describe the anatomical appearances of the almost innumerable lesions
met with in the scrofulous. Holding as we do that scrofula is not a
disease per se, but merely a condition resulting from malnutrition and
consequent faulty construction of the tissues during the early years of
childhood, no peculiar or distinct anatomical lesion can be ascribed to
it; and yet every lesion of nutrition as well as of function may have
certain specific characteristics impressed upon it by the scrofulous
diathesis. These may be briefly summed up as great slowness in
evolution, intractableness, incurability, and chronicity of all
pathological processes, and in all inflammatory processes abundant
cell-production and tendency to caseation.
SYMPTOMS, COURSE, DURATION, AND TERMINATIONS.--A great deal of fine
writing has been expended in describing the physiognomy of scrofula,
and for ages writers exercised their descriptive powers upon the type
of face and form supposed to be indicative of the disease. It is almost
needless to say that much of this has been evolved from the
imaginations of the writers, while many of these descriptions are not
pictures of those liable to suffer from scrofulous processes, but of
those who are already the subjects of these, and are simply types, not
of the scrofulous diathesis, but only of the scrofulous cachexia. Many
of these pictures, too, were drawn not from the scrofulous, but the
tuberculous patient, because they were considered identical. Scrofula
is not confined to the dark or the fair, the dull or vivacious, nor
even to the weak and puny or the strong and robust; but all these may
have this faulty and often fatal construction. Nor do we believe that
scrofulous children are either more brilliant or more stupid than other
children. At most we can only say that the scrofulous habit is marked
by a deficiency of blood and a bad nutritive state of the more
important and more highly organized tissues. In some an abundance of
fat is found, giving to the individual a certain amount of plumpness,
which might be thought to be inconsistent with a state of bad health;
in others there is an imperfect development not only of the
subcutaneous fat, but of the skin and muscles also, so that they appear
tender and delicate. In the first of these conditions there is supposed
to be an indolent state of the processes of constructive and
destructive assimilation; in the second, an unnatural activity of these
processes. These differences have led to a classification of scrofula
into the phlegmatic or torpid and the sanguine or erethistic forms,
which Canstatt has thus described: "An unusually large head, coarse
features, a thick chin, a swollen abdomen, enlarged cervical glands,
and flabby, spongy flesh." The erethistic form is said to possess "a
skin of remarkable whiteness, with a tendency to redden easily, and
through which the {244} rose-pink or bluish subcutaneous veins are
visible, a deep redness of the cheeks and lips, blueness of the thin
and transparent sclerotica, which imparts a swimming and languishing
look to the eyes. The muscles of such persons are thin and soft, and
their weight is light in proportion to their stature, indicating a
slightness of their bones. The teeth are handsome and of a bluish
lustre, though long and narrow; the hair is soft."[11] Although this
description may be characterized as diagrammatic, since it describes
rather the extremes and not the mean of the general appearance of the
scrofulous, and numerous cases will be met with that cannot be assigned
to either of the above categories, yet as quite a large number of cases
will be seen that obviously belong to one or the other of these types,
and as, moreover, we shall see that by this classification we shall
obtain valuable data for therapeutic indications, it may be well to
preserve this division of the scrofulous into the lymphatic and
sanguine types.
[Footnote 11: Niemeyer's _Text-book of Practical Med._, vol. ii. p.
741.]
The leading points in the physiognomies of each of these types were
admirably shown in the composite photographs exhibited by Dr. Mohamed
at the last International Congress in England. By some special process
a composite photograph of many faces was, as it were, condensed into a
single picture, in which all that is common remains, all that is
individual disappears. And although Mohamed's pictures were all of
phthisical patients, it must be admitted that the two types of coarse
struma and sanguine struma were strikingly illustrated, and were very
suggestive of Canstatt's descriptions as given above. But it must be
borne in mind that a large number of the strumous belong strictly to
neither of these types, but rather to a medium between the two. "Such a
type would include what is known as pretty struma. The general features
of the individuals so termed belong to the so-called phlegmatic type,
but the coarseness of the features is toned down; the lips would be
called full, not tumid; and a coarse flabbiness would subside into a
pretty, plump condition of the body. The limbs, if not actually
graceful, are at least prettily rounded. The skin may not be thin and
fine, but it is soft, white, and clear. The general expression is not
absolutely apathetic, but would be termed gentle and eminently
feminine. Excellent representations of this type of pretty struma were
also shown in the photographic series above mentioned."[12]
[Footnote 12: Treves, _Scrofula and its Gland Diseases_, p. 84.]
This matter of physiognomy of the scrofulous has this much at least of
practical importance--viz. that to the sanguine or erethistic type
belong those cases that show distinct heredity, while the phlegmatic or
torpid is usually the type assumed in the acquired forms. While there
are doubtless numerous exceptions, it will generally be found that
scrofula in the rich assumes the first, and in the poor the second, of
these forms. It has been asserted that the erethistic form is more apt
to develop tuberculosis or phthisis; and to a certain extent this is
doubtless true, but the torpid are by no means exempt from this grave
accident. The first are undeniably more liable to the more severe and
fatal forms of the disease, which run a more rapid course and are less
amenable to treatment, while in the second phthisis is more apt to be
chronic and incomplete recoveries are by no means rare. The first form
is said to {245} be more frequent in women, while the second is more
frequent in males; and this accords with my own observation and
experience.
There are certain features more or less peculiar to scrofula, besides
those appertaining to the general physiognomy already discussed, which
it may be well to call attention to, since these may aid us in
detecting the scrofulous diathesis even before the grosser
manifestations have declared themselves.
Allusion has already been made to the defective blood-vascular
capillary network in the scrofulous as a necessary consequence of the
excessive predominance of lymph-spaces and lymphatic vessels. Indeed,
there can scarcely be a doubt that the slowness of evolution of various
pathological processes, their chronicity, and the absence of tendency
to resolution and cure of inflammatory lesions, so prominent a feature
in all scrofulous manifestations, is due to this very condition. It is
especially in the coarser type of struma that these defects in the
circulation are most conspicuous. In these the pulse is often below the
average, soft, and wanting in vigor. The cheeks and limbs often assume
a bluish and mottled aspect, due perhaps to a tendency to stagnation of
the blood in exposed parts. The extremities appear swollen as if from
cold, and in the winter generally appear chapped. They are particularly
liable to chilblains, which persist far into the summer and often take
on a very unhealthy action. This last feature is so common as to
constitute an important symptom in scrofula. These defects in the
circulation also probably explain the frequent catarrhs and eczemas
with which such persons are affected, and account also for their
intractableness as well as the unwholesome character of their wounds.
For the same reason (deficient circulation) the temperature is
generally found to be a little lower in the coarsely strumous than in
healthy children, and even in their fevers a very high temperature is
rarely met with. Acute sthenic inflammations are rarely seen, and hence
these persons seldom have acute croupous pneumonias; it is rather the
catarrhal variety, and of this the subacute and chronic forms, which
they suffer from.
Opinions are completely at variance as to the influence of the
scrofulous habit in delaying or hastening menstruation. Lugol referred
to the frequency of dysmenorrhoea among the strumous, and there is no
doubt that the scrofulous as a rule often suffer from suppressed or
scanty menstruation. But it is improbable that the diathesis exerts any
influence whatever in determining the period of puberty in either sex.
We have already stated our belief that the strumous are neither more
intelligent nor stupid mentally than other people. An exception ought
perhaps to be made to this in the case of the exaggerated type of the
coarsely strumous. In these extreme cases we must confess that we have
generally found associated great slowness and dulness of the mental
faculties. If great intelligence and precocity are sometimes met with,
it is only in the erethistic or pretty struma, who, because it is the
delicate one of the family, is petted, has more notice taken of it, and
afforded every facility for the development of the points that make up
the precocious infant. The prettiness of these children, moreover,
attracts more attention to them than to other children or than the bulk
of the sickly would receive.
In young scrofulous children we often observe a considerable amount
{246} of close-lying downy hair upon the forehead, more abundant upon
the sides of the forehead. Upon the arms and back from the occiput to
below the shoulders also a like condition is often seen. Later the
eyelashes appear thicker and longer, and the eyebrows more abundant,
coarser, and longer, than in the non-scrofulous. The color of these is
also apt to be darker than the rest of the hair.
Constantine Paul, as quoted by Treves, has drawn attention to certain
changes in the ears, after they have been pierced for earrings, that he
considers to be diagnostic of scrofula. The mere weight of the earring
seems to cause the puncture to slowly ulcerate, and the ring thus cuts
its way out, either leaving behind it a linear scar or a slit in the
lobule. If the lobule be repaired the ring may cut its way out again,
and this may occur three or four times. These changes seem not so
frequently to be observed in England and America, and may be due in
part to the fact that earrings of greater weight, and more frequently
of base metal, are worn in France than in the countries named. But
still, from what has been said concerning the histology and minute
anatomy of the scrofulous, and the consequent less resistance of the
tissues, this cutting-out process by earrings is just what we would be
led to expect in strumous persons.
The thick upper lip is never absent from the older descriptions of the
physiognomy of the strumous. This is almost invariably present in the
coarse type of struma, and seldom absent even in the erethistic. It is
not always due to irritation from acrid discharges from the nose, as is
maintained by Treves, though doubtless the eczematous and herpetic
eruptions are often caused and maintained by these discharges, and
these may in time cause and increase this thickening.
The teeth in scrofula show nothing that is distinctive, though there is
undoubtedly a tendency to early decay. As this tendency to decay is,
however, so common in many persons who have at least shown no other
evidences of the scrofulous diathesis, no positive conclusions can be
drawn from this fact.
Clubbed fingers, too, so common in persons who have become cachectic
from the long persistence of scrofulous disorders, are not
characteristic. Clubbed fingers and incurvated nails will generally be
found in persons suffering from any disease characterized by slow
wasting. They are seen in phthisis of all varieties, as well as in
cancer, heart disease, aneurism, Bright's disease, empyema. They
therefore have no significance as far as struma is concerned.
GENERAL MANIFESTATIONS OF SCROFULA.--As, according to our view, there
is no such disease per se as scrofula, but simply a diathesis which
impresses its own malign influence upon every other disease with which
the strumous individual may happen to be afflicted, increasing perhaps
the general predisposition to be injuriously affected by all morbific
influences, or impairing the powers of resistance to these, and
especially intensifying any special predisposition which age, sex,
personal peculiarities, occupation, habits, mode of life, or heredity
may have created, we cannot describe any morbid processes as
specifically scrofulous. At most, we can only say that struma is more
apt to impress its malign influence upon certain diseases or upon
inflammations and injuries of certain tissues, that some diseases in
the scrofulous are more apt to be {247} attended by certain
complications and followed by certain sequelæ, and that all of these
are characterized by chronicity and incurability, by slowness of
evolution of pathological processes, and, in the case of inflammations,
by a tendency to profuse cell-production and to rapid caseation. Thus,
measles is apt to be complicated with or followed by otorrhoea, chronic
bronchitis, caseation of bronchial glands, phthisis, and even
tuberculosis; scarlatina by otitis, hyperplasia of the tonsils,
caseation or suppuration of the submaxillary and other lymphatic glands
about the neck, and by chronic catarrh of the renal mucous membranes,
causing dropsy and finally death; eczemas about the face or catarrhs of
the mouth and throat by hyperplasiæ and caseation of lymphatic glands
in the neighborhood. Boils and other subcutaneous inflammations of the
areolar tissue, so common in childhood and adolescence, do not run
their usual rapid course, ending in suppurations and cicatrization, but
become in the one case the scrofulous gumma, degenerating into the
scrofulous ulcer, or if more deeply seated become a cold abscess. A
single injury of a joint, whether mechanical or rheumatic, will
"sometimes take the form of a simple hydrarthrosis, sometimes that of a
so-called tumor albus, while at others it assumes the nature of a
malignant arthrocace, accompanied by suppuration, caries of the
articular surfaces, burrowing of pus, and the establishment of
fistulæ."[13] A slight injury inflicted in the sports of childhood and
soon forgotten--the prick of a pin perhaps--is followed by a disease
sometimes beginning in the periosteum, sometimes in the bone itself,
and presenting at one time the character of periostitis and ostitis,
and at another that of caries or necrosis, or of the two combined.
[Footnote 13: Niemeyer, _loc. cit._]
"As long as the existence of cheesy masses," says Niemeyer, "was
regarded as characteristic of the tuberculous nature of a disease, it
was of course necessary to ascribe many of the inflammations of the
joints and bones of scrofulous persons to a complication of scrofulosis
with tuberculosis."[14]
[Footnote 14: It is a well-established fact, however, that true miliary
tubercles are often found in the neighborhood of bone and joint
affections in the scrofulous, as well as in lupus, in cold abscess, and
in softening caseous glands, which last are considered by many as
specifically scrofulous diseases. It is suggested that an explanation
of this may be found in the probable fact that caseous pus may be
capable not only of producing a general tuberculosis when carried by
veins or lymphatics into the blood, but that it may also set up a local
tuberculosis by a morbid influence exerted upon the neighboring
lymphatics and blood-vessels with which it may come in contact. We are
aware that Wilson Fox (according to the _Medical Times and Gazette_),
captivated by the theory of Koch, has recently recanted his belief in
the inoculability of tuberculosis with anything except tubercle. But we
are afraid that Dr. Fox (who we believe was one among the first to
confirm Ferdinand Cohn's experiments in producing tuberculosis in
rabbits and guinea-pigs by inoculating them with caseous pus) is
suffering from that most active and virulent of all contagions, the
contagion of popular belief. Just now a belief in specific bacilli and
micrococci may be said to be riding upon the crest of a very high wave
of popularity, and we are afraid that many of those who are rushing
forward to mount this wave also will ultimately find themselves
stranded upon that shore which has been strewn with so many wrecks in
the past.]
A simple bronchitis, possessing nothing specific in its origin at
least, will persist and extend to the lobuli of the lung and excite a
catarrho-pneumonia which ends in consumption and death; a simple
intestinal catarrh will result in inflammation and caseation of the
mesenteric glands--a tabes mesenterica; or a simple dysentery,
persisting in spite of the most {248} approved treatment, causes
proliferation and caseation of the endothelial cells of the follicles,
terminating in that obstinate and intractable follicular ulceration
which wastes the strength and wears out the life of the little patient.
A simple conjunctivitis of the globe often will be followed by
ulceration of the cornea, giving rise to intense photophobia, and leave
behind it opacities of that organ which remain a perpetual evidence of
the scrofulous diathesis, if they do not shut out for ever the light
from the eye. Or if it is the palpebral conjunctiva that is affected,
the meibomian glands and follicles of the ciliæ become involved,
destroying the lashes and leaving the lids raw and everted or
inverted--a perpetual deformity. In short, there is no conceivable
disease or injury occurring in what we may call the intensely
scrofulous that does not have impressed upon it some one or more of the
malign characteristics which we have spoken of as indicative of the
scrofulous diathesis. But it is not probable that there is ever any
special disorder or lesion which can be said to be caused exclusively
by scrofula; or, in other words, there is no such disease as a
specifically scrofulous one. Lupus, cold abscess, and particularly
caseous glands, are especially attributed to struma, because they are
often thought to make their appearance independent of any assignable
cause; but as boils, eczema, impetigo, and numerous other affections of
the skin and areolar tissue affect children who are not scrofulous, and
equally independent of any known causes, the argument is not
conclusive. Besides, all these affections occur sometimes in the
non-scrofulous; and even caseation of a single inflamed gland quite
often occurs in children who are weak or in ill-health, but who show no
other evidences at that or at any other time of the scrofulous habit.
DIAGNOSIS.--The only affection likely to be mistaken for scrofula is
congenital or acquired syphilis in its later manifestations. In this
disease we see the same tendency to increased cell-production, the same
tedious, slow, and intractable inflammations and ulcerations, which are
characteristic of scrofula. And this apparent similarity has induced
many persons to believe that scrofula is nothing else than syphilis in
the second or third generations. But in congenital syphilis the lesions
usually make their appearance soon after birth or are present at birth,
and long before even hereditary scrofula begins to show its malign
influence. In most cases, too, a history of syphilis can be obtained,
and even when this is not obtainable a few inunctions or fumigations
with mercury, in connection with a few large doses of iodide of
potassium, will very quickly decide the question of diagnosis for us.
In the case of lupus, in which Erichsen admits there is no means of
positively distinguishing the syphilitic from the so-called scrofulous
varieties, the diagnosis is more difficult. But as this disease appears
later in life than the more ordinary scrofulous manifestations--when,
therefore, a history of syphilis can generally be obtained if there is
one, and when there would almost certainly be also a history of
scrofula if it existed--it would seem that the diagnosis even in this
case cannot be so difficult. Diagnosis here, however, is of little
consequence, since the treatment recommended for both forms is the
same.
PROGNOSIS.--This of course depends upon the nature of the special
lesion. The simpler lesions incident to childhood, such as glandular
{249} hyperplasiæ, catarrhs, eczemas, impetigoes, etc., usually do well
under appropriate treatment and proper hygienic conditions. Diseases of
joints, bones, mesenteric glands, etc. often terminate fatally or
result in serious deformities and permanent impairment of function. Not
infrequently diseases of the bones and articulations, attended with
profuse and protracted suppuration, cause amyloid degeneration of the
liver, kidneys, spleen, or other glandular organs, and, as a
consequence, death. Catarrho-pneumonia in a scrofulous subject almost
invariably causes phthisis sooner or later. Occasionally the caseated
cellular exudation in the air-sacs remains quiescent for months, and
even years, the patient remaining quite well except for a harassing
cough during the winter months; but sooner or later the caseous mass
will soften, the symptoms of active consumption ensue, with fever and
wasting, and death closes the scene. Far more frequently, however,
softening and suppuration follow swiftly upon the caseous degeneration,
and the whole process occupies a period of only a few months.
Tuberculosis especially runs a rapid course in these subjects, and
while a few perhaps only develop tuberculosis of the lungs--in which
case the duration of the disease may be a little longer--in by far the
larger number there is a generalization of the tubercular process which
puts a speedy end to their existence.
TREATMENT.--This may be most profitably discussed under two
heads--prophylactic and therapeutic.
Prophylactic.--Scrofulous persons who are closely related by blood
should be earnestly advised not to intermarry. We have so often seen
the deplorable results upon offspring of such marriages that we cannot
too strongly urge this upon the profession. Such persons should be
frankly and clearly told what are most likely to be the consequences of
such marriage, and all possible moral influences should be exerted to
prevent them. The canons of the Church wisely interdict such marriages,
but, unfortunately, its ministers seldom attempt to enforce them, or if
they do their efforts are made ineffectual by the facility with which
the marriage-rite can be obtained from civil officers in most of the
States of the American Union. The medical profession can do more than
any other class to diffuse knowledge and create a correct public
opinion upon this subject, but, unfortunately, it too often neglects
this important mission.