similar eruption is almost--or quite--unknown in the adult, has been
removed by the observations of Cornil, who has shown that it belongs
properly with the papular rather than with the bullous eruptions, and
should be classed with the roseola and papules of early syphilis--just
where, from its clinical history, we should expect to find it. The
raising of the epidermic layers is due chiefly to their delicacy, their
slight resistance, and their previous immersion in the amniotic
fluid--_i.e._ to {276} conditions which are peculiar to the skin
shortly after birth.[73] He founds these very important opinions upon
the autopsy of a child stillborn a little before full term, the mother
being in the height of secondary syphilis. The child presented
characteristic bullæ on the soles and palms. After hardening these were
found to consist of the two layers of epidermis placed one above the
other. Fig. 6 represents a bulla about one centimeter in diameter which
was situated on the plantar surface of the great toe.
[Footnote 73: Cornil, _op. cit._, p. 203.]
[Illustration: FIG. 6. Pemphigus bulla from a new-born syphilitic
child. The superficial epidermic layer _e_ is elevated by a fluid
exuded between it and the rete mucosum. The rete mucosum, _c_, is also
partly raised, so that there exists a space filled with fluid between
it and the papillæ, _p_. The epithelial prolongations and the ducts of
the sudorific glands _m_, placed between the papillæ, and which run
between them into the derm, are broken and suspended from the rete
mucosum. _d_. Derm. _a_. Fibrous and muscular layers. _t_. Tendons and
fibrous tissue. _o_. Cartilage of ossification of the first phalanx.
_v_. Vessels. X 8.]
[Illustration: FIG. 7. Section of the rete mucosum and papillæ from the
same case of pemphigus as Fig. 6. _o_. Orifice of a sudorific gland.
_m_. Cells of the rete mucosum, some of which are excavated, _c_. _p_.
Papillæ. _v_. Their vessels. _n_. Prolongations of the rete mucosum
between the papillæ. X 200.]
If, then, we find an infant at birth or immediately after[74]
presenting on the soles, the palms, the fingers and toes, or on the
limbs, an eruption consisting of blebs more or less perfectly distended
with a liquid which may be clear, cloudy, or bloody, circular or oval
in shape, sometimes irregular, seated on inflamed, reddish skin, and
surrounded by trifling areolæ, we may strongly suspect the presence of
syphilis in an active and most menacing form. And this suspicion
becomes a certainty if, in combination with such an eruption, the
general cutaneous surface is yellowish or muddy in hue, is hard, dry,
wrinkled, without elasticity or softness--owing to the absence of
subcutaneous fat--and, for the same reason, is furrowed and wrinkled
about the face, imparting an appearance of senility; if the child has a
hoarse cry, a discharge from the {277} nostrils; and, of course, if
there are at the same time other and unmistakable syphilodermata. This
eruption is specially important, however, because upon the recognition
of its specific character in cases of stillbirth, or in those in which
the child survives only a few days--not long enough for the development
of further symptoms--will depend the opinion as to the cause of death,
which, whether expressed or not, will determine the future treatment of
both parents during the interval and of the mother during the next
pregnancy.
[Footnote 74: Non-syphilitic pemphigus is said to be never present at
birth, nor until the child has become considerably exhausted by wasting
from some defect of nutrition. It therefore does not appear until it is
several weeks old. It then attacks the trunk in preference to the palms
and soles.]
We may now consider the other symptoms of the secondary period in the
child.
Coryza is one of the most characteristic, and at the same time one of
the most important, of these in its influence on the health of the
child. It is due to the same condition of the mucous membrane lining
the nasal fossæ as manifests itself simultaneously or soon afterward on
the skin in the shape of erythema, roseola, or papules; in other words,
it is a hyperæmia with papillary infiltration. Now, on the skin this
condition, except in so far as it indicates the presence of a grave
constitutional disease, is of no special importance. In the nostrils of
a sucking infant, already debilitated and impoverished by the anæmia of
syphilis, and depending upon its nutrition for the continuance of the
miserable flickering life which was its original endowment, the same
condition assumes the gravest significance.
The excessive supply of blood to the parts induces a catarrhal
condition which shows itself in a thin, watery discharge, which, as the
child during sucking is compelled to breathe through the nose, is
rapidly dried into crusts. These become adherent, fill up and lessen
the channel for the passage of air, and in so doing add to the rapidity
and force of the respiration through the nose, and thus increase the
tendency to the deposit of these crusts. The peculiar nasal, noisy
respiration of the child has given the affection the popular name of
snuffles. As the child can no longer breathe, or can breathe only with
great difficulty, while sucking, it takes the breast only to drop it
again immediately on account of impending suffocation.[75] As the
disease progresses ulceration occurs beneath the crusts, and often
involves the entire thickness of the delicate mucous and periosteal
layers underlying the thin bones of the nose; perforation of these
bones results, sometimes with caries to such an extent as to cause an
entire loss of the nasal septum, with flattening of the nose--a symptom
comparable to one which sometimes occurs in the tertiary period of
adults, but produced, as we have seen, by other causes. In adults
syphilitic caries and necrosis are usually due to lesions seated
primarily in the osseous or subperiosteal tissues; in the child, at
least in this instance, these tissues are involved secondarily.
[Footnote 75: For an admirable description of the mechanism of this and
other symptoms of coryza see Diday, _op. cit._, pp. 78-83.]
Erythema, or roseola as it is differently called, is apt to present
itself about the second or third week[76] after birth. As in the adult,
it begins upon the abdomen in the form of little oval, circular, or
irregular spots, dull red in color and disappearing upon pressure.
Later the color becomes deeper, the eruption extends to the trunk and
limbs, and, as exudation and cell-proliferation succeed to simple
capillary stains, it {278} ceases to disappear when pressed upon. It is
often moist, owing to the thinness of the epidermis, sometimes
excoriated. Occasionally it is confluent, and covers large areas with
an almost unbroken sheet of deep-red color.
[Footnote 76: Bassereau gives an instance of its occurrence within
three days.]
The diagnosis in the early stage is often difficult on account of the
resemblance to the simple erythema of infancy. As the disease
progresses, however, maculæ form here and there; the cell-infiltration
involves the papillæ, several of which coalesce, forming flat papules;
the nutrition of the superficial layers of the epiderm is interfered
with, especially where it is thick, as on the palms and soles, and the
eruption in those regions becomes scaly, and then the diagnosis is not
difficult.
Papules and Mucous Patches.--In the ordinary evolution of the disease
the next manifestation is usually the development of papules upon the
general cutaneous surface and of mucous patches on the tongue, lips,
and cheeks--probably also on other mucous membranes not exposed to
examination. The papules are apt, for the reason already mentioned--the
thinness and moisture of the skin--to be of the broad, flat kind,
especially, as in the adult, in those regions where the elements of
warmth and friction are superadded to the moisture, as in the folds of
the skin about the genitalia, the neck, the flexures of the joints,
etc. They are then moist, covered with a grayish secretion or a thin
crust, and are in reality mucous patches. Occasionally they take on a
little hypertrophy and develop condylomatous excrescences which closely
resemble the simple acute condylomata of infants. In syphilis, however,
the growth springs from a previously existing papule, which is not apt
to be solitary, there being others in the neighborhood which will
probably establish the diagnosis. The syphilitic condylomata also have
a peculiar fetid discharge, resembling that of mucous patches and more
or less characteristic.[77]
[Footnote 77: Van Harlingen, article "Syphilis" in the _International
Encyclopædia of Surgery_, vol. ii. p. 560.]
Mucous patches in the infant are among the most important of the early
syphilitic lesions--not to the child itself, because they do not
materially affect its health, save in those exceptional instances where
they are accompanied by a marked degree of stomatitis, and thus
interfere with its nursing. Their importance is due to the fact that
they are almost constantly present, and they are thus by far the most
frequent vehicle of contagion from the child to its nurse or to others
with whom it may come in contact. At times they do not differ
materially from the same lesion occurring in the adult, but lose much
sooner their epithelial investment (on account of the delicacy and
comparatively slight attachment of the epithelium at this stage), and
they then appear as oval or irregular red, slightly depressed spots,
distinct or coalescing, ulcerating or oftener covered by a false
membrane. They especially affect the angles of the mouth and the sides
and dorsum of the tongue; and indeed their disposition to select the
former situation constitutes a diagnostic difference between them and
non-specific stomatitis which is to be found in the sulci between the
gums and cheeks and on the gums themselves--locations rarely invaded by
mucous patches.[78] When the latter are ulcerating or are concealed by
diphtheritic membrane, and are situated on the tongue, they may be
mistaken for either simple or parasitic stomatitis. The {279} diagnosis
can often be made by the presence of other syphilitic symptoms--coryza,
erythema, and especially papules. In their absence, however, it must be
remembered that in simple stomatitis, the inflammation not being
limited to special areas, the whole tongue is apt to be involved or a
much larger portion of the buccal mucous membrane; and as there is no
marked tendency to cell-proliferation in these cases, the accompanying
exudation is apt to be serous or watery and to result in
vesiculation--a condition never seen in syphilitic stomatitis. In the
parasitic disease, too, the inflammation is less localized, there is
more swelling and congestion, and the false membrane is said to be of a
whiter color.
[Footnote 78: Bumstead and Taylor, _op. cit._, p. 750.]
No child that has even been suspected of having a taint of hereditary
syphilis should be permitted to nurse at the breast of any one but the
mother, to share its cup or nursing-bottle with other children, to
receive the caresses of relatives or friends; and in this last
restriction we would include the father, even if the suggestion[79] be
true, that in the case of syphilitic children the protection from
contagion probably extends to the male as well as the female parent.
Paternity is sometimes a more doubtful problem than would seem
probable, and even if the father were protected the husband might not
be. The mucous patches, if any are found to exist, should be actively
treated both locally and constitutionally, and during their
demonstrable presence a most rigorous quarantine should be observed.
[Footnote 79: Hyde, _op. cit._ See p. 264.]
Syphilitic condylomata are due to hypertrophic changes in the papules,
which under the influence of heat and moisture in certain regions
coalesce and become more elevated. They vary in size from an eighth of
an inch to a quarter or even a half of an inch in diameter. Their
surface is flat and covered by a crust or by an offensive secretion.
They are found most commonly about the anus or at the angles of the
mouth.
Pustular Syphilides.--A little later in the secondary period, usually
at about the sixth week, but sometimes much earlier, the papules become
transformed into pustules, the change taking place slowly, so that if
examined at any time after it has begun the child will present an
eruption which is markedly polymorphic, showing here and there
yellowish or reddish-yellow maculæ left after the absorption of the
cell-element of certain papules, at other places beefy-red papules at
the height of their development, or papules crowned by a ring of
desiccated and desquamating epidermic scales, and in still other
regions pustules in various stages of formation. Or the various
formative stages of the pustules may be passed through so quickly that
the eruption will be almost entirely pustular, few if any unmodified
papules being discovered. The pustules may remain distended with pus
for a considerable time, after which they may wither and slowly
disappear or may rupture and leave ulcerated surfaces. A number of
these ulcers sometimes run together and make extensive patches covered
with thick, dark-colored crusts. These patches may resemble areas of
impetigo or of impetiginous eczema, but in those affections the crusts
are usually thinner and of a lighter color, and the skin beneath them
is generally on a level with the surrounding surface, bright red and
glazed; while under the crusts of the syphilide will be found a more or
less depressed or excavated ulcer, often covered with pus. The
diagnosis may indeed often be made by gently detaching and {280}
raising one of the crusts and noting the character of the surface
beneath. The erosion under the crusts of eczema heals over more readily
and without leaving a cicatrix.
A so-called furuncular eruption[80] is said to appear at variable
periods between the sixth month and the third year, but does not appear
to me to be clearly differentiated from the large pustular syphilides
with thickened and elevated bases on the one hand, or the ulcerating
tubercular eruption on the other.[81] They are all so rare in
hereditary syphilis, at any rate, as to have little clinical
importance.
[Footnote 80: Bumstead and Taylor, _op. cit._, p. 750.]
[Footnote 81: The distinction between the two forms is usually manifest
if the development of the lesions has been observed; but even this
fails in regard to the tubercular eruption. They both occur at the same
period; they both begin similarly, the furuncles as "small nodules in
the corium," the tubercles as "deeply-seated papules or nodules;" they
both run on to ulceration and pursue a chronic course (Van Harlingen,
_op. cit._, p. 561).]
Iritis.--Another symptom of the secondary period, but of later
development and of rarer occurrence than the syphilodermata which have
been described, is iritis. In spite of its rarity this is extremely
important, because it is frequently overlooked until it has reached
such a stage that occlusion of the pupil results, and also because when
it is recognized it constitutes an almost pathognomonic sign of
syphilis.[82] This statement may now be made unhesitatingly, although
for many years it was contended that iritis, and even the still more
characteristic symptom keratitis, were only associated with syphilis as
coincidences, the constitutional disease, when hereditary, having no
causative relation to the local condition.
[Footnote 82: "When primary iritis occurs in syphilis in young children
it is almost always due to syphilis" (Soelberg Wells, _Treatise on
Diseases of the Eye_, Philada., 1873, p. 173).]
To Mr. Hutchinson belongs the credit of having first clearly developed
the specific character of this trouble,[83] which, on account of the
mildness of the attendant symptoms, is often overlooked. The sclerotic
zone of congestion so marked in the adult, and therefore so valuable a
diagnostic sign to the general practitioner, is very slight, sometimes
absent; and as a consequence the attention of neither parent nor
physician is attracted to the condition until in the more serious cases
it has done irreparable mischief. In milder cases, particularly where
the child is under mercurial treatment for concomitant symptoms of
syphilis, it may run its course and escape notice altogether;[84] and
it is possible that owing to this fact the rarity of the affection has
been overestimated. It is also possible that in such cases changes
occurring at this time may in some instances lay the foundation for
some of the deeper-seated ocular troubles of later life.
[Footnote 83: _Med. Times and Gazette_, 1860, July 14; _Ophthalmic
Hospital Reports_, vol. i. pp. 191, 226; _A Clinical Memoir on Certain
Diseases of the Eye and Ear consequent on Inherited Syphilis_, London,
1863. In the introduction to this volume Mr. Hutchinson states that
acute iritis dependent on hereditary syphilis was first described in
connection with its true cause by Mr. Lawrence, but, as from the date
of that gentleman's first case (1830) up to 1863 but six cases had been
recorded, the announcement had made but little impression on the
profession.]
[Footnote 84: "In the cases of this form of iritis which are seen in
ordinary eye-practice much damage has often been done by occlusion of
the pupil and deeper mischief. Probably many of the slighter cases
escape the notice of the parents and are not brought to the surgeon"
(Mr. Edward Nettleship. See Hill and Cooper, p. 271).]
If, however, attention has been attracted to the eyes, the diagnosis is
not usually difficult. The pupil is irregular, especially under
atropia; {281} there are streaks of lymph, dulness, swelling, change of
color, and on very careful inspection a faint pink zone may be seen in
the sclerotic. The conjunctiva and cornea are generally clear.
Mr. Hutchinson's analysis of the twenty-three cases reported by him is
still of interest as furnishing reliable data for prognosis. The
average age at the time the iritis commenced was five months and a
half. The oldest was sixteen months at the time of the outbreak, the
youngest six weeks. In twelve cases but one eye suffered; in eleven
both were affected. In seven cases (ten eyes) the cure was complete; in
two or three other cases very slender adhesions remained; in twelve
cases, in nearly all of which the patients came under care only at a
late period of the disease, one pupil was permanently occluded by
organized false membrane. In nearly all, coincident symptoms of
syphilis of the skin or mucous membranes were present. Of the thirteen
cases in which alone a history of the family is recorded, the affected
infant was the only living child of his parents in twelve instances. In
the only case in the whole series in which it is stated that there were
other living children the mother had lost four infants out of seven
live births.
The prognosis depends on the stage at which they come under treatment.
The lymph if recent, no matter in what quantity, will probably be
absorbed under mercurial treatment, which will often be of great
benefit even in those cases in which a certain amount of organization
has occurred.[85]
[Footnote 85: It will not be uninteresting, perhaps, to append the
aphorisms regarding iritis in infants which Mr. Hutchinson at that time
enunciated: 1. The subjects of infantile iritis are much more
frequently of the female than the male sex. 2. The age of five months
is the period of life at or about which syphilitic infants are most
liable to suffer from iritis. 3. Syphilitic iritis in infants is often
symmetrical, but quite as frequently not so. (In his article in
Reynolds's _System of Medicine_, written in 1866, three years later,
but revised in 1870, he describes it as "usually symmetrical," vol. i.
p. 444.) 4. Iritis, as it occurs in infants, is seldom complicated, and
is attended by but few of the more severe symptoms which characterize
the disease in the adult. 5. Notwithstanding the absence of phenomena
of acute inflammation, the effusion of lymph and the danger of
occlusion of the pupil are usually very great. 6. Mercurial treatment
is most signally efficacious in curing the disease, and, if recent, in
procuring the complete absorption of the effused lymph. 7. Mercurial
treatment previously adopted does not prevent the occurrence of this
form of iritis. 8. The subjects of infantile iritis, though often puny
and cachectic, are also often apparently in good condition. 9. Infants
suffering from iritis should always show one or other of the
well-recognized symptoms of hereditary taint. 10. Most of those who
suffer from syphilitic iritis are infants born within a short period of
the date of the primary disease in their parents.]
We have now a group of symptoms characteristic of the secondary period
of syphilis, or that extending from birth, or much more commonly from
the age of three or four weeks to about the end of the first year. The
syphilitic child during this time has several or all of the following
symptoms: Coryza with snuffles; an erythematous, papular, or pustular
eruption on the skin; mucous patches on the lips, tongue, cheeks, etc.;
a marked tendency to general wasting; a hoarse cry or cough; senility
of aspect; iritis. The majority of syphilitic children born alive die
during this stage.
Before its termination, sometimes even at birth, other lesions have
been noticed (especially those affecting the liver), which, however,
may better be described in connection with the special organ or organs
involved.
Succeeding this stage--_i.e._ beginning in about a year or eighteen
{282} months--comes an intermediate period, which extends to second
dentition, to puberty, or even much later, and which is characterized
rather negatively--that is, by the absence of symptoms--than otherwise.
The evidence of the general diathesis will of course be present in the
shape possibly of malnutrition, stunted growth, or retarded
development, perhaps shown in the weazened or withered face, the sunken
nose, the pallor of the skin, the premature loss of the upper incisor
teeth or the malformation of the others if they have erupted.
There is but little tendency to recurrence or relapse of any of the
secondary symptoms; and in certain cases, not a very small proportion,
in which these symptoms have been light and have been well and
thoroughly treated, this stage extends throughout life; or, in other
words, as is frequently the case with the adult who has followed a
proper course of treatment, the disease appears to terminate with the
secondary stage. In other cases, however, it recurs, and the symptoms
which it then presents may be taken up in connection with the different
organs or tissues involved.
Syphilis of the ear is for obvious reasons not often discoverable until
the patient has reached an age at which interference with the function
of hearing becomes a noticeable phenomena. The only symptom likely to
attract attention during the stage of inherited syphilis which we are
now considering is a catarrh of the middle ear, which may have for its
starting-point some inflammation, ulceration, or mucous patch of the
pharynx, causing a temporary or permanent occlusion of the orifices of
the Eustachian tubes.[86] This may lead to perforation of the membrana
tympani, purulent infiltration of the mastoid cells, etc., and when
accompanied by an otorrhoea which attracts attention to the ear will be
easily discovered by the physician. These cases are, however,
exceptional, otorrhoea only being present in nine out of Hutchinson and
Jackson's[87] one hundred cases of inherited syphilis, and consequently
but little is known about the frequency or gravity of lesions of the
auditory apparatus in the secondary stage of this form of syphilis.[88]
The changes which occur later on are chiefly those which involve either
the nerves themselves or their distribution in the labyrinth.
[Footnote 86: Bäumler, _Ziemssen's Cyclopædia_, vol. iii. p. 226.]
[Footnote 87: Hutchinson and Hughlings Jackson, _Med. Times and Gaz._,
Nov. 23, 1861.]
[Footnote 88: Schwartze (quoted by Hill and Cooper) found also that
otorrhoea was a rare complication in deafness from syphilis.]