of a gummy deposit, but that seems to be entirely hypothetical, none
having been discovered. Weisflag (quoted by Bumstead) arrives at the
following conclusions after studying the lesion and the literature of
the subject: 1. This thymus abscess does exist. 2. When associated with
other signs of congenital syphilis it indicates that the father or
mother of an infant suffers or has suffered from syphilis. 3. It is
possible, but not proved, that this affection may exist in children in
whom there are no symptoms of syphilis, but its existence renders the
diagnosis of hereditary syphilis probable, even if the disease of the
parent is not proved. 4. Such is the great similarity in the appearance
of pus and of the secretion of the thymus that they cannot always be
distinguished.]
THE DIAGNOSIS AND PROGNOSIS OF INHERITED SYPHILIS.--In reviewing the
general course of a case of inherited syphilis it seems evident that
the differences between it and the acquired disease which have been so
much dwelt upon are apparent rather than real.[207] The primary stage
is of course missing, and on any theory of the essential nature of
syphilis this is readily comprehensible. Whether the chancre is the
first symptom of a constitutional disease, or, as I believe to be the
case, is the simple accumulation at the point of original inoculation
of the cells which constitute the syphilitic virus--or are at any rate
its carriers--it would naturally be in the first case undiscoverable,
in the second nonexistent.
[Footnote 207: "That the noteworthy differences between
chancre-syphilis and the inherited disease are to be interpreted by
considerations of the tissues of the growing child and the adult, is
made very probable by what is observed when a mother near the end of
pregnancy becomes infected with primary disease. In such a case the
foetus nearly full grown acquires the disease, without a chancre,
directly from the maternal blood. It is acquisition, not inheritance,
for at the date of conception both the paternal and maternal elements
were free from taint, and during the first six, seven, or even eight
months of intra-uterine life the foetus remained healthy. Yet, as I
have proved elsewhere by citation of cases, syphilis obtained in this
peculiar method resembles exactly that which comes by true inheritance,
and not that which follows a chancre. This important fact goes, with
many others, in support of the belief that the poison of syphilis
remains identical, however obtained, and that the differences which are
so patent in its manifestations are due to differences in the state of
its recipient" (Mr. Hutchinson, article on "Transmission of Syphilis,"
_Brit. and For. Med.-Chir. Rev._, Oct., 1877, p. 475).
"It is not true that the diversity of symptoms presented by infants
authorizes us to admit a congenital and an hereditary syphilis.
Whatever the mode of infection, it is impossible to make this
distinction" (Ricord, note to _John Hunter's Works_, 1883).]
The secondary stage, characterized in the acquired form chiefly by
{310} lymphatic engorgement and symmetrical, widely-spread, polymorphic
cutaneous and mucous eruptions, and pathologically by a marked tendency
to the proliferation of certain new small round nucleated cells, upon
the presence of which depend all the manifestations of the disease, is
in inherited syphilis strictly analogous. Eruptions of the same
character make their appearance, differing only in minor points, as in
a greater tendency to become moist or ulcerated, due to the more
delicate texture of the infantile epidermis. To the same cause must be
assigned the macroscopic peculiarities of the only syphiloderm said to
be peculiar to infantile syphilis--pemphigus--which has been shown,
however, to have a papular basis, and in that way to conform to all the
other secondary eruptions.
The lymphatic engorgement either exists in the infant as in the adult
or has its analogue in the enlargement of the spleen and
liver--especially the former, which is almost as constant a phenomenon
as is general glandular enlargement in acquired syphilis. The same
pathological changes occur, the same infiltration of cells producing,
according to their situation, papular, pustular, or mucous patches, or
inflammation of such structures as the iris, choroid, or retina.
The tertiary stage, except in the fact that its phenomena may appear
unusually early and may be commingled with those of the secondary
period,[208] does not widely differ in the hereditary from that of the
acquired disease. It affects the same tissues, results in the same
pathological formations, and is preceded by the same period of latency
or quiescence of variable duration. There is no reliable evidence with
which I am familiar to show that in this stage inherited syphilis is
either contagious or transmissible--another point of close resemblance
between the two varieties under consideration.
[Footnote 208: This is by no means unknown even in the acquired form;
frequent examples of it have been recorded, and it can be readily
explained either on the theory of relapses in parts previously diseased
(Hutchinson), or on that of obliteration of lymphatic trunks and
accumulation of nutritive waste (Otis).]
In considering the question of diagnosis, therefore, we have an
excellent guide in the fact that the disease conforms in most respects
to the general laws of acquired syphilis, and that our knowledge of the
latter affection will be a valuable aid to recognition of the former.
The chief elements of diagnosis and prognosis of inherited syphilis in
its various stages may then be summarized as follows:
A history of syphilis in either parent is important just in proportion
to the shortness of the interval between the time of infection and the
date of conception. In other words, the shorter that interval the more
likely (_a_) that the child will have syphilis, (_b_) that it will have
it in a severe or fatal form. If the mother has been syphilitic and the
father healthy--which is rare--it is perhaps more likely that the child
will be diseased than when the reverse is the case. If both parents
were syphilitic at or before the time of conception, the probability
that the disease will be transmitted, and in a severe form, is much
increased. There is no evidence to show that inheritance from one
parent results in a graver variety of the disease than when it is
derived from the other.
A history of abortion or miscarriage on the part of the mother should
have weight in the determination of any given case, and if such
accidents {311} have been very frequent their diagnostic importance is
greatly increased. The loss of elder brothers or sisters and the causes
of death, with the precedent symptoms, should be carefully inquired
into. The nearer either of these occurrences--abortion or death of
elder children, if there is a fair presumption that they were due to
syphilis--has been to the birth of the patient in question, the greater
the likelihood that the latter has been infected.
Upon examining the product of abortion or stillbirth the most easily
observable symptoms will be those of the skin. Maceration and elevation
of the epidermis into bullæ are in themselves hardly characteristic,
though they may--especially the latter--be regarded as suspicious. If
the cutaneous lesions are, however, distinctly papular or pustular or
ulcerative, or if the bullæ have all the characteristics of syphilitic
pemphigus, the diagnosis is assured.[209]
[Footnote 209: "It is probable that very early abortions are less rare
than statistics indicate, but are often unsuspected."
"It is impossible to demonstrate the existence of syphilitic lesions in
foetuses expelled during the first months of pregnancy. Later, the
signs which have the greatest value are the lesions of the epiphyses of
the long bones. When the foetus has nearly arrived at full term, and is
not macerated, visceral and cutaneous lesions may be observed.
According to Mewis, the skin eruptions cannot be seen before the eighth
month, and are only recognizable on foetuses whose death has been very
recent or who are born living. Pulmonary lesions may be determined at
the end of the sixth month. Those of the pancreas are met with in about
half the foetuses which perish a little before or a little after birth.
The lesions of the liver, the spleen, and the bones may be recognized
even in macerated foetuses, this frequency increasing from month to
month" (_Nouv. Dict. de Méd. et Chir._, vol. xxxiv. p. 864).]
The most distinctive symptom--one which may really be considered as
pathognomonic, is, however, the inflammation of the diaphyso-epiphysial
articulations, with or without their disjunction. Distinct enlargement
of the spleen or liver, and arachnitis with hydrocephalus, are valuable
diagnostic points, and the presence of gummata--not very
infrequent--would of course be conclusive.
At birth the syphilitic child may be small, stunted, emaciated,
weazened, senile in appearance; this would properly give rise to
suspicion, but may be associated with any disorder of nutrition on the
part of child or mother. It may also disclose cutaneous or mucous
eruptions evidently specific in character. The most common of these at
this early date is the bullous eruption affecting the palms and soles,
sometimes distributed over the whole body, and, as it indicates a
feeble resistance of the tissues to the tendency to exudation and
cell-growth, is usually a precursor of an early and fatal termination.
In any event, marked symptoms at time of birth render the prognosis
highly unfavorable.
It is quite as common, however--perhaps more so--for the subject of
hereditary syphilis to give no evidence of the disease at birth, but
even to appear healthy and well-nourished. In such cases the first
symptoms of the disease appear, on an average, in from six weeks to two
or three months, and consist principally of coryza (snuffles),
hoarseness of voice, and syphilodermata. The latter may be macular,
papular, pustular, or bullous. They are usually polymorphous, irregular
in shape, dark coppery-red in color, with sometimes a glazed or
crusted, but oftener a moist or ulcerating, surface, with a strong
tendency to coalesce into large patches, or to form irregular
serpiginous ulcers, or to take on hypertrophic growth {312} and develop
into condylomata. Eruptions which are squamous and are situated about
the mouth and chin and on the body, the legs, or the soles of the feet,
though exceptional, are of more value than those on the nates, where
the results of irritation from urine and feces may closely simulate
syphilodermata.
Mucous patches on the tongue, cheeks, tonsils, and pharynx are common,
often extending to the larynx, increasing the hoarseness, and to the
nasal cavities, aggravating the snuffles. Both of these occurrences, by
interfering with the respiration of the child and rendering its nursing
interrupted and insufficient, greatly add to the gravity of the case.
Enlargement of the spleen (common), enlargement of the liver (less so),
and iritis (rare), may be mentioned among the phenomena of this stage,
often associated with the skin eruptions.
About the time of the subsidence of the rash there may be developed the
specific inflammation at the junction of epiphyses and diaphyses which
produces a swelling of the long bones near their ends. The child will
be noticed to cry a little when, for example, the wrist or elbow on one
side is washed, and not to use these joints as much as the
corresponding ones on the other side. The parts are not hot, only
slightly tender, and as yet there is but little swelling. Later, the
droop and the disuse of the affected limb become more noticeable and
simulate infantile paralysis. There is, however, no wasting, no
alteration of reaction by faradism, no real loss of power, so that the
term pseudo-paralysis is an appropriate one. In a week or two similar
symptoms will occur in the bone on the opposite side, and finally the
ends of all the long bones may be affected; ordinarily the elbows,
wrists, knees, and shoulders are the joints involved. Suppuration is
rare, disjunction of the epiphysis from the diaphysis common. Recovery
is apt to take place spontaneously within a month. The associated
changes are chiefly endosteal at the junction of the shaft with the
epiphysis, but there is also a little periostitis or perichondritis,
which is the principal cause of the external swelling. Moderate
deformity may ensue.[210]
[Footnote 210: For the diagnosis from rickets see p. 290.]
Similar changes occurring in the cranial bones give rise to what has
been called the natiform skull. During the first year it is very common
for syphilitic children to develop a number of lenticular swellings on
the cranium, which appear symmetrically around the anterior fontanel,
but at a little distance from it; _i.e._ one on each frontal and one on
each parietal bone. These are said to be "bossed." They are at first
circumscribed, and in a child nine or ten months old often measure
three-quarters of an inch to an inch in diameter. They are at first
circular, afterward more irregular, and finally tend to organize,
becoming diffused and massive and causing a permanent thickening of the
skull.
These symptoms which have been described are the prominent ones
occurring during the first six or eight or twelve months of life. If
they do not manifest themselves before the eighth month, it is highly
probable, even in a case with a syphilitic parental history, that the
child will either escape altogether or that the secondary stage has
been very slight and altogether intra-uterine and unattended with
noticeable phenomena. If during this first year the child's cachexia is
marked, if there are any intercurrent diseases, if the symptoms show
themselves early, if the nasal or laryngeal affection is severe, if the
eruptions are markedly bullar or {313} pustular or ulcerative, if the
enlargement of the spleen is great or the osseous lesions precocious or
grave, and if, especially, there is any intermingling of tertiary
symptoms, gummata, nodes, etc.,--the prognosis will be unfavorable.
From adolescence on through adult life the diagnosis of inherited
syphilis will depend on the following points: First, of course, the
history of parental or of infantile syphilis, or of both. Then a group
of physical and physiognomical peculiarities, which are not definitely
characteristic, and are of little value when taken separately, but are
of considerable importance when all or a majority are present in any
given case. These are low stature or puny development proportionate to
the severity of the intra-uterine and infantile symptoms; a pasty,
leaden, or earthy complexion,[211] a relic of previous syphilodermata,
probably also a result of malnutrition; a prominent forehead, bulging
in the middle line at and within the frontal eminence, and due either
to thickening of the skull or to a previous arachnitis and
hydrocephalus before the ossification of the fontanels; a flat, sunken
bridge to the nose, due to the coryza of infancy extending to the
periosteum of the delicate nasal bones, and either interfering with
their nutrition or partially destroying them; dryness and thinness of
the hair, with brittleness and splitting of the nails; synechiæ and
dulness of the iris (rare); ulcerations of the hard palate;[212] and
periosteal thickenings or enlargements of the shafts of the long bones
near the ends, or slight angular deformity, results of the
osteo-chondritis of infancy.
[Footnote 211: Trousseau (_Clinical Lectures_, vol. ii. p. 588,
Philada., 1873), after calling attention to this peculiar hue of the
face, says: "It not unfrequently happens that the physician, taught by
long familiarity with this appearance, will almost at once diagnose
syphilis after having simply seen the child's face, although the
peculiar hue can be but vaguely described in words. The visage presents
a special shade of bistre; it looks as if it had been lightly smeared
with coffee-grounds or a very dilute aqueous solution of soot. There is
neither the pallor, the icteric hue, nor the straw-yellow tinge of skin
seen in other cachectic affections; the tinge is not nearly so deep,
but is almost like that of the countenance of a recently-delivered
woman, and either does not extend at all, or only partially, to the
rest of the body. I know no disease except syphilis in which a child's
skin has this peculiar color; and consequently, when it is well marked,
it has more diagnostic value than any other symptom."]
[Footnote 212: Mr. Oakley Coles reports (_Proc. of Path. Society of
Great Britain_, vol. vii. p. 5) several cases of inherited syphilis in
which there was wide separation of the jaws in the median line. In one
family one member had typical teeth and wide separation; three others
had the same separation, but not the characteristic teeth. It was
suggested that in such cases the teeth were in size far below the
average, and that the condition was that often observed where the jaws
are in development in excess of the teeth which they contain. I. E.
Atkinson details some interesting cases of this lesion in late
hereditary syphilis, and attributes to it considerable diagnostic
importance (_American Journal of the Medical Sciences_, New Series,
vol. lxxvii., Jan., 1879, p. 71).]
A much more valuable group of symptoms, however, are the following,
which are mentioned in the order of their importance, any one of the
first three being almost or quite conclusive:
Dwarfed permanent median upper incisors, broader at the top than at the
cutting edge, which is crescentically notched, separated by an undue
interval and converging toward each other.
Evidence of past or present interstitial keratitis--a dusky and thin
sclerotic in the ciliary region and slight clouds here and there in the
corneal substance, there being no scars on its surface--or of
disseminated choroiditis; patches of absorption especially around the
periphery.
{314} A radiating series of narrow cicatricial scars extending right
across the mucous membrane of the lips, or a network of linear
cicatrices on the upper lip and around the nostrils, as well as at the
corners of the mouth and on the lower lip.
Periosteal nodes on one or many of the long bones; sudden, symmetrical,
and complete deafness, without otorrhoea and unattended by pain or
other subjective symptoms.[213]
[Footnote 213: In a few instances there has been noticed an arrest of
sexual development; in one case of Hughlings Jackson's there was such
an entire absence of all sexual characteristics that it was supposed
that the ovaries had been destroyed by syphilitic inflammation in early
life.]
Late or tardy hereditary syphilis is rarely dangerous to life. The
prognosis is almost unvaryingly favorable unless some grave visceral
complication, such as interstitial pneumonia, gummata of the brain,
liver, or kidney, or meningeal and periosteal inflammation within the
cranium, should occur.
TREATMENT.--The prophylactic treatment, or that directed to the health
and sexual relations of the parents previous to conception, has already
been sufficiently considered. That of the mother during pregnancy,
after having conceived from a syphilitic husband, or having had
antecedent syphilis, or having contracted it by direct contagion
subsequent to impregnation, is simply that of acquired syphilis in
either adult or child. Mercury in its full physiological dose is the
drug indicated. It may not be amiss to combine with it iodide of
potassium in moderate doses, but the practice of employing the latter
to the exclusion of the former is both theoretically and clinically
unsound. Care should especially be taken to give it in such a manner,
either by inunction or vaporization or so guarded with opium, that it
will not produce any irritating effect on the intestinal canal, the
sympathy between which and the uterus may, in the event of a strong
purgative action being set up, lead to an abortion.[214]
[Footnote 214: "In respect to prophylaxis as applied to infants, all
chances of infection should be entirely removed whenever constitutional
symptoms exist or the nature of the primary symptoms renders them
probable. Our caution should be carried still farther, and in the
absence of all appreciable symptoms we should assure ourselves by the
antecedents, so far as possible, that the parents are not under the
influence of a syphilitic diathesis; in which case they may give birth
to infected infants until appropriate treatment shields the latter from
infection. With still stronger reasons, when the mother during
pregnancy is affected with primary syphilitic symptoms of such a
character as to give rise to secondary symptoms, or if the latter
already exist, we should hasten to cope with them, and, far from
regarding pregnancy as a contra-indication to treatment, should
recollect that it generally prevents the disease in the infant, and
when skilfully administered obviates the frequent abortions which
syphilis excites. When primary symptoms have been contracted by the
mother a short time before delivery, since the infant may be infected
in its passage into the world, the same course should be followed with
it as with a person who has just exposed himself to an impure
connection" (Ricord, note on prophylaxis of venereal disease appended
to his edition of John Hunter's _Treatise on Venereal_, Philada., 1853,
p. 481).]
As we have seen that the pathology, the stages, and the general course
of hereditary syphilis are all closely related to or identical with the
same phenomena in the acquired disease, and so know that they both
depend upon the same ultimate cause, whatever that may be--a virus, a
fungus, or a degraded cell--it follows that the same principles should
govern us in the treatment of the one as in that of the other.
We know from clinical experience that mercury exercises an almost {315}
controlling influence over the secondary manifestations of acquired
syphilis, whether by acting as a true antidote or as a tonic, or by
virtue of its property of hastening destructive metamorphosis and
thereby facilitating the absorption or elimination of new cell-growths.
We know also that iodide of potassium, probably by virtue of its
powerful stimulating influence on the lymphatic system, has an equal
power over the tertiary growths, which by their pressure upon or
situation in important tissues or organs may be so destructive. There
is no reason, therefore, by analogy why these drugs should not,
comparatively speaking, be equally beneficial in hereditary syphilis;
and such is, indeed, found to be the case. In the latter affection,
however, there are two elements which should modify the treatment
somewhat, and must be taken into consideration. These are--1st. The
existence of a more or less profound cachexia influencing all the
nutritive and formative processes, and in itself, entirely apart from
any definite specific involvement of vital organs, threatening life.
2d. The not infrequent occurrence during the secondary period of
symptoms--notably gummata--belonging to the tertiary stage.