Part 32

SCROFULA.

BY JOHN S. LYNCH, M.D.

SYNONYMS.--Scrophula, Scrofulosis, Morbus scrophulosus, Struma, King's evil, The evil, Quince, Cruels and Crewels (Scotice).

DEFINITION.--A morbid condition of the system manifested by a peculiar liability to certain forms of nutritive disorders of the skin, mucous membranes, joints, bones, organs of special sense, and especially the lymphatic glands.

There is probably no disease of which it is more difficult to give an exact and satisfactory definition than scrofula. The general tendency of medical opinion within the last few decades has been to narrow the significance of the term, and even to restrict it to those slow and indolent inflammations and over-growths of lymphatic tissue which end in caseation and finally imperfect suppuration. Formerly almost every deviation from healthy functional activity in the young, as well as every disorder of nutrition which could not be assigned to any definite cause, was called struma; and thus, as Heule well remarks, "Scrofula became the receptacle into which one vaguely casts all the ailments which afflict children under fourteen years, and of which we do not know the cause."

Before hereditary syphilis was understood all its manifestations were classed as scrofulous, and at least one eminent authority in the United States has expressed the opinion that scrofula is only a manifestation of the syphilitic poison in the second or third generation. Rickets, chronic hydrocephalus, favus, lice, and worms (Lugol), diabetes (Carmichael), and even scirrhus and cancer (R. Hamilton), have all been classed as scrofulous diseases. Then there is a large class of unhealthy persons whose morbid state can be no more definitely expressed than by saying that they are "delicate" or of "feeble health" or of "frail constitution," and by some all these are included under the term scrofulous. But as knowledge advances, and pathological knowledge as well as diagnostic acumen becomes larger and keener, many of these affections and morbid conditions can be eliminated from scrofula and assigned their true pathological and nosological position.

To many who have been educated in the more modern schools of medical thought, therefore, our definition will appear much too broad, while to others it may appear too narrow.

Scrofula is essentially and purely a diathetic, not a cachectic, disease. {232} It is true that what may be called the manifestations or lesions of the disease are often excited by some preceding dyscrasia, and also that the long persistence of these lesions may excite a cachectic condition which we might call the scrofulous cachexia; still, as many children suffer from the lesions of scrofula who have never exhibited any evidence of a precedent dyscrasia, but on the contrary appear to be in perfect health, and many others, on the other hand, show unmistakable evidence of ill-health and are decidedly dyscrasic, yet are never attacked by scrofula, it is believed that every subject of scrofula becomes so not because of any pre-existing dyscrasia or cachexia, but because of some peculiar condition of the system--innate or acquired--which constitutes a diathesis.

"The hypothesis," says Niemeyer, "that scrofula depends upon a faulty composition of the blood (dyscrasia), and that the lesions found in scrofulous persons were due to a deposit in the tissues of a matter circulated by the blood and called a scrofulous material, is almost universally abandoned."

But while insisting upon the peculiar and, so to say, specific origin of the disease in some special condition of the system, without which it will never exist, it is admitted that the lesions of scrofula do not differ essentially from other similar lesions of the same tissues of a non-scrofulous origin. They are mostly of an inflammatory nature, and are only to be distinguished by the often trivial character of their exciting causes--often, indeed, by the total absence of any known exciting cause--and by their tediousness and intractability.

ETIOLOGY.--We believe, as already stated above, that the essential cause of scrofula is some peculiarity in the constitution of the tissues of the scrofulous subject; and we think it highly probable that H. F. Formad of Philadelphia has pointed out what constitutes this peculiarity. He declares--and the correctness of his observation has been abundantly verified--that microscopic examination of the tissues of certain animals characterized by their extreme aptness to be affected by scrofula and tuberculosis, as well as of children known to have been scrofulous or tuberculous, discloses the fact that the lymph-spaces in these subjects are always more numerous, larger, and more crowded with cells than in non-scrofulous subjects. The tissues of the scrofulous are therefore coarser, less compact and solid, and there is a greater tendency to undue cell-growth, than in the non-scrofulous. And these are precisely the characteristics which they present clinically, and such as we might have, a priori, expected to find.

This peculiarity of anatomical structure is in a large number of cases undoubtedly inherited from the parents, but while heredity plays, as is well known, an important part in the etiology of struma, it is not the essential factor. Bad hygienic surroundings, overcrowding, and consequent want of fresh air, improper food, consisting of a too great proportion of starch, during the early months or years of life, will cause the growing tissues to assume the peculiar anatomical arrangement alluded to above. "A coarse diet, containing but little nourishment in comparison with its bulk, is especially held in evil repute. The earlier this injudicious feeding of an infant commences, so much the greater danger that it will become scrofulous; hence the children fed on pap furnish a very important contingent to the army of scrofulous persons." The {233} well-known fact that few children at the breast suffer from scrofulous lesions, but that a large number do so within the first two or three years after weaning, certainly tells in favor of the belief that too much starch and an insufficiency of animal food favor, if they do not actually produce, that faulty nutrition and construction of tissues which we believe lies at the foundation of the scrofulous diathesis. Independent, however, of improper food and the other predisposing causes mentioned, it is quite probable that faulty nutrition caused by accidental disease of the digestive or assimilative organs during infancy may create a predisposition. How else can we account for those not very rare cases in which from parents perfectly free from any scrofulous taint a large family of children may be reared, of which only one will suffer from any scrofulous lesions? Two such instances have been brought to my notice, and as the children in these cases lived upon a farm on the water-side, and enjoyed an abundance of pure air and salt-water bathing, and were certainly not stinted in food of proper quality, it is difficult to account for the acquired diathesis except upon the hypothesis above. Among the general predisposing causes of scrofula in addition to the special ones I have mentioned may be added--

1. Locality and Climate.--It has long been believed that scrofula is more common in the temperate zone than in the extreme north or in the tropics. While this is probably true, it must be stated that a sufficient amount of reliable statistics bearing upon this point have not yet been collected to prove the fact beyond cavil. That we should find that the disease prevails more extensively in cold and damp situations than in warmer and drier ones is to be expected, since the former conditions involve a greater confinement within dwellings, and consequently a diminished supply of fresh air, which, as we have seen, constitutes one of the predisposing causes of scrofula. Moreover, it is in these situations we would encounter a greater number of catarrhs, which, as we shall see, are known to be among the most active of the exciting causes of the glandular affections of scrofula.

2. Season.--For the same reason we find that a large number of cases of scrofula make their appearance in the early spring months, the results of catarrh contracted during the previous winter or of the sudden changes of temperature which accompany the transition of winter to summer.

3. Age.--Scrofula is essentially a disease of early life, but not exclusively so. As the diathesis can only be acquired directly from the parent, or fortuitously by malnutrition during the period of active growth, it follows that it becomes established, if at all, before the age of twenty years. And as the predisposition seems to be quite strong in most cases, and as the exciting causes are more apt to be applied during the earlier years of life, it is not surprising that a very large majority of the cases occur between the ages of three and fifteen years. A few, however, escape during childhood, and only suffer from it between twenty and thirty, while a small number only develop the disease in old age. Rindfleisch mentions the period between twenty and thirty as a common one for the development of hereditary scrofula; and senile scrofula was first pointed out by Sir James Paget. In all these cases of deferred {234} manifestation of the scrofulous diathesis--and they are not very numerous--it is to be presumed that they have escaped the most active of the exciting causes of the disease. Indeed, it is natural that a person having inherited the predisposition should be more sedulously guarded--at first by his parents, and afterward by himself--against the exciting causes of scrofula during infancy and adolescence.

4. Sex.--There is no reason to believe that sex plays any part in the predisposition to this disease. Both sexes seem to be affected in about equal proportions, but from the statistics bearing upon this point it does seem to have some influence in determining the variety of its lesions. Thus, females seem to be more frequently affected with glandular disease, while males suffer from diseases of the joints in the form of coxalgia, white swelling of the knee, and Pott's disease.

5. Condition in Life: Social Position.--If what we have said about the predisposing influence of improper or insufficient food, overcrowding, etc. be true, it will naturally be inferred that a large proportion of the cases of scrofula will be found in the lower strata of society; and this is true. Especially in cities, where the disease prevails most extensively, we always find that the denizens of narrow streets, lanes, and alleys furnish the largest contingent to the deaths as well as the deformities from scrofula. It is here that the poor congregate to avail themselves of the cheaper rents, and here will be found combined all those predisposing causes which may be briefly summed up in one word--poverty. It is true that cases of scrofula are quite numerous in the country, and in a note to Sir Thomas Watson's Practice of Physic (1851) D. Francis Condie quotes from a work on The Nature and Causes of Scrofula, by Phillips, statistics which showed a greater preponderance of deaths from scrofula in a given number of the rural population than a nearly equal urban one. But at the time these statistics were gathered in England (and perhaps now) it is probable that there was a comparatively greater number of abjectly poor people among the rural population than in London, where was congregated such a large number of small tradesmen, artisans, and laborers, who, though not well-to-do, were better paid, and consequently lived better, than the agricultural laborers. Of course, a certain number of cases of scrofula are found in the United States, and perhaps in all other countries, among the children of the wealthy. These, however, are almost invariably caused either by direct transmission from parents or by some accidental injury to the digestive and assimilative organs in early childhood, as we have already pointed out. When it is remembered that in the constantly changing fortunes which are so frequently witnessed in this age of excessive activities, and that in the grand opportunities for obtaining wealth furnished by the liberal institutions and rapidly-growing industries of the United States the descendant of the pauper of the last generation may be the millionaire of the present, it is not surprising that so many who are now wealthy may possess the strumous diathesis as an inheritance from their parents or grandparents, and which they in turn transmit to their offspring.

6. Consanguineous Marriage.--It has long been a popular belief that the offspring of parents closely related by blood are more apt to be scrofulous than when no such relation has existed. Indeed, not only scrofula, but numerous other diseases, deformities, and imperfections have been {235} ascribed to such unions. Idiocy or feeble-mindedness has also been especially accredited to the production of such marriages. But a thorough investigation of this point in England some years ago demonstrated positively that no more idiotic, feeble-minded, or insane children are born of such marriages than of an equal number of marriages contracted between persons not related by blood to each other. There is, however, this amount of truth in the popular belief: if persons closely related to each other possess the scrofulous diathesis, there will be a greater probability--almost certainty--that the diathesis will be transmitted to their offspring. If one parent only is tainted with scrofula, and the other is entirely free from it, there is a possibility--even a probability--that some or all of the children may escape.

7. Complexion and Temperament.--It has been stated by some observers that scrofula occurred principally in the fair-haired, and with equal positiveness by others that it was in the dark-haired that the disease found the most of its victims. Such statistics as have been furnished, however, upon this subject seem to show that there is no connection whatever between scrofula and complexion. It will generally be found that whenever in any country or locality more cases of scrofula occur in persons of one or the other of the complexions, it is only because that particular complexion is the predominant type among the inhabitants of that locality.

8. Race and Nationality.--While it would seem that no race or nation is entirely free from struma, yet there are certainly in the United States two peoples who furnish an enormously disproportionate number of scrofulous cases: these are the Irish and Jews. Among the first of these both scrofula and tuberculosis abound with exceeding frequency, while among the latter it is scrofula alone which seems to predominate. The last, however, are not exempt from tuberculosis, but only exhibit about an equal predisposition to it with their fellow-citizens. It is not difficult to explain the special predisposition of these peoples to scrofula when their past history is taken into account in connection with what has been said about the bad influence of food and surroundings in producing the scrofulous diathesis. The principal food of the Irish peasantry--oppressed and ground into poverty by their Anglo-Saxon conquerors for hundreds of years--have been bread and potatoes, often potatoes alone. It cannot be surprising, therefore, that Irish children fed upon this diet and reared in ill-ventilated hovels should develop the scrofulous diathesis in legions. The Jews, too, oppressed by all nations through ages, have been during many generations reared in poverty and squalor. Even those of them who in not very remote times had acquired by thrift the means of securing both the comforts and luxuries of life dared not live according to their means, lest a show of wealth should attract the unpleasant, often fatal, attention of their rapacious and unscrupulous Christian or Mohammedan neighbors. This condition, this mode of life, has existed among them for many hundreds of years, and has so intensified the strumous diathesis among them that almost the whole race may be said to be patently or latently scrofulous. The negro or African race, however, as observed by the writer in the Southern States of the American Union, do not seem to have developed any special predisposition to struma, notwithstanding their servile condition. This, at first sight, would seem to {236} be contrary to our expectation based on what has been said about Jews and Irishmen. But as my remark has been predicated only on observation of the African in the Southern States, where the climate is not favorable for the development of scrofula, the fact is not so surprising. Besides, the food of these people consisted largely of bacon or pork, fish, milk, and the succulent fruits and vegetables, with a moderate quantity of corn bread, and very rarely potatoes. As the rude cabins in which they dwelt were usually constructed of unhewn logs and covered with rough boards, and cost almost nothing except labor, overcrowding was unknown and ventilation always perfect. The waiter practised medicine fourteen years in Wilcox county (S. W.), Alabama, containing a population in 1870 of 28,377, of whom 21,610 were colored, and during this time saw only two cases of genuine scrofula and one of tuberculosis among the colored population.

Pork as an article of food has often been accused of producing a tendency to scrofula, but evidently with great injustice, for we have seen that the Jews, who never eat it, are almost universally scrofulous, while the Southern negroes, whose staple animal food it was, were conspicuously free from it.

9. Acquired Scrofula.--Although in perhaps a majority of all scrofulous 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." 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.

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.