cases the diathesis has been inherited from the parents, the fact
cannot be too strongly emphasized that in a large number of cases the
disease may be developed de novo, independent of such heredity. To
scrofula developed from the influence of bad ventilation and
overcrowding, absence of sunlight, insufficient, bad, or unsuitable
food, cold and damp, imperfect clothing--in short, all those conditions
associated with poverty, squalor, and ignorance--Grancher has well
applied the term la scrofula a miseria. And it is only by a clear
comprehension that scrofula may be, and often is, developed under these
conditions that the medical profession in general, and municipal health
authorities in particular, may be induced to teach and enforce upon the
poor both the knowledge and the practice which may prevent it. Even in
the open country, where there is at least no lack of pure air and
light, the lesson can be enforced with equal profit; for the children
of the farm-laborer are likely to be imperfectly and improperly fed,
and lodged in apartments at night that in the matter of foul air and
filth could not be well surpassed in the purlieus of the dirtiest and
most overcrowded city.
EXCITING CAUSES.--The actual exciting causes of scrofula when the
diathesis already exists are too numerous to be mentioned in detail.
Indeed, almost any trivial injury or inflammation, any disease which
has produced a temporary cachexia, may rouse into activity the perhaps
hitherto latent tendency. How often do we see a slight blow upon the
knee-joint produce a white swelling which lames for life the heretofore
healthy and active boy or girl! A fall upon the hip which was almost
unnoticed at the time excites a coxalgia which either destroys life or
renders the child for life a cripple; or a slight jar of the spine
induces a disease of the vertebræ which, if recovered from at all,
produces a terrible deformity. A slight eczema of the face or scalp or
a catarrh of the mouth or throat will excite that slow and generally
painless enlargement and induration of a neighboring lymphatic gland
which always ends in its caseation and final destruction by
suppuration. A slight injury to the {237} periosteum may excite a
destructive caries or necrosis of the underlying bone, and a temporary
catarrh of the intestines a tabes mesenterica with all its fatal
consequences.
Speaking generally, it may be said that anything that produces a local
disorder of nutrition or impairs the health generally of a person
predisposed to scrofula is sufficient to bring about some
manifestations of the disease. They are especially apt to follow the
eruptive fevers. Measles and scarlatina are very commonly arousers of
the scrofulous process, not only by the temporary impairment of health
which follows them, but also through the catarrhs which are usually
present in both diseases. Vaccination has often been accused of
imparting scrofula; and, although this is untrue, since scrofula cannot
be imparted in the sense of transference from one person to another,
there can be no doubt that the predisposition may be roused into
activity by the slight impairment of health associated with vaccination
or by the slight injury inflicted at the point of introduction of the
vaccinal virus. In some cases the disease has manifested itself for the
first time during pregnancy or lactation, and there is no doubt that in
cases where the disease has existed in childhood these conditions often
cause it to reappear. In conclusion, it must be said that many cases
apparently occur spontaneously--"the disease came on of itself"--or if
there are any exciting causes they were so trivial as to have escaped
notice altogether.
Finally, it must be remembered that the eczemas, catarrhs, ophthalmias,
otitis, chilblains, erysipelas, and numerous other local disorders of
nutrition which are often the causes of graver manifestations of the
disease, are themselves very prone to run a peculiar course
characterized by chronicity and intractableness; and many regard these
disorders as themselves manifestations of scrofula. Indeed, Virchow,
basing his argument upon the fact "that scrofulous enlargement of the
lymphatic glands of the neck often follows upon certain diseases
affecting the throat, such as mumps, diphtheria, and scarlet fever,
maintains that scrofulous proliferation of these glands, like ordinary
inflammatory hyperplasia of the same organs, is always secondary to
some peculiar process going on at the mucous surface or other part
which is in direct relation with them by means of the lymphatic
vessels; that scrofulous disease of the glands of the neck is traceable
to some inflammatory condition of the throat, fauces, or contiguous
parts; of the bronchial and mediastinal glands, to pulmonary or
bronchial inflammation; and of the mesenteric and retro-peritoneal
glands, to similar conditions of the alimentary canal. But he considers
that there may be some special element or quality in the primary
inflammation, and a tendency in its products to undergo rapid decay
similar to that which characterizes the morbid products of the diseased
lymphatic glands, but that generally they are not recognizable, from
the fact that in this case the cells are mostly developed at a free
surface, and are speedily shed from it." He admits, however, "that
there may be some special aptitude or weakness, congenital or acquired,
in the lymphatic glands of certain persons, or of certain parts of
them, which makes their inflammations, induced by indifferent causes,
assume the scrofulous character."[6] From the last of these
propositions no one will be likely to dissent, but that there is "some
specific quality or {238} element in the primary inflammation," etc.
few will be inclined to agree. There is nothing peculiar in the
eczemas, ophthalmias, catarrhs, etc. spoken of, except that they occur
in persons possessing the scrofulous diathesis; and it is this alone
that gives them their special characters, if there are any. In other
words, we cannot say that these disorders occur in certain children
because they are scrofulous, but that they are specially intractable on
account of the scrofulous diathesis upon which they are engrafted.
Millions of children have catarrh and ophthalmia (indeed, few escape
these disorders throughout the first ten or twelve years of life) who
never show any other evidence of the scrofulous taint; and nearly all
have measles and scarlatina, but it is only the scrofulous who usually
suffer severely from the secondary effects of these diseases. But they
do not have measles or scarlatina because they are scrofulous, and we
can with no more justice say that they have catarrhs or other
inflammations because they are so. We do not believe, therefore, that
strumous children have cutaneous and catarrhal inflammations simply
because they are strumous; and if we speak of scrofulous catarrh or
ophthalmia or eczema, we use the terms in the same sense as when we
would speak of a scrofulous measle, scarlatina, or whooping cough.
[Footnote 6: _The Theory and Practice of Medicine_, by Jno. Syer
Bristowe, M.D., 1879, pp. 80, 81.]
Although we have few reliable statistics bearing upon the question, it
can scarcely be doubted, judged by the results of casual observation,
that scrofula is much less frequent in America than in Europe, and that
in the latter there is less of it than formerly. The cheapness of land
in America has prevented that excessive overcrowding that exists in the
older and more densely populated countries, and the abundance and
cheapness of animal food has prevented that excessive feeding on bread
and potatoes which constitutes such an important factor in the
production of the scrofulous diathesis in some other countries.
PATHOLOGY AND MORBID ANATOMY.--The most important and central
anatomical and pathological facts both in the causation and progressive
development of struma, according to the writer's views, are--
1st. That faulty anatomical--or rather histological--construction of
the tissues of the scrofulous individual already alluded to as having
been first brought to the notice of the profession by Formad of
Philadelphia, which consist of an unusually large number of
lymph-spaces (which are also unusually large), and consequently an
excessive number of lymph-vessels and lymph-glands.
2d. Excessive production of rudimentary lymphoid cells, and probably
also of lymphatic tissue.
3d. Diminished and insufficient number of the capillary blood-vessels;
and, as a necessary consequence of these,
4th. Diminished nutritive activity of all those processes, both
physiological and pathological, which depend upon a full supply of
nutritive blood.
The most striking feature in all scrofulous inflammation is excessive
cell-growth, but these cells show little tendency to differentiation
and organization, probably for two reasons: 1st, because they are
derived from the blood-vessels principally, and not from proliferation
of the proper connective-tissue cells of the part; and 2d, because they
are insufficiently supplied with nutrition from the scanty
blood-vascular {239} network, and this supply is too rapidly absorbed
into the lymph-spaces, and is carried off by the too numerous
lymph-vessels. The cells, therefore, speedily perish, undergo partial
or imperfect fatty degeneration, and finally caseation, unless the
process is going on at a free surface, in which case, of course, they
are shed and thus gotten rid of.
Virchow some time ago called attention to the predominant cellular
character of the scrofulous exudation and the low vitality of the cells
which compose it. Rindfleisch declares that the fresh scrofulous
exudations contain relatively large cells with glistening protoplasm,
and that the white blood-corpuscles have a tendency in scrofulous
persons to grow larger on their way through the connective tissue. He
adds that they swell up by the imbibition of albuminous substances, and
by this very swelling die and slowly degenerate.
It seems to the writer, however, that it is probable that herein lies
the reason why swelling and apparent hyperplasia of the lymphatic
glands in the neighborhood of a local inflammation occurring in a
scrofulous person always takes place. The swollen cells become arrested
at the first gland they reach, and block the channels through the
gland. Successive additions of cells continue to block these channels,
and finally the passage of lymph through the gland becomes impossible,
and then begins that secondary increase of the lymph-cells in the gland
resulting from their inflammatory proliferation.
"In scrofulous inflammation," say Cornil and Ranvier,[7] "there is a
remarkable tendency to permanent infiltration of the affected tissue.
In simple inflammation (_i.e._ inflammation in non-scrofulous persons)
the infiltration is a temporary condition which terminates in
suppuration, in organization, or in resolution." Now, the several steps
in this process of resolution are--contraction of the distended
blood-vessels, thus cutting off the excessive supply of blood which has
caused the exudation and cell-proliferation; fatty degeneration of the
new cell-formation; liquefaction of this fat by union with the alkaline
blood-plasma, converting it into a dialyzable (saponaceous) liquid
which can now be readily absorbed by the veins. In scrofulous
infiltration the cells are speedily attacked by fatty degeneration
(which seems to be strictly a physiological process), but instead of
becoming liquefied, it (the fat) remains, slowly dries and hardens, and
finally becomes converted into the so-called cheesy mass or cheesy
infiltration. It does not liquefy, because it does not receive a
sufficiently abundant supply of the alkaline blood-plasma from the
scanty blood-vessels, and that which is supplied too rapidly flows into
the numerous large lymph-spaces and is carried off by the
lymph-vessels. In the case of the infiltrated gland the supply of this
plasma is cut off in both directions. The passage of lymph through the
gland is blocked, when, of course, none can then reach it through the
lymph-vessel leading to it, while the swelling of the gland itself from
accumulated cells compresses the neighboring nutrient vessels and cuts
off the supply from this direction also. Hence the speedy death, fatty
degeneration, and caseation (not liquefaction) of the cells.
[Footnote 7: Ed. 1880, p. 114.]
"The newly-formed material not only interrupts the lymph-passages of
the gland, but also compresses the blood-capillaries in such manner
that the circulation completely stagnates. It is impossible by any
{240} method of injection to penetrate into the most swollen parts of
the gland. With the supply of blood the nutrition also self-evidently
ceases; the gland falls into caseous degeneration. Where this enters in
the gray mass first becomes opaque, then whitish-yellow,
non-transparent, dry, friable. If the whole gland has passed into the
caseous condition, it appears upon a section as a fresh potato, only
not quite so moist, but just as homogenously yellowish-white."[8]
[Footnote 8: Rindfleisch, _Textbook of Pathological Histology_, 1870,
p. 202.]
The subsequent fate of these glands seems to depend somewhat upon their
situation. In the mesenteric and bronchial glands almost always the
caseous mass is attacked by calcareous infiltration, and finally dries
into a solid chalky concretion. The writer counted seven of these
chalky masses around the primary bronchi of a boy about fifteen years
old whose body was brought into a dissecting-room in Baltimore City.
But the more common result of the caseous process in the glands of the
neck is softening. "The caseous dépôt melts from within outward into a
whitish-yellow, whey-like fluid, which holds a fatty granular detritus
suspended in smaller or larger fragments. If all the caseous material
has softened, the neighborhood of the gland is wont to inflame; this
inflammation facilitates the way for the scrofulous pus outward. This
is evacuated, and we have the scrofulous ulcer, with its overhanging,
bluish, hyperæmic, flabby edges. At length this opening also closes,
and a drawn-in, radiated cicatrix marks the place where the evacuation
took place."[9]
[Footnote 9: _Ibid._, _loc. cit._]
But it must be borne in mind that all so-called scrofulous hyperplasias
of lymphatic glands do not run this destructive course. Undoubtedly, in
a few cases there remains a sufficient nutritive supply to carry on the
liquefactive process which normally follows fatty degeneration, and
thus resolution of the affected gland takes place. We are, however, of
the opinion that Virchow was mistaken when he asserted that complete
resolution of the cheesy material could take place; and from what we
know of the dangerous and usually fatal consequences of the absorption
of this cheesy detritus, Rindfleisch is certainly in error in
describing this as the most desirable possibility of decomposition.
We have heretofore purposely avoided any mention of tubercle or
tuberculosis as a part of the scrofulous process. In the views of many
physicians the relations between the two processes are so close that to
them tuberculosis and scrofulosis mean one and the same thing. While
medical opinion as to the true meaning of the word tubercle was so
discordant and unsettled, while so many products of diverse
pathological processes were included in that term, and while many,
following the view of Burdon-Sanderson of England, believed that
tubercle always takes its origin in small, even microscopic,
collections of lymphatic tissue, such a belief in the identity of the
two processes was not only possible, but reasonable. But since, by very
many good authorities, the term tubercle is now limited to the miliary
or submiliary tubercle, since numerous inoculation experiments have
shown that tuberculosis can be induced in non-scrofulous animals, and
Koch of Germany has proved that there exists in decaying tubercle a
peculiar and distinctive bacillus which even when cultivated out of the
body of a tuberculous person will excite tuberculosis also if
inoculated upon a non-scrofulous animal,--a belief in the identity of
the two diseases seems to be no longer tenable. Certainly, it {241}
would seem that to Sanderson's view that tubercle always takes its
origin in lymphatic tissue it is only necessary to reply that the
subjects of miliary tuberculosis do not more frequently than those
suffering from other non-scrofulous diseases present those larger
glandular hyperplasiæ which are so distinctly characteristic of
scrofula, and to which many persons limit the term scrofulosis. It may
be said, perhaps, that the converse of this is not true, and that
scrofulous persons are more frequently attacked by miliary tuberculosis
than an equal number of non-scrofulous persons. But the extreme
susceptibility or liability of the scrofulous to be attacked by
numerous and even diverse morbid processes, and the profound cachexias
and dyscrasias which the scrofulous processes engender, amply account
for the apparent susceptibility of the scrofulous to be attacked by
miliary tuberculosis. The strumous are more susceptible to the exciting
causes of tuberculosis undoubtedly, but perhaps the same may be said in
regard to measles, scarlatina, and the various other exanthemata. It is
undoubtedly true also that among the lower animals (and probably also
in the higher ones) the introduction into the circulation of the
semi-purulent fluid resulting from the breaking down of a cheesy
scrofulous gland will produce that peculiar (perhaps specific)
dyscrasia which results in miliary tuberculosis. But as it has also
been abundantly proved that a similar fluid derived from a cheesy
pneumonia, or from the inflammatory products of any other disease which
have undergone the cheesy degeneration, will also excite tuberculosis,
the fact does not seem to tell in favor of the identity of, or even of
any close relationship between, the two processes. Still, as the
scrofulous more frequently than other people are the subjects of this
cheesy process, it is not surprising that they should more frequently
be poisoned by the entrance into their blood of the cheesy detritus.
We do not deem it necessary to adduce all the evidence or to state
authorities upon this subject, but we think we are justified in stating
the following doctrine in regard to the relation of scrofula to
tuberculosis as best supported by facts and by the consensus of medical
opinion:
Scrofula is a purely diathetic disease inherent in the individual.
Tuberculosis is a cachectic (possibly a purely dyscratic) one, not
inherent in the individual, but always caused by some morbid influence
from without. Tuberculosis may therefore occur in the non-scrofulous as
well as in the scrofulous.
But the scrofulous are more likely than others to have
tuberculosis--1st, because of their greater susceptibility to all
morbid influences; 2d, because the scrofulous processes are apt to
produce some cachectic condition which is always a condition precedent
to tuberculosis; and, lastly, because the products of decay resulting
from scrofulous processes may enter the circulation and directly
produce the tuberculous dyscrasia. These remarks of course apply only
to primary tuberculosis.
But while we thus deny anything else than a purely incidental relation
of scrofula to tuberculosis, we believe that there exists the very
strongest possible relationship of scrofula to pulmonary consumption.
We think we are justified in stating that fully 95 per cent. of all
cases of pulmonary consumption are of inflammatory origin, and of that
variety miscalled catarrhal pneumonia. Broncho-pneumonia or
catarrho-pneumonia more exactly describes the process. It begins as a
catarrh, {242} sometimes in the nasal passages or post-nasal fossæ,
sometimes in the pharynx, but most frequently in the trachea and large
bronchial tubes, and sometimes rapidly, but oftener more slowly,
travels downward and invades the lining membrane of the air-sacs, which
soon become packed with cells derived partly from emigration of
leucocytes, partly from proliferation of the epithelium lining the
sacs. These cells soon undergo the cheesy degeneration, and, finally
breaking up, as in the case of the scrofulous gland, cause the
formation of vomicæ attended with the familiar signs of pulmonary
consumption. Every step in this process is attended with that abundant
cell-production, and the process itself is marked by that inveteracy
and intractableness, which always characterize scrofulous
inflammations, or rather inflammations in the scrofulous. Occurring as
they most frequently do in young adults, these cases are often mistaken
for pulmonary tuberculosis; and as post-mortem examination generally
reveals a more or less abundant secondary tubercular eruption caused by
absorption of infective material from the centres of cheesy
degeneration and softening, the diagnosis is claimed to be confirmed.
But they are for the most part, nevertheless, cases of genuine
scrofulous inflammation of the bronchial membrane and lining membrane
of the alveoli, and should be called scrofulous pneumonia. It is true
that Mr. Phillips, Mr. Kiener, Villemin, Grancher, Mr. Treves, and
others have collected numerous statistics which would show that
comparatively few of those who had died of pulmonary phthisis bore any
evidence of previous scrofulous disorder. But as the principal evidence
relied upon to prove this fact was an absence of scars resulting from
suppurating glands, their statistics are inconclusive. Besides, it is a
well-known fact that there is a decided antagonism between scrofulous
diseases of all kinds, and a patient who has one severe or well-marked
manifestation of scrofula is not likely to develop another strumous
disease at the same time. The records of the Margate Infirmary for
Scrofula show this fact very strongly, and numerous writers--among whom
may be mentioned Holmes, Birch-Hirschfeld, Walsh, Mr. Treves, and
others--strongly express the same opinion. Indeed, some of them go so
far as to maintain that one form of the scrofulous manifestation
confers protection against others. The question may perhaps be more
clearly stated by saying that the scrofulous, like the non-scrofulous,
have their special predispositions and indispositions to certain morbid
affections, and while one scrofulous child may be specially predisposed
to affections of the bones, joints, skin, or other tissues, it may have
no predisposition whatever to affections of the lungs or lymphatic
glands, etc. This difference in vulnerability or invulnerability of
certain tissues or organs in individuals, whether scrofulous or not, is
so distinctly recognized as a controlling factor in determining the
special form of disease resulting from a given irritant that its
discussion is entirely unnecessary. It is argued against the identity
of scrofula and pulmonary consumption that the commoner manifestations
of the former occur in childhood for the most part, while consumption
is a disease of adult life. But this is readily accounted for by the
different morbid tendencies and exposures in the two periods of life.
"Scrofula tends to appear in early life on account of the unusual
activity of the lymphatic system at that period, and phthisis somewhat
later--at a time, indeed, when the lungs are in more active use, when
{243} sedentary and perhaps unhealthy pursuits are assumed in exchange
for the liberty of childhood, when the modifying influences of puberty
are active, and the structural responsibilities of adult life press
heavily on an organization never other perhaps than frail.... I would,
on the contrary, assert that scrofula and phthisis are as much
manifestations of the same morbid change as acute bubo, acute orchitis,
and acute pneumonia are outcomes of one single process--acute
inflammation."[10]
[Footnote 10: _Scrofula and its Gland Diseases_, by Frederick Treves,
F.R.C.S., Eng.; New York, 1882, p. 62.]