of acquired syphilis, but are apt to be milder, to involve fewer bones,
and to yield more readily to treatment. This would of course be
expected, inasmuch as the same difference in favor of the acquired
form, as compared with that which is inherited, extends to all the
lesions. As Diday succinctly expresses it: "In the one case the poison
vitiates only the elements of nutrition; in the other it vitiates at
the same time those of formation and those of nutrition." It would
exceed the limits of the present article to describe acquired syphilis
in children.]
The most important differential diagnosis to be made in these cases is
between the rachitis of young children and the form of syphilis in
question. Much difference of opinion still exists as to the relation
between these diseases, syphilis being claimed, on the one hand, as
having in the majority of cases a definite causative influence, while,
on the other, the existence of this relation is denied. When we come to
contrast the pathology of the two diseases, we can readily understand
why they should be confounded, the minuter changes which occur being
essentially the same--viz. cell-proliferation and accumulation, with
subsequent inflammatory changes, associated with irregular deposits of
lime salts.
Compare, for example, the description of the pathology of bone diseases
in inherited syphilis already given (pp. 287, 288) with the following
terse summary of the changes which take place in rickets in cases where
no suspicion of syphilis exists, either ancestral or acquired: "The
changes are more distinctly noticed at the epiphyses than in the
diaphyses. Instead of the regular stages and distinct boundaries
observed in the normal development of bone, there is a singular
disorderly commingling of the exaggerated cartilage-proliferation and
transition substance, with calcification. The cartilage-cells,
stimulated to excessive multiplication, are transformed, some into
bone-corpuscles, some into medullary cells, {290} and others into
connective-tissue forms. The same process is in active operation in the
deep periosteal layers, the material accumulating to such a degree as
to add much to the thickness of the shaft."[120]
[Footnote 120: Agnew's _Surgery_, vol. i. p. 1030.]
The points of resemblance are manifest, just as they are between a
syphilitic and a variolous pustule, but they end in both cases when we
come to study the evolution of the phenomena either from an anatomical
or from a clinical standpoint. They may be expressed as follows in
tabular form:
OSSEOUS LESIONS DUE TO INHERITED | RICKETS.
SYPHILIS. |
|
The swellings, particularly those| Rarely appear before six months,
of the long bones, show | generally still later.
themselves at or soon after |
birth. |
|
A history of syphilis or evidence| No such history necessarily.
of existing syphilis in one or |
both parents. |
|
Preceded or accompanied by | No such prodromata.
snuffles, coryza, and cutaneous |
and mucous lesions. |
|
No such prodromata in most cases.| Pallor, restlessness, sweating,
| nausea, diarrhoea, etc.
| constitute a combination of
| symptoms which often precede the
| bone disease.
|
Cachexia absent or moderate. | Cachexia marked.
|
Physiognomical peculiarities of | Not present as a group.
syphilis present.[121] |
|
Circumscribed tumors on frontal | Cranial bones thickened in spots,
and parietal bones, rarely on | usually upon the occiput.
occiput. |
|
Ribs not markedly affected. | All or nearly all involved.
|
Swellings on long bones or | Extremities symmetrically
extremities irregular. | enlarged.
|
Disease of ribs, when existent, | Nearly always so.
not ordinarily coincident with |
that of other bones. |
|
Fontanels close at usual period. | Closure delayed.
|
Other syphilitic symptoms | Syphilitic symptoms absent.
present; enlargement of |
phalanges, metatarsal bones, etc.|
|
Often accompanied by sinuses, | Little external or surrounding
synovitis, abscesses, cutaneous | involvement.
ulcers, etc. |
|
Generally disappears by | Usually leaves some bending of
resolution, without leaving any | shaft and distortion of the
permanent change. | neighboring joint.
|
Mortality among children in whom | Much less.
many bones are involved is very |
great. |
|
Specific treatment useful. | Of no benefit.
|
In the first stage there is an | This is less marked. There is
exuberant calcification of the | formed, instead, a soft and
ossifying cartilage, causing | non-calcified osteoid tissue.
necrosis of the new-formed tissue|
and a consecutive inflammation, |
which terminates in the |
separation of the epiphyses.[122]|
[Footnote 121: See p. 313.]
[Footnote 122: This table is founded on one which I added to the
translation of Cornil made by Dr. Simes and myself, and is compiled
chiefly from the excellent work of Dr. Taylor already alluded to.]
The diagnosis of the bone lesions of hereditary exostosis can readily
be recognized in a short time by noting the fact that they are
stationary, {291} even if their later appearance, larger size, the
absence of syphilitic history or symptoms, and the resistance to
specific treatment left us in doubt.
The diagnosis from accidental separation of the epiphysis, or from
fractures, may be made from the history of the case.
In cases of separation of the epiphysis, complicated with suppuration,
sinuses, etc., the trouble may be mistaken for a similar condition due
to non-specific inflammation. In all the recorded instances, however,
the latter has occurred much later in life, is attended with much more
acute inflammatory symptoms, lymphangitis, etc., and is of course
without concomitant symptoms of syphilis. In both these cases there is
a decided osteo-periostitis, and as so much depends on the early and
vigorous use of specific treatment, it may be worth while to contrast
the two forms of the disease.
SYPHILITIC OSTEO-PERIOSTITIS. | NON-SPECIFIC OSTEO-PERIOSTITIS.
|
Occurs in infants under three | No instance of its occurrence in
months of age. | children under one year of age.
|
History of syphilis in child and | No history of syphilis; sometimes
its parents. | a history of traumatism.
|
Implication of other bones. | Usually confined to one bone.
|
Coincident with the development | Coexists with the ossification of
of the shaft of the bone. | the epiphyses.
|
Other lesions of syphilis: nodes,| No such symptoms.
skin eruptions, etc. |
|
All the local symptoms | Pain, redness, and swelling very
comparatively mild. | marked.
|
Disease sharply localized. | Involves neighboring parts.
|
Lymphatics of limb unaffected. | Lymphangitis present.
|
Beneficial effect of specific | No such effect.
treatment if employed early.[123]|
[Footnote 123: Cornil, _op. cit._, p. 274.]
Syphilitic dactylitis in the inherited variety of the disease, as in
the acquired, consists of two varieties. The one of these which usually
appears earlier involves chiefly the periosteum and the fibrous and
integumentary structures surrounding a joint, usually a metacarpo- or
metatarso-phalangeal articulation, involving a phalanx, and is
characterized by slow, almost painless, swelling and discoloration of
the affected member. (Fig. 9.) This is due to a gummous infiltration
which, after absorption under proper treatment, leaves the toe or
finger temporarily stiff, but not permanently disabled.
The second form is a specific osteo-myelitis, with periostitis, coming
on later, and often destroying the bone or the articulation involved.
(Fig. 10.)
[Illustration: FIG. 9. FIG. 10. From Bumstead on _Venereal Diseases_,
illustrating Syphilitic Dactylitis.]
The absence of acute inflammatory symptoms in the first variety
distinguishes it from paronychia, whitlow, and gout. Rheumatoid
arthritis begins in the joints, is associated with other symptoms;
deformity of the fingers comes early in the disease, and there is a
teno-synovitis with contraction.
The second variety might be taken for enchondroma or exostosis, but
these growths increase much more slowly, involve only a limited portion
of the bone, are of greater density, and are much more strictly
circumscribed.
As a rule, especially in cases which are recognized early and treated
{292} actively, the prognosis is good. Iodide of potassium should be
used in combination with mercury.
Syphilis of the Teeth.--Syphilis of the teeth has its chief interest to
the general practitioner from its very important bearing on diagnosis.
As manifesting itself at an age when the child is not apt to present
the active and unmistakable cutaneous and mucous lesions of the
disease, and when, consequently, its recognition is often extremely
difficult, this diagnostic importance is greatly increased.
The teeth of the first dentition, although exhibiting the usual signs
of interference with nutrition in their irregular development, opaque
and chalky enamel deficient in quantity and unevenly distributed, soft
and friable dentine, incongruity of size individually and relatively,
and proneness to decay, do not often display any distinctive evidence
of syphilis. {293} The same conditions may, and often do, depend on
other causes, and are commonly associated with various cachexiæ--the
strumous, gouty, rheumatic, rachitic, etc.--and even with other
slighter ailments tending to produce imperfect assimilation and
malnutrition.
In the permanent teeth, likewise, the same condition may be due to the
same causes. Stomatitis, however produced--by mercury, by
gastro-intestinal derangements, by local irritation of any kind--is apt
to result in imperfectly organized dental structures. Mercurial teeth,
for example, are usually irregularly aligned, horizontally seamed,
honeycombed, craggy, malformed, of an unhealthy dirty yellow color,
separated too widely, and deficient in enamel.[124] The diseases of
childhood, especially the eruptive fevers, eclampsia, typhoid fever,
etc., by temporarily arresting or greatly interfering with nutrition
during the developmental period of the teeth, often cause horizontal
furrows across their crowns, which are, of course, persistent
throughout life, and mark indelibly the influence of such disorders on
all the formative processes.
[Footnote 124: The latter defect is particularly noticeable on the
cusps of the sixth-year molars. (See note on "Syphilis of the Teeth,"
by Dr. James W. White, in Am. ed. of Cornil, pp. 287-290.) The
discussion as to the effect of mercury in producing the condition of
the teeth known as honeycombed is still going on, but the evidence
seems to point clearly to a direct connection with the administration
of mercury in infancy, either for syphilis or in excessive doses as a
purge, or in some of the teething powders, which often consist of
calomel and opium. Mr. Hutchinson, at a meeting of the Odontological
Society (see _Proceedings_ for 1877, p. 249), gave an interesting
description of the way in which the supposition was arrived at.
Lamellar cataract is a disease which affects the eyes of children who
have suffered from convulsions: it was noticed that in cases of that
form of catarrh there were also honeycombed teeth, and it was thought
that the convulsions, the cataract, and the honeycombed teeth were all
due to the same unknown cause. At last a few exceptions were
found--patients with cataract, but with good teeth, and then some who
had had convulsions only, and yet had honeycombed teeth; lastly, it was
noticed that most of the patients had been treated with mercury. So it
came to be recognized that the honeycombed teeth were only accidentally
associated with the cataract, and that they were, in fact, the result
of the mercury which had been given to cure the convulsions. The same
gentleman figures a case of this disease in his _Illustrations of
Clinical Surgery_ (London, 1875), and thus describes it (p. 55): "The
present state of his permanent teeth is so characteristic as to deserve
more detailed description. The change about to be mentioned affects all
the incisors, canines, and first molars of both upper and lower jaws,
the bicuspid being scarcely implicated at all; the second molars are
also quite healthy. In the first molars the alterations consist of
deficiency of enamel on the upper surface of the crown and the presence
of spines of uncovered dentine. In the case of the incisors a
considerable portion of the crown of each tooth is totally devoid of
enamel, and its dentine is also deficient to some extent, so that the
teeth are thin, sharp-edged, and of a dirty yellowish color. The
transition from the enamel-covered to the diseased part occurs suddenly
in a horizontal line at some little distance from the crown of each
tooth; the position of this line or step being in each tooth nearly at
the same distance from the gum. The general effect when all the teeth
are seen together is as if a string had been tied around them when soft
and the distal part had withered."]
None of these conditions, however, are in the least degree
characteristic of syphilis, the special expression of which in the
mouth is to be found only in the permanent upper median incisors. For
the recognition and description of the peculiarities of these teeth in
the subjects of inherited syphilis we are indebted, as we are for so
much else of inestimable value in the study of the disease, to Mr.
Hutchinson. In 1863, in a memoir on _Syphilitic Diseases of the Eye and
Ear_, he wrote as follows[125] {294} concerning the symptoms which, in
a suspected case, would aid in determining the diagnosis: "By far the
most reliable amongst the objective symptoms is the state of the
permanent teeth if the patient be of age to show them. Although the
temporary teeth often, indeed usually, present some peculiarities in
syphilitic children of which a trained observer may avail himself, yet
they show nothing which is pathognomonic, and nothing which I dare
describe as worthy of general reliance.[126] _The central upper
incisors of the second set are the test teeth_, and the surgeon not
thoroughly conversant with the various and very common forms of dental
malformation will avoid much risk of error if he restricts his
attention to this pair. In syphilitic patients these teeth are usually
short and narrow, with a broad vertical notch in their edges and their
corners rounded off. Horizontal notches or furrows are often seen, but
they, as a rule, have nothing to do with syphilis. If the question be
put, Are teeth of the type described pathognomonic of syphilis? I
answer unreservedly that when well characterized I believe they are. I
have met with many cases in which the type in question was so slightly
marked that it served only to suggest suspicion, and by no means to
remove doubt; but I have never seen it well characterized without
having reason to believe that the inference to which it pointed was
well founded."
[Footnote 125: Chapter on "The Means of Recognition of the Subjects of
Hereditary Syphilis during the Tertiary Stage," p. 204. Before this,
however, he had called attention to the same peculiarities in a paper
on "The Means of Recognizing the Subjects of Inherited Syphilis in
Adult Life," _Medical Times and Gazette_, London, Sept. 11, 1858, p.
265.]
[Footnote 126: So far as I know, the only recorded instances by
reliable observers of the temporary teeth presenting the peculiar
characteristics of syphilis are as follows: In the _Transactions_ of
the Odontological Society of Great Britain, vol. ix., 1877, p. 258, Mr.
Oakley Coles described a case--without, unfortunately, giving
details--in which the "peg-shaped temporary teeth were very
characteristic of syphilis." Mr. Coles's abilities are well known, and
the fact that at the previous meeting the society had been addressed by
Mr. Hutchinson on this very subject would seem to indicate that he was
quite familiar with the importance of his statement.
M. Fournier calls attention (_Archives de Derm. et Syph._, Sept. 25,
1883) to a cast (No. 48) to be found in the collection of M. Parrot in
the museum of the Foundling Hospital at Paris. This displays the
dentition of a child twenty-seven months old, in which the two upper
median incisors are typical Hutchinson teeth. M. Fournier adds that
later researches show unmistakably that the temporary teeth may be
affected by hereditary syphilis in the same manner as those of the
second dentition, although not more than one case of the former is
recognized to fifteen or twenty of the latter. He thinks, however, that
this proportion would be greatly modified if in the autopsies of young
children the alveoli were opened to examine the embryonic teeth. M.
Parrot by this means claims to have often discovered lesions of the
milk teeth.]
As a matter of course, so positive a statement in a matter of such
gravity and importance excited considerable criticism, and the views of
Mr. Hutchinson have never been without earnest and often able
opponents; but it is safe to say that time has only served to place
them on a surer foundation and to enhance their value in the eyes of
the profession. That they have undergone no material change in the mind
of their distinguished author is shown by his expressions of opinion
during the debate on syphilis in the London Pathological Society in
1876,[127] and still later by the following memoranda which he gives as
a guide in diagnosis:[128]
"1. No special peculiarities are to be looked for in the first set of
teeth.
"2. There can be no more serious blunder than to imagine that bad teeth
in proportion to their badness of form are to be suspected of syphilis.
{295} "3. The upper central incisors are the only teeth which are
positively characteristic. The others may afford corroborative
testimony, but are not to be relied upon alone.
"4. The chief peculiarity is a general dwarfing of the tooth, which is
both too short and too narrow, and, from its sides slanting together,
presents a tendency to become pointed. This tendency to pointing is
always defeated by the cutting off of the end, the truncation being
usually effected in a line curved upward, so as to produce a single
shallow notch. At the bottom of this notch the enamel is deficient and
the dentine exposed, but there is no irregular pitting, as in
stomatitis teeth.
"5. The malformations are unusually symmetrical and affect pairs of
teeth. The two central incisors resemble each other, and the two
laterals are also alike. If any defect passes horizontally across all
the incisors at the same level, and affects them all alike, it is
probably not due to syphilis.
"6. In syphilis the lateral incisors usually show little or no
malformation.
"7. The occurrence of the peculiarities due to syphilis and those due
to mercury in the same mouth are exceedingly common."
[Footnote 127: _London Lancet_, 1876, pp. 56 and 535.]
[Footnote 128: _Illustrations of Clinical Surgery_, fasciculus xi.,
London, 1878.]
The great importance of the subject seems to me to justify one more
quotation, as showing the opinion at a very recent date of men well
qualified to judge of the correctness or inaccuracy of these
statements. Mr. C. Macnamara and Dr. Thomas Barlow[129] say: "The
characters of the teeth are so valuable when present that it is
important to have them clearly noted--the more so that, in spite of Mr.
Hutchinson's clear description, they have been much misrepresented. It
may be pointed out--(1) That only the upper median permanent incisors
are characteristic, and sometimes only one of them is typical, of the
disease; (2) that these teeth are generally a little apart, instead of
being in apposition, and are more or less dwarfed; (3) that in a
typical specimen the width of the cutting edge is narrower than the
width of the tooth as it emerges from the gum; (4) that a typical
syphilitic tooth presents a single notch, not a serrated margin; and
that occasionally, if the notch has not been actually scooped out,
there is a little lunula-shaped area which may readily become a notch;
(5) finally, that although such teeth, when present, are absolutely
pathognomonic, the existence of normal permanent upper median incisors
by no means excludes the existence of hereditary syphilis."
[Footnote 129: On behalf of the Collective Investigation Committee, who
have issued a circular designed to elicit information as to the effect
of syphilis on the civil population of Great Britain. This circular has
been sent to physicians, and contains queries as to various points
relating to the symptoms of inherited syphilis; among others as to the
existence in any given case of "notched, dwarfed upper median
incisors," which, with or without other symptoms, would establish the
diagnosis of that case. The observations above quoted are explanatory
of this question (_The British Medical Journal_, Dec. 16, 1882).]
I believe this may fairly be taken to represent the general belief at
the present day among those best qualified to pronounce upon the merits
of the case; and I may say that it is unqualifiedly my own opinion,
arrived at after some experience and considerable investigation into
the literature of the subject. It is not uncommon, however, to hear
doubts expressed as to the value of this sign in the diagnosis of
syphilis, and at intervals articles are written or papers read to prove
that it is not of the uniform and distinctively conclusive significance
that has been attributed to it.[130]
[Footnote 130: "I cannot say more in favor of the diagnostic values of
these teeth than that, when {296} present in typical form, they have a
certain weight in favor of the existence of hereditary syphilis in the
given subject" (Van Harlingen, article "Syphilis" in _Internat. Encyc.
of Surgery_, vol. ii. p. 565). "It has been the custom from time to
time since Mr. Hutchinson made his observation to question the validity
of his views, both as to the fact of interstitial keratitis being due
to hereditary syphilis, and as to the diagnostic values of the
so-called characteristic teeth. Thus, it has been asserted, not only in
England, but on the Continent, and especially in Germany, that the
disease may be the result of malnutrition in scrofulous and rickety
subjects; and it has been maintained that the malformation of the teeth
is the simple arrest of development in a perverted constitution from
other causes than syphilis" (Bumstead and Taylor, _op. cit._, p. 701).
Garretson says (_Oral Surgery_, p. 316): "Observations will be found to
greatly vary concerning the existence of any constancy in phenomenal
expressions of the teeth in this relation."
M. Magitot, who has for some years been supposed to pay especial
attention to this subject, has recently written an elaborate paper
(_Gazette des Hôpitaux_, Sept. 29, Oct. 4, 11, and 18, 1881) to prove
(1st) that dental erosions, as he calls them, are not due to syphilis;
and (2d) that they are due to, or are almost invariably associated
with, infantile convulsions. He has collected a number of interesting
facts, but a very careful study of his article and inspection of his
diagrams have convinced me that he does not recognize at all the
special peculiarities of the Hutchinson teeth, but includes under his
title of "erosion" a variety of widely-differing conditions. He has
altogether misinterpreted Mr. Hutchinson's views as stated in his
_Memoirs on Certain Diseases of the Eye and Ear due to Inherited
Syphilis_, and represents him as at that time (1863) believing that the
cause of the change in the incisors was a mercurial stomatitis. The
quotation on p. 294 sufficiently refutes this absurdity. Of course the
paper as an argument against the syphilitic origin of these teeth is
without the slightest value.]