infection, and does not conflict with the statistical fact expressed by
Hutchinson (Reynolds's _System of Medicine_, vol. i. p. 431) in his
words: "In the large proportion of cases met with in practice the taint
is derived from the father only." This numerical predominance of
paternal influence is very readily explained. There are many more
syphilitic men than syphilitic women, and especially among the couples
who contract fertile marriages the number of women who are infected
before becoming mothers is inconsiderable. On the other hand, it
frequently happens that men who have had syphilis, but have been
without symptoms for a longer or shorter interval, marry and transmit
to a series of children a disease which has ceased to be directly
contagious to their wives, the transmissive power continuing after the
possibility of ordinary contagion has disappeared. As in the majority
of such women the disease is latent, and may be only displayed in their
immunity from infection, it becomes evident that, history and symptoms
both being wanting on their part, the conditions justify the assertion
of Mr. Hutchinson. (See _Nouveau Dict. de Médecine et Chirurgie_, p.
684.)
That assertion (quoted above) has, however, been thought by several
writers to indicate his belief in the escape of the mother. That I have
not misinterpreted him is evident from the following extract from an
article on "The Transmission of Syphilis," written by him (_Brit. and
For. Med.-Chir. Rev._, Oct., 1877): "I take it for granted (although I
know that there are still some who doubt) that it is possible for a
father to transmit the taint, the mother being at the time of
conception wholly free. I believe, indeed, that in practice this is by
far the most common way in which syphilis is transmitted. Whether in
these cases it is correct to speak of the inheritance being paternal
only is, as we have just seen, another matter, since it is possible
that in every instance the mother derives an infection from the father,
and may thus in turn influence it."]
5. It is highly probable, though it can hardly be considered as proven,
that in all cases where a child becomes syphilitic through paternal
influence the mother is also the subject of syphilis, which may,
however, assume a latent form, the only evidence of its presence in a
few cases being the protection which it affords against contagion
through the medium of the child.
6. Syphilis may be transmitted from mother to child even when it is
acquired by the former as late as the seventh month of utero-gestation.
Since writing the above the thirty-fourth volume of the _Nouveau
Dictionnaire de Médecine et de Chirurgie_ has been published. In the
article on syphilis seventeen pages are devoted to the question of
heredity, which is reviewed in a most thorough manner and finally
summed up as follows (p. 698):
"The most definite views which we possess on the subject of the
hereditary transmission of syphilis may be thus expressed:
"Children may be infected by heredity, not only when the two parents
are syphilitic, but also when only one, either the father or mother, is
diseased at the time of conception.
"When both parents are diseased at that time there is more certainty
that the child will be infected, and infected gravely, than if only one
of them has the pox.
"The hereditary disease is not always fatal, even when both progenitors
have actual specific symptoms. The more recent the disease of the
parents the greater the chances of their transmitting the disease and
of its assuming {271} a serious form. There is no proof that inherited
syphilis is more grave when derived from the father than when coming
from the mother.
"It is altogether exceptional for the mother, healthy at the moment of
conception, not to participate in the disease transmitted by the father
to the child. If she escapes direct contagion--which is rare when the
disease of the father is active--she undergoes a species of infection
from contact with the contaminated foetus.
"Syphilis by conception, which is thus transmitted from the foetus to
the mother, may present the usual characters and evolutions of the
acquired disease; frequently, also, it is latent, and is betrayed only
by the existence of immunity from further contagion on the part of the
mother. It may finally manifest itself by tertiary symptoms or by
systemic troubles without specific characters.[51]
"The power of transmitting syphilis hereditarily decreases
spontaneously as the disease of the parent becomes older. The influence
of treatment is no less certain than that of time. When the two
progenitors are at the moment of conception free from syphilis, the
foetus may still be infected if the mother acquire the disease during
her pregnancy."
[Footnote 51: "This form of syphilis shows itself in the mother in
three ways:
"1st. By the usual signs of syphilis by contagion, with the exception
of the primary sore, appearing about the sixty-fifth day after
conception.
"2d. Appearing at a later date as secondary or even as tertiary
symptoms, and preceded merely by a little disturbance of the general
health, unhealthy appearance of skin, falling of hair, etc., but
nothing truly specific.
"3d. Showing itself some years afterward in a tertiary form, having in
the mean while given no indication of its existence save only in the
protection it afforded against contagion from the child" (_Nouveau
Dict. de Méd. et Chir._, vol. xxxiv. p. 696).]
* * * * *
Syphilis of the placenta is of especial interest in its relation to the
abortions and stillbirths so frequent in syphilis. Until the elaborate
paper of Fraenkel upon this subject (see foot-note, p. 265) almost
nothing was known about it. He describes[52] the macroscopic changes as
consisting of increased size and weight of the placenta, closer and
firmer texture of the placental tissue, the presence of old and recent
extravasations of blood in all stages, opacity and thickening of the
decidual covering and of the amnion and chorion, which are in places
adherent to each other. Microscopically, it was found that the
placental villi were filled with small nucleated cells, which were
especially abundant in the centre of the villous spaces along the axis
where the vessels usually take their course. The ends of the villi were
enlarged with knob-like processes.
[Footnote 52: I condense here from the translation of Fraenkel's paper,
which constitutes the bulk of chap. xxvii. of Bumstead and Taylor's
excellent work.]
Fraenkel's explanation of these changes is as follows: Under the
influence of syphilis[53] cell-proliferation begins in the villi, which
are, normally, only sparingly supplied with cells. These new cells
excite proliferation of the cells of the connective-tissue stroma and
of the epithelium. This proceeds to such an extent that it leads to
compression of the vessels,[54] interfering with the circulation, and
finally obliterates them. The {272} vascular spaces into which the
villi dip become filled up and narrowed, and often disappear. In this
way, and also by reason of the thickening of the epithelium, the
interchange between the maternal and foetal blood is interfered with,
and at last is prevented.
[Footnote 53: Fraenkel took as his criterion of syphilis the presence
of the osteo-chondritis described by Wegner (see p. 286).]
[Footnote 54: "Hennig was the first who called attention to the
intimate relation of the cell-growths to the vessels. The obliteration
or compression of numerous vessels of the villi interferes with the
mutual interchange of gases between the maternal and foetal blood,
causing fatty degeneration of the villi and, if the process is
extensive, the death of the foetus" (article "Syphilis," _Ziemssen's
Cyclopædia_, vol. iii. p. 237).]
If this process is spread over the whole placenta, the foetus perishes
before it is complete. If it is limited to circumscribed areas, it may
continue to live for a shorter or longer period.
These observations require to be repeated and confirmed, but they have
served to open up a most interesting branch of syphilitic pathology.
Syphilis in the parents will manifest itself in the children in one of
several ways, which are determined chiefly by two factors--viz. first,
the length of the interval between the infection of the parent and the
date of conception; and, second, the thoroughness of the treatment of
the parents during that interval. To these may be added as subsidiary,
but still of definite importance, a third, the type of disease which
has affected the father or mother, whether mild or severe, benign or
malignant.
From what has already been said in reference to the question of
marriage, it will be at once understood that the danger to the
offspring in untreated cases, and in those where conception has
occurred during the early secondary period of the disease, is of the
most extreme gravity.[55] In such cases the usual result of pregnancy
is abortion at from the first to the fifth or sixth month,[56] the
foetus sometimes exhibiting the evidences of the disease in the shape
of large bullæ upon the palms and soles, or in the presence of
characteristic visceral lesions, but quite as often showing nothing
distinctive. It has generally undergone more or less maceration, and
the skin, which is readily detachable, is of a congested, purplish
color.
[Footnote 55: According to Kassowitz, in women who are not treated all
pregnancies occurring within the first three years of their infection
terminate either in abortion or in the birth of children who survive
for only a few weeks or months.
Weber (quoted by Parrot, _Le Progrès Médicale_, Nov. 24, 1877, p. 882)
treated thirty-five pregnant women by mercurial inunction, and they all
went to full term under normal conditions. Among those treated with
mercury and iodide of potassium, but who, by reason of intolerance of
the former drug, took chiefly the latter, 20 per cent. aborted; when
the mixed treatment was carried out regularly 15 per cent. aborted; and
when only iodide of potassium was given 36 per cent. aborted. His
studies were made on 109 syphilitic pregnant women. Parrot himself says
(_ibid._) that "abortion occurs in about one-third of all syphilitic
women. Syphilis should always be suspected when this accident occurs
several times successively." "The date of abortion depends on the age
of the syphilis of the woman. It is most to be feared during the
evolution of secondary symptoms, particularly when they are grave.
There is more probability that it will occur when infection has
preceded pregnancy than when it occurs during its course. Nothing is so
much to be feared as contamination of the ovule. The nearer the date of
infection of the mother approaches to full term, the fewer the chances
of abortion. It is not likely to occur even when the mother is infected
at five months."]
[Footnote 56: Many observers think that abortion results directly from
the death of the foetus. Babington (notes to Hunter's _Treatise on
Venereal_), Trousseau, and Von Baerensprung were of this opinion.]
Dr. Cory thinks that in many cases it is possible that the effect of
syphilis may be to effect so early an abortion that the case is simply
regarded as one of delayed menstruation or of menorrhagia. Such a
conception would, however, be competent to infect the mother, and might
seem to explain cases otherwise involved in obscurity.[57] At least
one-third {273} of all syphilitic children are dead born.[58] As time
goes on, however, and the intensity of the parental disease is
lessened, or in cases where appropriate treatment has been applied,
either the abortion occurs at a later period of pregnancy or the
children are brought alive into the world. Even then, however, and
although at birth they may show no evidences of the disease, their
chance of escape is but small. One-fourth of them die within the first
six months. If they survive that period, the chances for life are
slightly in their favor, but those for health or freedom from deformity
and disease are still overwhelmingly against them.
[Footnote 57: As, for instance, when a woman married to a syphilitic
man, but without issue, remarries a man with no history of syphilis,
and yet gives birth to a syphilitic child. In such a case there would
be no history of direct infection and none of pregnancy, the only two
modes by which she could have contracted the disease, and the
father--the second husband--might be unjustly suspected.]
[Footnote 58: Kassowitz, _op. cit._]
The course of inherited syphilis differs strikingly from that of the
acquired disease. It will hardly be necessary to do more than remind
the reader of the ordinary stages of the latter affection--the primary,
which includes the period of the chancre and of lymphatic engorgement,
lasting about sixty to seventy days; the secondary, or exanthematic,
characterized by copious cutaneous eruptions and extensive involvement
of mucous surfaces, lasting from one to three years; the
intermediate,[59] or the stage of latency and relapses, lasting for a
very variable period, from three to ten years, but under proper
treatment very much reduced or altogether abolished; and finally, the
tertiary period, beginning four or five years after contagion,
extending indefinitely throughout life, but often in cases properly
treated absent altogether.
[Footnote 59: So designated by Mr. Hutchinson, and for clinical
purposes a very valuable addition to the periods of syphilis. He
describes it as follows: The patient may be either wholly free from
symptoms and in good health, or he may remain pale and rather feeble,
and liable from time to time to slight returns of eruption on the skin,
sores on the mucous membranes, condylomata, etc. He is protected as
regards fresh contagion, and should he beget children they are almost
certain to suffer. The relapses during this stage are usually easy to
be distinguished from true secondary symptoms. There is little or no
febrile disturbance, the rash is not copious, and often not
symmetrical. Acute iritis, retinitis, etc. never occur for the first
time, though they may do so in the form of relapses.]
For purposes of description and of contrast we may similarly divide the
whole period of evolution of a case of inherited syphilis,[60] omitting
the primary stage, which has never been found to exist in true cases of
hereditary syphilis. Of course in congenital or infantile syphilis, in
which by direct contagion, either from the mother or from any one else,
the disease was acquired by the child, the course would not differ
materially from that observed in the adult. But as this stage in all
probability corresponds to the period during which the poison is
already finding its way into the system through the lymphatics, of
course it is not found in the child who is infected from the moment of
conception or who receives the poison from the mother directly into the
circulation.[61] For from one to three weeks the infants often show no
symptoms of the disease. In 158 cases collected by Diday, 86 manifested
symptoms of the disease before the expiration of the first month, and
60 of the remainder before the end of the third month.[62] When to
these are added the {274} statistics of Roger, we find that of a total
of 172 cases, 159 showed syphilitic symptoms before the end of the
third month.[63] When the symptoms are present at birth, they consist
largely in a general withered, atrophied, weazened appearance of the
child; a hoarse cry, due to swelling, with subacute inflammation, or
even ulceration, of the laryngeal mucous membrane; a coryza,[64] due to
a similar condition of the Schneiderian membrane; and certain cutaneous
eruptions, the most common of which at this early date is the large
vesicular or bullous eruption known as syphilitic pemphigus.
[Footnote 60: The idea that the character of the symptoms which first
appeared depended upon and corresponded with the stage of the disease
in the parent has now, I believe, no supporters. It was once thought,
at least by some syphilographers, that if the parents were in the
tertiary stage at the time of conception the child would develop
tertiary symptoms, omitting the other stages.]
[Footnote 61: If chancre were the first symptom of constitutional
syphilis, why should it not appear in cases of hereditary syphilis?]
[Footnote 62: _Infantile Syphilis_, p. 101]
[Footnote 63: About 1823, from 16,000 to 17,000 children were admitted
annually to the wards devoted to foundlings at Lyons. Clièt, recording
his experience with this disease, says that syphilis is one of the most
common of their maladies, but that "it exceedingly seldom shows itself
at birth by evident signs" (_Compte-Rendu Méd. Chir. des Observations à
l'Hôpital général de la Charité de Lyon_, 1823). Cristoferi, physician
to the foundling hospital at Bologna, says that syphilis generally
manifests itself between the ages of one and three months. Never once,
he states, was a newly-born infant admitted with the disease
unquestionably developed (_Gazette Medica di Milano_, 1844). Trousseau
says that it "rarely appears before the second week, and very
exceptionally after the eighth month" (_Lectures on Clin. Med._, vol.
iv. p. 331, London, 1871, ed. of New Sydenham Society).]
[Footnote 64: Usually appears later, but exceptionally at birth.]
Pemphigus.--With regard to the specific or non-specific character of
this eruption there has been much difference of opinion, and, as it is
often the earliest distinctive expression of syphilis, a diagnosis of
which could hardly be founded on the general appearance of the child,
or even on the hoarse cry and the coryza, it becomes important to have
definite ideas upon the subject. Nearly a century ago (in 1794) it was
denied[65] that this eruption was a manifestation of venereal disease;
and this view has been supported by many able and accurate observers
down to the present day. In 1851 a discussion upon the subject took
place in the French Academy of Medicine, which elicited the opinions of
the majority of those members who were entitled to speak with authority
in the matter, and which has since been referred to by most writers.
Cazeaux upheld the non-syphilitic hypothesis on the ground (1st) that
the so-called syphilitic pemphigus of children does not differ from the
simple pemphigus of adults, presenting none of the physical characters
which distinguish the specific cutaneous eruptions; (2d) that it
appears at birth or immediately after, while the symptoms of hereditary
syphilis generally show themselves later;[66] and (3d) that at that
time there had been seen no cases of pemphigus at the Lourcine
Hospital, where so many syphilitic children were born.[67] In this view
he was supported by Trousseau, Lasègue, Gibert, Bazin, and other
obstetricians and syphilographers of note.[68]
[Footnote 65: Ariander, _Mémoires de Méd. et d'Accouch._, quoted by
Diday.]
[Footnote 66: This is now known to be an unreliable distinction,
expressing perhaps a general rule, but one with so many exceptions as
to render it void of diagnostic significance.]
[Footnote 67: This may have been true at that time, but has certainly
not continued to be so. Cornil says: "We often see at the Lourcine
children born prematurely or at the full term with pemphigus, either
fully developed at the moment of birth or appearing a few days
afterward, and who commonly die with syphilitic cachexia, the sad
heritage derived from their maternal parents."]
[Footnote 68: Pemphigus may indeed be a specific affection, but no
characteristic sign has been discovered sufficient to distinguish it
from the ordinary form of pemphigus. "On the other hand, there is no
inconsistency in admitting that syphilis, which so deeply impairs the
constitution of the parent, may act like any other common cause and
excite non-specific pemphigus; for an infant is badly lodged and poorly
nourished in the womb of an enfeebled mother, apart from the influence
of the virus" (Ricord, note to _John Hunter's Works_, 1853).]
On the other hand, Dubois claimed a specific character for the
affection {275} on the ground (1st) of the fact that in the majority of
cases there was a syphilitic history in the parents; and (2d) that the
eruption often coexists with well-known syphilitic lesions. This was
supported by Cazenave, Danyan, Bouchut, Vidal, Ollivier, and
others.[69] Diday, who devotes several pages of his interesting work on
_Infantile Syphilis_ to this subject, regards the eruption as simply a
manifestation of a cachexia produced by syphilis,[70] founding this
opinion on (1st) the absence of specific characters in the eruption;
and (2d) that syphilitic pemphigus is a rare affection in the adult, if
it occurs at all, so that to recognize it in the child would be to make
a single exception to the general rule that "all the syphilitic
eruptions of new-born children have their equivalents in those of
adults." He explained the two cases which were then (1858) recorded of
cures of pemphigus by mercury[71] by saying that it was the treatment
of the diathesis, not of the disease, which caused the improvement. He
acknowledges, however, the very frequent association of pemphigus in
the child with syphilis in the parent, and says that it springs from
the latter affection, "specially, but not specifically"--a rather
wire-drawn distinction.[72]
[Footnote 69: Jullien (_op. cit._, p. 1005), after considering the
opposing views as to the character of this eruption, says: "We have no
hesitation in declaring ourselves in accord with Roger, Ollivier,
Ranvier, Parrot, and others, and in distinctly separating from the
specific affection the rare eruption known as simple pemphigus,
sometimes epidemic, occasionally febrile, and appearing most frequently
about three months after birth. We consider likewise that an evidence
of congenital syphilis which is by no means doubtful is found in the
bullous eruption seen at birth or within the first two weeks,
comparatively frequent, and involving by preference the palms and
soles. This opinion is based upon (1st) its appearance in children
whose parents are known to be syphilitic; (2d) its association with
syphilitic lesions of the lungs, liver, kidneys, thymus gland, etc.;
(3d) its partial disappearance under mercurial treatment, and its
reappearance when that treatment is discontinued."]
[Footnote 70: _Op. cit._, pp. 70-77.]
[Footnote 71: Depaul, _Gaz. Méd. de Paris_, 1851, p. 472, and Galligo,
_Gaz. Med. Toscana_, 1852, p. 123.]
[Footnote 72: Trousseau (_Clinical Lecture on Syphilis in Infants_),
after detailing a case in which there was some doubt as to the
existence of hereditary syphilis in a child born alive, and in which
case the previous pregnancy had resulted in a stillborn child at seven
months, the body of the latter having been preserved in alcohol and
exhibiting numerous traces of pemphigus, says: "So far as I was
concerned, this demonstration did not amount to more than the
establishing of a probability, and several physicians who participated
in this indecision finally accepted a compromise. They considered that
maternal syphilis had determined a sort of cachexia in the foetus which
had led to an eruption of bullæ which was not specific. By accepting
this too-facile hypothesis you will imprudently open a door which you
will with difficulty be able to close."]
As these differences of opinion have been perpetuated to the present
day, it has seemed to me proper to make this reference to their
history, although I am strongly convinced that the progress of clinical
and pathological knowledge enables us now to assert that although, as
an exception, bullæ may be due to a profound cachexia not dependent on
syphilis, yet that in the large majority of cases they are specific in
their character.