad,” considered to be pathogno-
monic of congenital syphilis; the third is deafness. Although the word syphilis is
never mentioned in this Victorian era story, the diagnosis is clear. “This is very
typical—very typical indeed,” says the doctor. “Curiously enough I am writing
a monograph upon the subject. It is singular that you should have been able to
furnish so well marked a case.”
As the story unfolds, it develops that Norton himself is truly innocent of
wrongdoing. It was his grandfather, a notorious rake, who had first become
infected with this dread disease. He had apparently transmitted the disease to
Norton’s father, who had himself transmitted it to Norton “in the third genera-
tion.” Norton is to be married the following week, but must ask a question,“one
on which my whole life must depend.” Would his children suffer? The doctor
suggests that they would, and that the marriage must not take place. They then
discuss the possibility of Norton’s avoiding the marriage by backing out or by
one or another subterfuge, but the young man’s personal honor will not permit
him any sort of dishonesty. He accepts a prescription for some powders and an
ointment and leaves, with the vow to return in the morning. But at breakfast the
next morning, Dr. Selby learns from the newspaper that “A Deplorable Acci-
dent” had occurred late the previous night, in which Sir Francis Norton had
fallen under the wheels of a heavy two-horse dray and been killed. Norton had
obviously felt that he could no longer live with the shame—that of his disease
and especially that of his unfulfillable obligation to his fiancée.
As with all of Doyle’s writings, the medical aspects of this story have been
examined and analyzed often and in great detail (Accardo 1987; Rodin and Key
1984; Van Liere 1959; see especially Rodin and Key 1992, pp. 81–82). These
commentators point out that congenital (hereditary) syphilis had been known
since 1565, and that instances of its first manifestation long after birth are well
known. They describe the presumed course of events in a sequence familiar to
modern syphilologists: Norton’s diseased grandfather must have infected his
wife; she then infected Norton’s father by transplacental transmission; his father
then infected his mother; and finally, Norton himself was infected in utero by his
mother.
This is all very straightforward and modern, but probably incorrect. There is
good reason to wonder whether this is what Doyle actually had in mind 110
years ago when he wrote this sad tale. In the days before the rise of modern
genetics, a different concept of the transmission of certain characteristics from
parent to offspring was prevalent. This included the manner of inheritance not

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Dr. Arthur Conan Doyle and the Case of Congenital Syphilis

only of certain diseases, but of immunity as well. It was widely thought that the
father might transmit these characteristics directly via the sperm, without the
necessary intercession of the mother. It is the history of this idea that we shall
examine, with special reference to the era in which Doyle pursued his medical
studies.1 The evidence considered below strongly suggests that it was this La-
marckian theory of pathogenesis that young Arthur Conan Doyle learned at
Edinburgh in the late 1870s and that informed his scenario of 1894.

Concepts of the Pathogenesis of


Congenital Syphilis
It is necessary at the outset to point out the very special role that syphilis played
in the medicine of the late 19th century.Aside from being a notorious disease by
virtue of its venereal transmission, it was protean in the many clinical and path-
ological forms it could assume.2 Thus, prior to the introduction of the Was-
sermann serodiagnostic test in 1906 (Wasserman, Neisser, and Bruch 1906), syph-
ilis was widely over-diagnosed. To cite but two examples: most inflammatory
disease of the uveal tract of the eye was attributed in this era to syphilis (Silver-
stein 1997;Woods 1956, pp. 47–48). Similarly, syphilis was held to be responsible
for most cases of congenital disease in the newborn (Corbin 1981). In this envi-
ronment, it should not occasion surprise that the disease would figure signifi-
cantly in the medical affairs and medical education of the 1870s, nor that Doyle
would choose the complications of tertiary syphilis as the subject of his thesis for
the M.D. degree in 1885 and return to it as a subject for a story in 1894.
Syphilis also appeared prominently in the literature of the era, often con-
cerned with the tragic consequences of congenital syphilis (Davidson and Hall
2001; Hall 2001). Thus in Ibsen’s 1881 play Ghosts, after Mrs. Alving returns to
her unhappy marriage, she watches as “the sins of the father” are visited on her
son Osvald in the form of “hereditary” syphilis. Again, Eugène Brieux’s (1901)
play Les avariés (novelized by Upton Sinclair in 1913 as Damaged Goods) deals
with a case of a young man diagnosed with syphilis who is advised by his doc-
tor not to marry, but who has not the principles of Sir Francis Norton. He mar-
ries, and the result is the birth of a syphilitic child. Perhaps most widely known
is Sigmund Freud’s treatment of “inherited” syphilis in Dora (1905). Freud
observed that over one-half of severe cases of hysteria had fathers who had suf-
fered from syphilis. The patients had no signs of disease, but “Their abnormal

1 The question of “hereditary” syphilis has been examined by historians in other contexts (Lomax
1979; Quétel 1986;Valleur 1982). For more general discussions of the inheritance of acquired char-
acteristics in medicine, see Churchill (1976) and Rosenberg (1976, pp. 25–63).
2 The disease was so notorious that each blamed another: the Italians called it “the Spanish disease”;
the French named it “le mal de Naples”; and to the English (and many others) it was “the French
pox.” It was long regarded as a punishment from God for carnal sinning (Quétel 1986).

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Arthur M. Silverstein and Christine Ruggere

sexual contribution was to be regarded as the last echo of their syphilitic heritage”
(Freud 1905, emphasis added).
It is necessary also to appreciate the semantic difference between two words—
hereditary and congenital—that figure importantly in the history of syphilis infec-
tion of the fetus.While they may at times have been employed interchangeably,
the earlier (1597) term hereditary has always implied “the transmission of charac-
teristics or disease from generation to generation,” as the Oxford English Diction-
ary has it—a direct transfer from parent to offspring that in modern times would
be labeled “genetic.” Congenital, on the other hand, first used some 200 years later
(1796), merely means “existing at or present from birth” or “born with.”This lat-
ter term presumably arose with the recognition that certain conditions might be
acquired in utero.Whereas both terms were used until the early years of the 20th
century, only congenital is currently used to describe infectious diseases of the
fetus.
As syphilis spread widely in the late 15th century, the disease was seen also to
affect the unborn fetus, with symptoms often appearing at or shortly after birth.
In his extensive review of the history of congenital syphilis, Etienne Lancereaux
(1873) cites 16th-century writers who discussed the existence of “hereditary”
syphilis and that it could be transmitted to the fetus by semen corruptum. Indeed,
he quotes Paracelsus from 1536: “Tandem fit morbus hereditarius et transit a
patre ad filium, ab infecto ad alium” (It becomes finally a hereditary disease and
passes from father to offspring, from the infected one to the other) (Lancereax
1873, pp. 404–5).This theory of pathogenesis was passed on for over three cen-
turies and was still widely believed at the time that Doyle was studying and then
practicing medicine.We shall illustrate the thinking about the mode of transmis-
sion of syphilis from parent to offspring during the period from the 1870s on-
ward by reference to the two most prominent writers and three of the major
information sources of the period.

Alfred Fournier (1832–1914)


Alfred Fournier was France’s and perhaps Europe’s leading expert on syphilis and
its congenital manifestations. Indeed, the French referred to him as le pape des
syphilographes (the pope of syphilologists). In his magnum opus L’hérédité syphili-
tique, Fournier (1891) carefully defines his terms. Heredity is “that which is
transmitted at the time of fecundation. It is the contribution made to the germ, to
the future embryo, of qualities proper to the two generative cells (sperm and
ovum) at the moment of conjunction” (p. 9). He further emphasizes this by
adding that “hereditary syphilis is that which originates in syphilitic infection of
the parents before procreation; and . . . syphilis that may be transmitted to the fetus
. . . after procreation may not be considered as hereditary” (p. 10, emphasis in
original). Fournier devotes a chapter each to “Maternal Heredity” and “Paternal
Heredity,” proving the validity of these mechanisms of transmission by reference

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Dr. Arthur Conan Doyle and the Case of Congenital Syphilis

to numerous clinical cases. So convinced is he of this that he allows for the pos-
sibility that a fetus infected by the father’s sperm might then infect the mother
by the transplacental route.
Fournier also includes a chapter on “Heredity in the Second Generation”
(Doyle’s third generation). He confesses to a belief in this possibility but con-
cedes that it is difficult to prove, since documentation for the grandparent is not
often available. But Fournier’s gravest problem is to explain how the infected
mother can give birth to normal as well as syphilitic offspring. Here is the best
proof that he envisioned the disease as becoming permanently incorporated into
the germline cells of the mother (and presumably also the father). So firm was
the belief in this mode of transmission of syphilis (especially among the French)
that Fournier’s son would later write an entire book devoted to the subject (E.
Fournier 1905).
Alfred Fournier wrote at great length to demonstrate that the overt signs of
disease in a congenitally infected individual might only appear years after birth
(Fournier 1886).Thus we should not be surprised that Doyle’s character Sir Fran-
cis Norton did not seek medical consultation for his newly appeared skin lesion
until age 21. (But one must wonder that no physician or dentist had noticed ear-
lier his “Hutchinson’s teeth,” supposedly pathognomonic for the disease.) In addi-
tion, Fournier (1880) wrote a book Syphilis et marriage, in which he advises
strongly against marriage for the active syphilitic until many years after remission,
because of the probability of transmitting the disease to both the spouse and off-
spring. Underscoring further his belief in the germline transmission of the dis-
ease, he repeats once again the possibility that a fetus infected through paternal
sperm might infect the mother during gestation, a process that he terms “syphilis
by conception,” quoting Jonathan Hutchinson (1856/57).

Jonathan Hutchinson (1828–1913)


Jonathan Hutchinson was one of the most distinguished medical leaders of
Britain during the late 19th and early 20th centuries.Already at age 35 he would
be recognized as an expert on syphilis. His book A Clinical Memoir on Certain
Diseases of the Eye and Ear, Consequent on Inherited Syphilis (1863) defined the
triad named for him and made clear that patients with “heredo-syphilis” might
develop inflammation of the eye as late as age 30. Hutchinson would go on to
become one of Britain’s foremost medical polymaths, becoming in turn Presi-
dent of the Hunterian Society, the Pathological Society, the Ophthalmological
Society, the Neurological Society, the Medical Society, and finally the Royal
College of Surgeons.
Hutchinson wrote and spoke widely on syphilis. His discussion of the pathol-
ogy of syphilis is oft-quoted and testifies to the complicated nature of the man-
ifestations of the disease in both its acquired adult and hereditary childhood
forms (Hutchinson 1876). But Hutchinson’s definitive work on the subject is his

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Arthur M. Silverstein and Christine Ruggere

monograph Syphilis, first published in 1877 and appearing thenceforth in many


new editions, including one published just one year before Doyle’s story
(Hutchinson 1893). Of interest here is Chapter 6, “On Congenital Syphilis and
the Laws of Inheritance.”
In outlining the modes of inheritance of the disease, Hutchinson (1893) fol-
lows Fournier closely (indeed he dedicated the monograph to Fournier):“It may
be that it is from the father alone, or from the mother alone, the poison being
in each instance present in the one parent at the time of their child’s concep-
tion. To these modes we may give the name of ‘conception inheritance,’ quali-
fied as ‘paternal’ or ‘maternal,’ or of ‘sperm inheritance’ and ‘germ inheritance’
respectively” (p. 65). Hutchinson then recognizes that “conception inheritance”
is not the only way that the fetus may contract syphilis. Whereas “sperm inher-
itance” is the only way that the father may transmit the disease, “The mother
may, however, be the means of communicating the disease to her offspring in
another manner. . . . and that she acquired the disease at some period of her preg-
nancy. In this case the foetus will receive the poison, not at the very starting
point of its existence, but after a period, of varying length, of healthy intrauter-
ine life” (p. 65). As Hutchinson concludes: “[There are] still some who doubt
whether it can inherit [congenital syphilis] from the father independently of the
mother.The evidence on this point seems to me overwhelming. It is a matter of
constant experience” (p. 67). He reinforces this view in Commentary CCIX:
“Thus it would appear that eleven conceptions have in succession been tainted,
the inheritance being from the father only” (p. 428).
Hutchinson clarifies one of the aspects of the transmission of syphilis from
parent to offspring that had puzzled Fournier. If, as Fournier and others thought,
the seed of the disease actually becomes integrated into the parents’ germ cells
(ovum or sperm)—the Lamarckian inheritance of an acquired characteristic—
how can some offspring suffer the disease while others of the same parents do
not? Here Hutchinson introduces the notions of the then-new bacteriology: the
specific “virus” of the disease passes into the sperm or germ cell, and thus “not
every child may ‘inherit’ the disease from either father or mother, but some may
be spared” (Hutchinson 1893, p. 71). This implies that the disease is not trans-
mitted genetically (to use a later term), but by an infected spermatozoon or
ovum. Hutchinson here uses Pasteur’s term virus (poison) to denote the patho-
genic organism, not to be identified as Treponema pallidum until 1905 (Schaudinn
and Hoffmann 1905).

Dictionnaire encyclopédique (1884)


The general view of “hereditary” syphilis in France of this period may perhaps
best be illustrated by the lengthy discussion in the 1884 edition of the Diction-
naire encyclopédique des sciences médicales (1884). The article on “Congenital and
hereditary syphilis” starts with reference to Paracelsus’ proposal that syphilis (le

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Dr. Arthur Conan Doyle and the Case of Congenital Syphilis

mal français) can pass directly from father to child. It then discusses in detail how
syphilis may be transmitted from the grandfather down through subsequent gen-
erations and that it goes from the father to the fetus without passing through the
mother. Here too the possibility is raised that the mother may be infected by the
fetus during gestation. In discussing a specific case, it is concluded that,“It is thus
quite certain that the impregnating sperm was the sole agent of the contamina-
tion; the wife remained innocent, the father transmitted the contamination”
(14:567).

Encyclopedia Britannica (1911)


Just as the general view of syphilis in France might best be seen in the Diction-
naire encyclopédique, so one would presume that the view in Britain might best be
summarized in the contemporary Encyclopaedia Britannica (1911). This view is
described in an article on “Venereal Diseases” written by Dr. Edmund Owen of
St. Mary’s and the Children’s Hospital, Great Ormond Street. Under the rubric
“Inherited Syphilis,” he writes:

there are the true specific effects [of syphilis] conveyed, along with all other in-
herited qualities, in the sperm-elements or in the ovum. . . . it is, in many cases
some months after birth before the congenital syphilitic effects show themselves,
whilst other effects come to light during childhood and youth. . . . During the
second dentition, three signs, as pointed out by Jonathan Hutchinson, may be
looked for—the notched incisor teeth of the upper jaw, interstitial corneitis and
syphilitic deafness. (14:985)

In a subsection on “Syphilis and Marriage,” we find:

The question as to how soon it would be safe for a person with secondary
syphilis to marry is of extreme importance, and the disregard of it may cause
lasting mental distress to the parent and permanent physical injury to the off-
spring. A man who finds himself to be the subject of secondary syphilis when he is
engaged to be married would do well honourably to free himself from responsibility.
(emphasis added)

The article concludes: “The transmission of syphilis to the third generation is


quite possible, but it is difficult of absolute proof because of the chance of there
having been intercurrent infection of the offspring of the second generation.”
All this as late as 1911! The author, who has already referred to Hutchinson
in the text, provides references to an English translation of Fournier’s Treatment
of Syphilis (1906) and to several other British books and journal articles dating
from 1907 to 1908.

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Arthur M. Silverstein and Christine Ruggere

“Index Catalogue of the Surgeon General,


U.S. Army”
The Index Catalogue of the Surgeon General might well be viewed as an objective
mirror of contemporary medical activity and thought, since it sought only to
present the world literature categorized and indexed for greatest ease of access,
using generally accepted terminology. Thus, any change in its nomenclature
should reflect current conceptual movement in a given field. It may be of inter-
est, therefore, to record the following differences found between the First Series
of 1893, and the Second Series of 1912. In 1893, there was a category “Syphilis
(Congenital, hereditary, and infantile)”; in 1912, the term hereditary was omitted.
In addition, in 1893 there was a category “Syphilis (Communication of) through
foetus or semen.”This rubric was dropped in 1912. It would seem reasonable to
conclude that recent advances in the understanding of bacterial infections and of
genetics argued against the continued acceptance of the Lamarckian view of a
direct paternal and maternal transmission of an infectious disease.

Parental Transmission of Immunity:


Paul Ehrlich (1854–1915)
The field of syphilology was not the only one that entertained the notion of
direct transmission of an acquired characteristic from parent to offspring; the lit-
erature of the 19th century was rife with such suggestions. We shall cite here
only one case, that in the nascent field of immunology. Not only was the claim
clear-cut, but the disproof of that claim was striking.
Louis Pasteur’s studies in the 1870s and 1880s focused attention not only on
the germ theory of disease but also on how active immunity might be induced.
With the observation that children might manifest an “innate” immunity against
certain diseases in the absence of demonstrable external stimulus (infection or
immunization), it appeared reasonable to speculate that this might stem directly
from the parents. In Les virus, Saturnin Arloing (1891) wrote:

When the father is cured of a virulent infection, when he participates in the


act of insemination he may transmit a part of the immunity which he enjoys.
The male ovule . . . has been dynamically modified . . . it carries a vaccinating
substance which spreads in all of the cells of the embryo and fetus. (Arloing
1891, p. 285)

This theory of the transmission of immunity from parent to offspring precisely


parallels the contemporary concept of disease transmission.
In 1892, shortly after he entered the young field of immunology, the experi-
mentally innovative Paul Ehrlich decided to investigate the origin of the pro-
tective antibodies often found in the newborn. In an experimentum crucis, Ehrlich
bred mice immune to the toxins abrin or ricin to nonimmune mates—immune
males to nonimmune females and vice versa.The results were clear.The offspring

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Dr. Arthur Conan Doyle and the Case of Congenital Syphilis

of immune mothers and non-immune fathers were protected at birth, whereas


those derived from immune fathers and nonimmune mothers were born unpro-
tected (Ehrlich 1892; Silverstein 2000). Moreover, the offspring of nonimmune
parents derived from immune grandparents never showed evidence of immunity.
(That Ehrlich would even do this last experiment indicates the prevalence of the
idea of multi-generational transmission.) Ehrlich (1892) concluded that: “It is
not to be doubted that the immunity that we have observed in the offspring of
immune mothers . . . depends on the transfer of maternal antibody” (p. 202).
Because antibody titers in the newborn would often increase after birth, Ehr-
lich then designed his most difficult experiment. He gave the newborn of an im-
mune mouse to suckle to a nonimmune foster-mother, and the newborn of a
nonimmune mother to an immune foster-mother. Again the results were defin-
itive: it was the milk of the immune mother or wet nurse that carries protective
immunity. Ehrlich was even able to show that antibody passively introduced into
the blood of a nursing mother would soon be found in the blood of the suck-
ling infant, although like all passive antibodies these did not last long in the
recipient.
Here was proof positive, at least for protective immunity, that such an acquired
characteristic in a parent could not be incorporated into the germ cells to endow
the offspring with protection. But we have yet to find evidence that syphilolo-
gists, who supported a Lamarckian concept in their own field, were aware of
Ehrlich’s contrary immunological results.

Comment
It is impossible to overstate the role of syphilis in the Europe of the 19th cen-
tury. Its importance in contemporary medicine can be measured not only by the
fact that such medical leaders as Fournier and Hutchinson were syphilologists,
but also by the space allotted it in the 1893 Index Catalogue of the Surgeon General.
Syphilis occupies a surprising 113 pages, as compared with the 55 pages devoted
to the more prevalent and more dangerous tuberculosis. In the wider society, it
came to serve broadly as a metaphor for all that was low, vulgar, and unclean
(Corbin 1981; Hall 2001; Quétel 1986). There is thus no cause to wonder that
Doyle the doctor would write his thesis on the medical subject, nor that Doyle
the author would write a story on its moral implications.
The 16th-century view that syphilis could be transmitted directly from father
or mother to the offspring was still prevalent when Doyle studied medicine and
when he wrote “The Third Generation.” 3 This strongly suggests that the mode
of transmission from one generation to the next—father infecting mother, who
then infects the fetus transplacentally—attributed to Doyle by commentators

3 Even the legendary Principles and Practice of Medicine by William Osler (1892) followed Fournier
on the paternal and maternal transmission of syphilis.

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Arthur M. Silverstein and Christine Ruggere

Rodin and Key (1992) would have been an anachronism in the 1890s. It is far
more likely that when he wrote this sad tale, Doyle had in mind the then-preva-
lent Lamarckian concept that all types of acquired characteristics might be trans-
mitted directly from parent to offspring.This view finds support in Owen Dud-
ley Edwards’s deconstructionist biography of Doyle, in which he discusses
Doyle’s worry about what he might have inherited from his forebears: “some
ancestral taint working its way through posterity, in the form he describes so
tragically about the doomed grandson of the foul old rake in ‘The Third
Generation’” (p. 312).

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