Part 33

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, "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.

"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."

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."

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."

It is entirely unnecessary--and indeed it would be too tedious--to 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." 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.

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."

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.