Chapter XIX: Front Matter (19)

A second group of erosions affects the free edge of the tooth, and includes the Hutchinson teeth, with several less important varieties. Dental erosions are multiple, symmetrical, maintain the same level on the crowns of corresponding teeth, and are situated at different heights on the crowns of teeth of different classes. It is evident, therefore, that they are the result of a morbid influence of a general character. There are three theories as to their etiology: (_a_) that they have no relation to syphilis,[140] but are always connected with infantile eclampsia; (_b_) that they are exclusively the result of hereditary syphilitic influence; and (_c_) that they are simply ordinary lesions originating from syphilis with marked frequency, and even in one form--the Hutchinson tooth--appearing to originate only from it. This latter view is the one adopted by Fournier himself. Continuing to group the symptoms due to arrest of growth and modification of structure, we have, second, _microdontism_, or dwarfing and stunting of the teeth--pegged teeth; third, _dental amorphism_, in {299} which the teeth are strangely distorted or even transformed in type; fourth, _dental vulnerability_, or extreme susceptibility to all traumatic or disintegrating influences.

[Footnote 140: M. Magitot, _Treatise on the Anomalies of the Dental System_, Paris, 1877; _Clinical Studies on Erosion of the Teeth considered as a Retrospective Sign of Infantile Convulsions_, Paris, 1881; Castanié, Paris, 1879, Thesis No. 384; Rattier, Paris, 1879, Thesis 569; and others.]

Interstitial Keratitis.--The frequency of this form of diffuse inflammation of the cornea, and the diagnostic significance which has been so positively attributed to it--and has been as positively denied--render it of special interest to the general practitioner, who is almost certain to meet with occasional cases, and should be prepared to recognize its possible relation with other, and often graver, conditions.

It begins, commonly, as a slight, diffused haziness situated in the substance of the cornea itself, usually not far from the centre, and at first affecting only one eye. This depends at this stage on the presence of a number of little distinct dots of inflammation, limited to circumscribed, almost microscopic, areas, but later, in a few days, these coalesce, and at the end of a few weeks the whole cornea will probably have become nearly or quite opaque, looking like ground glass. There is no ulceration, and but little congestion as compared with that seen in other inflammatory diseases of the eye, although in the majority of cases there is a fulness of the ciliary vessels and a little photophobia with pains around the orbit. This condition may persist for one or two months, after which the other cornea is nearly always attacked,[141] and is similarly affected, although the disease is apt to pass through its different stages rather faster than in the first eye.

[Footnote 141: In 91 out of 102 cases in Mr. Hutchinson's series--in 6, the left alone; in 5, the right alone (_op. cit._, p. 123).]

When the height of the disease is reached the corneæ are nearly opaque, a bare perception of light remaining, so that the patient is just aware of the difference between its presence and absolute darkness. Then the cornea which was first involved begins to clear; this is soon followed by improvement in the other one, which in the course of a year or two results in a return to fairly good sight, although in most cases there remain a slight haziness and an abnormal expansion of the cornea.

This favorable result is much influenced by the character of the case, which is sometimes very mild from the outset, and by the thoroughness of the treatment. It is sometimes complicated with iritis, kerato-iritis, cyclitis, posterior choroiditis, secondary glaucoma, etc., in which cases of course the prognosis is most unfavorable. Even in ordinary cases it should be guarded in respect to perfect restoration of function, as clearing of the corneæ may reveal adhesions from iritis or spots of choroiditis disseminata, which could not, of course, have been previously detected. In very mild cases, however, without much evidence of involvement of the other tunics, and which have been submitted to treatment early, it is not uncommon for the cornea to regain almost perfect transparency.

The diagnosis of this condition may generally be made with ease. The ground-glass appearance in the earlier stages and the dull pink or salmon color in the more vascular stage are very characteristic. The vascularity differs from that attending other chronic forms of keratitis, granular lids, etc., in which the vessels are large and superficial, in that in the syphilitic keratitis they are much deeper and very closely interwoven, so that the effect is almost that of an ecchymosis. In other cases both eyes are not so apt to be affected, nor is the tendency to spontaneous cure {300} so marked. The absence of ulceration and the very slight degree of accompanying sclerotic or ciliary congestion are also valuable features.

The conditions which Mr. Hutchinson has known to be most frequently mistaken for it[142] are certain forms of inflammation following small-pox and very superficial ulcers in a healing stage, together with cases of vascular conjunctiva.

[Footnote 142: _Op. cit._, p. 128.]

There is also said[143] to be some difficulty in distinguishing it from a form of non-syphilitic relapsing cyclitis with corneal opacities and iritis. This, however, is a disease of adults, often limited to one eye, with a strong tendency to relapse, the opacities being more abruptly defined and limited to the region near the circumference.

[Footnote 143: Edward Nettleship in Hill and Cooper, _op. cit._, p. 267.]

The chief point of interest, however, in the diagnosis of interstitial keratitis is its association with other symptoms of syphilis, upon which, for the general practitioner at least, the diagnosis will usually depend.[144] Mr. Hutchinson's conclusions, drawn from an analysis of 102 cases of interstitial keratitis, bear strongly upon this point, and are as follows:[145] A large proportion of all cases occur in patients between the ages of eight and fifteen, the disease being comparatively rare in early childhood, and still more so after adult age has been reached. He never saw it begin after the age of twenty-six. The patients presented the physiognomical peculiarities of inherited syphilis (see p. 313). In more than half the cases the previous history, especially as regards infancy, was one of hereditary syphilis, and in many instances there was a clear history of infantile syphilis in brothers or sisters. In half the cases no questions were asked as to the existence of venereal disease in the parents prior to the birth of the child. In 29 out of the other half such disease was freely admitted. All the patients had lost in early life nearly half their brothers and sisters. Omitting miscarriages and premature births, it was found that 77 mothers had borne 547 children, of whom only 284 remained alive--an excessive rate of mortality. It appeared in the large proportion of cases in the eldest children in their respective families--a circumstance to be expected in view of the usual behavior of hereditary syphilis in families. Undoubted syphilitic lesions, such as nodes, ulceration of the palate, etc., are not infrequently associated with the keratitis.

[Footnote 144: "To those who have not had opportunities for observation at an ophthalmic hospital I would recommend that the diagnosis should be held to be doubtful if the patient does not present the peculiarities of teeth and physiognomy which I have described, since we find that the latter are the almost invariable concomitants of the true disease" (Mr. Hutchinson, _op. cit._, p. 128).]

[Footnote 145: _Op. cit._, pp. 109-129.]

Examination of large numbers of cases has, I believe, established the general accuracy of these statements; and although there is still much difference of opinion as to the exact relation between syphilis and this form of keratitis--whether, for example, it is a symptom of syphilis itself or of a cachexia frequently produced by syphilis, but often by other systemic diseases affecting nutrition--yet, on the whole, so far as I am able to judge of the question outside of its purely technical and ophthalmological relations, I think the weight of modern authority is chiefly on the side of a distinct and practically invariable relation of cause and effect between inherited syphilis and the corneal inflammation.

{301} Mr. James Dixon,[146] for example, proposes to call the disease syphilitic keratitis, and says it is met with exclusively as a sequel of an inherited taint. He adds: "We may meet with some syphilitic keratitis in patients with healthy physiognomy and deformed teeth, or, still more rarely, in those with faultless teeth and the syphilitic cast of features; but to find the true form of keratitis in connection with both good teeth and good complexion is, I think, next to impossible." Many other ophthalmologists express themselves to the same effect more or less strongly. Nettleship,[147] Noyes,[148] Förster,[149] Macnamara,[150] De Wecker,[151] and Carter[152] may be mentioned as having ranged themselves upon this side. On the other hand we have Schweiger,[153] Maunther,[154] Sæmisch,[155] Soelberg Wells,[156] and others who are not convinced that syphilis is the sole nor even, in the opinion of some of them, the principal cause of this disease.

[Footnote 146: Article on "Diseases of the Eye," Holmes's _System of Surgery_, Am. ed., vol. ii. p. 71.]

[Footnote 147: _Op. cit._]

[Footnote 148: _Text-book of Ophthalmology_.]

[Footnote 149: _Handbuch der gesam. Augenheilkunde_, vol. vii. p. 186, 1876.]

[Footnote 150: _Op. cit._]

[Footnote 151: _Ocular Therapeutics_, trans. of Forbes, 1879, p. 124.]

[Footnote 152: He even asserts the converse to be true, which is going beyond what I believe can be established in regard to the invariable connection between the two diseases. He says: "The subjects of what we call simply inherited syphilis are liable--nay, are almost sure--to suffer from a peculiar form of interstitial keratitis."]

[Footnote 153: "Hutchinson's view, that this form of keratitis is to be regarded as the direct consequence of congenital syphilis, has not been generally accepted in Germany" (_Handbook of Ophthalmology_, p. 298).]

[Footnote 154: Zeissl's _Jahrbuch der Syphilis_, 1875, p. 288.]

[Footnote 155: Graefe and S.'s _Handbuch d. Augenheilkunde_, 1875, vol. iv. p. 264.]

[Footnote 156: _Diseases of the Eye_, p. 138.]

Probably the conclusions of Jullien[157] most nearly express the views of those who do not accept in its entirety the syphilitic theory of the disease. He concludes--(1st) that interstitial keratitis coincides frequently, but not invariably, with syphilitic derangement of the dental apparatus; (2) that it is associated with a feeble constitution and with malnutrition, and is thus produced indirectly by scrofula, rheumatism, or syphilis, each of which by its debilitating influence may give rise to a diathesis which favors such morbid developments.

[Footnote 157: _Op. cit._, p. 1013.]

As a specimen of the evidence which is produced by observers other than Mr. Hutchinson--who, as he himself observes, may be suspected of "that bias which almost necessarily warps more or less the judgment of one who supposes himself to have noted something new"[158]--the statistics of Förster,[159] who apparently takes an unbiassed view of the question, may be noted: In a total of 214 cases of interstitial keratitis evidence of hereditary syphilis other than the corneal disease was noted in 146 (68 per cent.). This number was made up as follows: _a_, evidence of syphilis in parent, 17; _b_, evidence of hereditary syphilis in other members of the family, 14; _c_, evidence in the patient other than keratitis, 115; characteristic teeth, 69; evidences in physiognomy, bones, skin, palate, or choroid, 46. In the remaining 68 cases (32 per cent.), though inheritance of syphilis was not proved, there was, almost without exception, strong suspicion of that disease apart from the keratitis.

[Footnote 158: _Op. cit._, Preface, p. x.]

[Footnote 159: Graefe and Sæmisch's _Handbuch_, vii., Part I.]

The condition of the permanent central upper incisors was noted in 138 of the cases; of these they were typical in 73 (53 per cent.); suspicious in 34 (24 per cent.); normal in 31 (23 per cent.). In 15 cases {302} the permanent teeth had not been cut, and in the remaining 61 the state of the teeth was not recorded.[160]

[Footnote 160: Note by Mr. Nettleship, Hill and Cooper, _op. cit._, pp. 263, 264.]

This seems to me such direct and such unequivocal clinical evidence that it is safe to say, as of the question of syphilitic teeth, that the burden of disproof rests with the doubters, and that we may venture the assertion that interstitial, diffuse, or parenchymatous keratitis is a symptom of inherited syphilis, and that the unmistakable presence of the former disease is sufficient proof of the existence of the latter.

Syphilis of the Nerve-Centres and Nerves.--Until a comparatively recent period our only guide to the course and progress of the nerve diseases of inherited syphilis was to be found in analogy. We knew, for instance, that in acquired syphilis three forms of cerebral disease could be recognized in a general way--one characterized by sudden attack of paralysis, in which the lesion was usually thrombosis from specific endo-arteritis; one in which the symptoms of brain tumor were present, and in which gummata were the cause of the difficulty; and one in which pain, headache, and various functional or convulsive disturbances--chorea, epilepsy, paralysis of single nerves, etc.--were the customary phenomena, and in which periosteal, meningeal, or neuroglial thickenings constituted the pathological basis. The last two are often intermingled both symptomatically and histologically.

Heubner[161] divides cerebral syphilis into three groups, two of which very closely resemble those I have described. In one, however, he includes both the general physical disturbances, incomplete paralysis, and final coma characteristic of tumor and the epileptiform attacks so often due to peripheral or meningeal irritation. This combination is explained by the results of his autopsies, which disclosed in 26 cases in which these symptoms were conjoined a gummous growth in the pia mater of the convexity of one of the cerebral hemispheres, either limited and superficial or involving more or less of the cortex and forming a distinct tumor. The epileptiform attacks were present in 19 out of these 26 cases, while in 20 other cases where the growth was limited to the white substance at the base of the brain they were present only twice. This second form is the apoplectic, followed by general hemiplegia, and depending on disease of the cerebral arteries. His third division is a very ill-defined one, depends much for its limitations upon subjective symptoms, and is of no special interest as applied to the subject of inherited syphilis.

[Footnote 161: _Ziemssen_, vol. xii.]

Althaus[162] also makes three divisions, two of which are as follows: (1) Cerebral tumor--a gumma either hard or soft. There are then nocturnal headache, sleeplessness, epileptiform attacks, the various phenomena produced by involvement of the cerebral nerves, etc. (2) Disease of the arteries, apoplexy, or softening, followed by hemiplegia.

[Footnote 162: _Medical Times and Gazette_, Nov. 10, 1877.]

Hutchinson makes a similar division of lesions and symptoms,[163] and the observations of Jaksch, Wilks, and Hughlings Jackson more or less closely coincide with this general classification.

[Footnote 163: _Ibid._, Feb. 17, 1877.]

Now, in spite of certain striking differences--more apparent than real, however--between inherited and acquired syphilis as regards cause, {303} duration of stages, etc., the essential pathological changes are the same. When syphilis in its later periods attacks the brain or spinal cord or nerve-trunks or vessels of a foetus, it proceeds just as in the adult, the same characteristic accumulation of cells taking place and setting up an arteritis or a meningitis, thickening the sheaths of nerves, or constituting a pericranial node or a gumma according to their number and their situation. We would accordingly expect to find in subjects of inherited syphilis manifestations closely allied to those observed in the adult; and the observations of Barlow,[164] Graefe,[165] Jackson,[166] Heubner,[167] Dowse,[168] and Hutchinson,[169] though comparatively few in number, have already demonstrated the correctness of this supposition.

[Footnote 164: _Trans. of Path. Soc. of London_, vol. xxviii., 1877.]

[Footnote 165: _Archiv für Ophthalmologie_, Bd. 1, Ab. i.]

[Footnote 166: _Journal of Mental Science_, Jan., 1875.]

[Footnote 167: _Ziemssen_, vol. xii.]

[Footnote 168: _Syphilis of the Brain and Spinal Cord_, London, 1879, chapter on "Hered. Syph.," p. 67.]

[Footnote 169: _Med. Times and Gazette_ (? Feb. 17, 1877).]

We find, thus, that in these patients meningitis, growths, and arterial disease constitute the three clinical divisions of the disease which have thus far been distinctly differentiated, and the reported cases, with or without autopsy, fall naturally into these classes.

These cases are naturally few, and to make them absolutely reliable it is necessary to have unmistakable evidence of hereditary syphilis in some other form and the demonstration of syphilitic lesions at an autopsy. The case of Dowse, however,[170] includes these requirements. A child twelve years of age, of syphilitic parents, with a history of coryza, sore eyes, and a tubercular syphilide, was attacked with epilepsy, diplopia, facial paralysis, etc., and finally died. At the autopsy three gummatous growths of the surface of the brain were found, and the vessels of the base were found to have undergone the special changes described by Heubner. Their lumen was in some places nearly occluded by an accumulation of spindle-shaped cells between the tunica fenestra and the epithelial lining; and interspersed with them, but particularly in the muscular and adventitious coats, were to be seen enormous quantities of round cells which in many parts seemed actually to replace the normal structures. Dowse's other cases are not at all conclusive in their clinical histories; even the diagnosis was not established by autopsy.

[Footnote 170: _Op cit._, pp. 71-75.]

Barlow's two cases were both very young children, and are extremely convincing.[171] A child four months old, with snuffles, serpiginous ulcers, etc., and with a syphilitic father, had epileptiform attacks, followed by laryngismus, carpo-pedal contraction, and changes in the choroid. She died aged about fifteen months, and the autopsy disclosed thickening of the pia mater, evidently not tubercular, and changes in the arteries, which in the gradual narrowing of the lumen of the vessel, the absence of ulceration or disintegration or calcification, and the continuity and extent of the cell-proliferation are as different as possible from ordinary atheroma, but correspond precisely with the description of Heubner's cases, which were undoubtedly the subjects of acquired syphilis.

[Footnote 171: _Transactions of the Pathological Society of London_, 1877, vol. xxviii. pp. 287-291.]

In the second case the symptoms were associated chiefly with the cranial nerves. These were nystagmus, paresis of facial muscles, laryngeal spasms, etc. He died at the age of fifteen months. The vessels of the base were extensively diseased as in the other case, and the fourth, {304} fifth, sixth, seventh, and eighth pairs of nerves were smaller by a new growth of a gummatous nature which had produced almost entire atrophy of the nerve-cylinders. There were cicatrices of the liver and spleen. Cases in which the diagnosis rested upon the history, upon the co-existence of undoubted syphilitic symptoms, and upon improvement under specific treatment are by no means rare.

Berkely Hill reports[172] a typical case of syphilitic epilepsy. A girl aged nine was the first child of the family that had lived, two having previously been born dead. She presented characteristic teeth, traces of choroiditis, and, while under treatment, suffered from both keratitis and iritis. Her first fit was when she was four years of age, and the attacks had recurred frequently since that time. The convulsion was confined to the left side. The left arm was very weak, the weakness having come on gradually, and being especially great in the extensors of the wrist. Under specific treatment the attacks ceased entirely. In this case there was certainly organic disease, probably a gumma on the surface of the right hemisphere.

[Footnote 172: _Op. cit._, p. 253.]

Other cases reported by the same author, numerous instances of nervous troubles in inherited syphilis reported by Hughlings Jackson,[173] Fournier,[174] Henoch,[175] and Hutchinson,[176] warrant the statement, then, that the nervous diseases of inherited syphilis fall into the same general category as those of the acquired disease; that they may appear at any age, from three or four months to that of puberty; that they depend for their production upon a cell-proliferation which, according to its locality, results in the development of new growths, the production of localized meningeal inflammations, or the obstruction and inflammation of arteries; and that the symptoms are those usually associated with such pathological changes, the diagnosis of syphilis depending chiefly on the history and the presence of other specific phenomena.

[Footnote 173: _Journal of Mental Science_, Jan. 8, 1875; _Transactions of St. Andrew's Med. Graduates' Ass._, vol. i., 1868.]

[Footnote 174: _Annales des Derm. et Syph._]

[Footnote 175: _Nouveau Dict. de Méd. et Chirurgie_, p. 885.]

[Footnote 176: _Op. cit._]

The prognosis is more unfavorable than in similar cases in acquired syphilis, the meningeal and vascular lesions being the most frequent and showing themselves very obstinate even under careful treatment. Spinal troubles, chorea, and idiocy have been attributed to inherited syphilis, but must occur with great rarity.

Hill[177] reports a case of a child aged five years who had been hemiplegic since he was two and a half years old, and who developed paralysis of the flexors of the ankle in each leg.

[Footnote 177: _Op. cit._, p. 254.]

Keyes reports a case of a boy five years of age, with nodes on tibiæ and other signs of inherited syphilis, who had two attacks of paraplegia.

Hill reports a case of imbecility associated with inherited syphilis, but there is no evidence that it was other than a coincidence.

Fletcher Beach found not more than 1 per cent. of syphilitic children in the Dareult Asylum, and Mr. Mercier could only trace syphilis in 5 out of 220 female idiots, probably imbecile from birth.[178]

[Footnote 178: _Ibid._, p. 255.]

Hughlings Jackson only found 1 case of inherited syphilis among 80 {305} cases of chorea. The most carefully recorded cases of the latter affection associated with hereditary syphilis are two reported by Alison.[179]

[Footnote 179: _American Journal of the Medical Sciences_, July, 1877.]

Syphilis of the Spleen.--Disease of this organ in inherited syphilis is especially important from two points of view. It is a valuable aid to diagnosis, and by its size and the degree of persistence of the swelling gives an approximate indication of the severity of the case.

Attention was first called to the frequency and importance of enlargement of the spleen in early hereditary syphilis by Gee in a paper read before the Royal Medical and Chirurgical Society in 1867.[180] He gave the histories of thirteen children in support of the statement that such enlargement occurred in almost one-fourth of all cases of hereditary syphilis, sometimes with, sometimes without, enlargement of the liver and lymphatic glands. According to him, the degree of splenic enlargement may be taken as a sort of index of the severity of the cachexia; the majority of cases with great enlargement die, but sometimes such children survive, the spleen gradually diminishing in size as the health improves--not diminishing, however, pari passu with such improvement, but remaining for a long time "a monument of past cachexia."

[Footnote 180: _British Medical Journal_, 1867, vol. i. p. 435.]

Barlow,[181] ten years later, thought Gee had rather understated the proportion of cases in which splenic enlargement occurs, he having found it in 22 out of 28 children with definite hereditary syphilis. Birch-Hirschfeld, Eisenschitz, and Tepel[182] corroborate these observations, finding that the enlargement is almost invariable and that the spleen is often double its normal size.

[Footnote 181: _Trans. of Path. Soc. of London_, Jan. 20, 1877.]

[Footnote 182: Quoted by Hill and Cooper, _op. cit._, pp. 164, 165.]

Mr. W. J. Tyson has reported[183] a cure of a child born of syphilitic parents, in whom, at two years of age, the spleen extended downward three and a half inches, reaching the crest of the ilium and approaching closely to the umbilicus. The liver was not enlarged; the urine was not albuminous. He ordered mercury with chalk, one grain every morning and evening, and one grain of iodide of potassium, with ten minims of syrup of iodide of iron to an ounce of water, three times a day. Sixteen months later the spleen had become imperceptible, and three years afterward the child was in excellent health.

[Footnote 183: _The Lancet_, Oct. 23, 1880.]

The diminution of the liver under treatment appears to take place before there is any diminution in the size of the spleen.[184] This persistence of the latter renders it, as has been stated, a valuable diagnostic sign. In the paper already quoted from,[185] Macnamara and Barlow allude to this as follows: Enlargement of the liver, although it ought to be noted because it is often present in hereditary syphilis, has but little value as a confirmatory symptom--first, because the liver is proportionally large in infancy, and it is difficult to state the limit of what is actually normal; and, secondly, because other causes besides congenital syphilis lead to its enlargement.

[Footnote 184: Barlow, _British Medical Journal_, Jan. 20, 1877.]

[Footnote 185: _British Medical Journal_, Dec. 16, 1882.]

With regard to enlargement of the spleen the case is different. Gee's observation, that in the early stage of infantile syphilis some enlargement of the spleen occurs in a large number of cases, has been abundantly {306} confirmed. Although with the subsidence of the other symptoms this enlargement often disappears, so that on post-mortem examination two or three months after there may be no trace of it, yet in a few cases it persists, and indeed sometimes increases, so as to be considerable when the other signs have quite vanished. The importance of this sign is greatest when noted early; as, for example, when the child is from two to three months old, for at that period the enlargement of the spleen due to rickets can hardly come into question.

The condition of the spleen during this period of enlargement seems to be simply that of hyperæmia, or at the most of hyperplasia, Gee's, Barlow's, and Birch-Hirschfeld's autopsies showing no evidence of new growth or of amyloid or other changes.[186]

[Footnote 186: Parrot reports (_Le Mouvement méd._, Paris, Nov. 23, 1872) two forms of splenic disease produced by inherited syphilis: 1. A simple hypertrophy, which he thinks is secondary to diffuse infiltration of the liver, obstructing the portal circulation and causing the spleen to act as a reservoir; 2. An inflammatory condition resulting in the formation of false membranes around the capsule. His explanation of the first condition is unsatisfactory, because there are numerous cases in which the spleen is enlarged without any involvement of the liver. His other observations have never been confirmed.]

The cause seems to me to be in all probability the well-known effect of syphilis on the glandular system in general, and the lymphatic system in particular, to which I believe the spleen is now generally assigned. The analogy between this slow, persistent, painless enlargement preceding the cutaneous symptoms,[187] unaccompanied by inflammatory symptoms, unattended by any breaking down of tissue, subsiding slowly but evenly under specific treatment, and the behavior of the buboes of acquired syphilis, is certainly very striking.[188]

[Footnote 187: Eisenschitz, _Wiener med. Wochenschrift_, Nos. 48 and 49.]

[Footnote 188: A similar enlargement occurs, but much more rarely, in the secondary period of acquired syphilis. Weaver noted it in 3 out of 79 soldiers suffering from early syphilis. Wilks and Moxon report cases in which the average weight was 19 oz.]

In most cases of hereditary syphilis there are evidences of disturbance of the gastro-intestinal tract. Vomiting, diarrhoea, colic, anorexia, and emaciation are well-known, but of course not at all characteristic, symptoms.

It has been supposed that the mucous membrane of the entire tract was probably, during the early period at least, and coincidently with the cutaneous eruption, in a condition of hyperæmia and irritation comparable to that of the skin.[189] Whether this be so, or whether it is due to associated involvement of the glandular apparatus, has not yet been determined.

[Footnote 189: "There is surely no a priori probability that a blood disease so severe as syphilis should produce lesions on the skin, in the mouth, and in the eye only--that it should, in fact, affect all the visible parts and avoid all the concealed ones" (Mr. Hutchinson, _The Lancet_, Feb. 6, 1876).]

Förster[190] has found fibroid degeneration of Peyer's patches in a syphilitic infant who died six days after birth, the glandular structure having been replaced by elevated grayish-red masses of nuclei, cells, and connective-tissue fibre. Ulcers of the intestines have been described, but appear to have had no specific characters.

[Footnote 190: Quoted by Bumstead and Taylor, _op. cit._, p. 757. His observations were confirmed by Eberth, Roth, and Oser.]

The pancreas has been most extensively studied by Birch-Hirschfeld, who examined seventy-three syphilitic foetuses. In thirteen of them he {307} found enlargement of the pancreas with increased weight and density, proliferation of connective tissue, and in some cases compression--almost obliteration--of the lobules, with atrophy and fatty degeneration of their epithelium.[191]

[Footnote 191: Klebs discovered a gumma in the pancreas of a six-months' foetus.]

The peritoneum has been described by Simpson, Von Baerensprung, and others as occasionally invaded in early hereditary syphilis. There seems to be no evidence that it is ever directly affected, the cases in which death occurs from peritonitis being due usually to trouble connected with the liver or spleen.

Syphilis of the lung, originally described by Depaul and Virchow, has been carefully studied also by Förster, Robin, Lorain, and Cornil, from whose description[192] of the pathology of the condition I condense the following: In the syphilitic foetus born before term, in the syphilitic child born dead at full term, and in the syphilitic children who live a few days, there are found at the autopsy, in the lungs, nodules or tumors, usually superficial, sometimes deep, hard, isolated or in groups, pink, gray, or red in color, with scattered whitish or yellowish points. Their size varies from a pea to a small walnut. They represent a portion of the lung more or less considerable in a state of special lobular hepatization. Sometimes a whole lobe is involved. The affected portion is very dense and covered with thickened inflamed pleura. The lesions are those of chronic pneumonia. The interlobular connective tissue enters into proliferation and presents a large quantity of embryonal cells; the alveolar walls are thick, while the narrow alveoli are livid, and even filled by epithelial cells, which are of the pavement form in contact with the walls, round in the centre of the alveoli. As the process progresses the epithelial cells become fatty, degenerated, and subsequently broken down and absorbed, while the embryonal interalveolar tissue rapidly organizes into fibrous tissue. Thus results a small fibrous tumor, in which a gumma may ultimately develop.[193]

[Footnote 192: _Syphilis_, trans. of Simes and White, Philada., 1882, p. 404.]

[Footnote 193: Cornil and Ranvier's _Path. Histology_, Am. ed., 1880.]

It is impossible to confound this syphilitic pneumonia with tuberculosis. The granulations of tubercle are never congenital.

The fact that in syphilitic interstitial hyperplasia the change begins in the interlobular connective tissue and around the interlobular vessels, at first consisting of small spindle-shaped and roundish cells which quickly develop into connective tissue, and the fact that blood-vessels are freely produced among the fibres of this new tissue, seemed, in the estimation of those pathologists who took part in the discussion on visceral syphilis in 1877, to constitute its most distinctive feature.[194]

[Footnote 194: _Trans. of Lond. Path. Soc._, vol. xxviii.--views of Green, Jones, Greenfield, Moxon, and others.]

Gummata in the lungs of children suffering from inherited syphilis have been described by a number of writers. They appear likewise to begin in the walls of the blood-vessels or the bronchioles. They differ from tubercular nodules in being few in number--not more than half a dozen usually--and are generally confined to one lung.

The condition of the arteries in the few cases in which they have been noticed as affected by inherited syphilis was precisely similar to that found by Heubner in the arteries of adults.[195]

[Footnote 195: See Cornil, _op. cit._, p. 305.]

{308} The symptoms are very variable; new-born children often die asphyxiated in a few days. If they live longer, the disease develops into a more generalized broncho-pneumonia.

Syphilis of the Larynx.--The hoarse cry of the new-born infant so characteristic of hereditary syphilis depends upon the presence of hyperæmia, of mucous patches, or even of extensive ulceration. I am inclined to think that the first is the more common, as if it were otherwise cases of death from oedema glottidis or other forms of laryngeal obstruction would be oftener met with. When ulceration does exist it is generally, but not invariably, secondary to pharyngeal ulcers.[196]

[Footnote 196: See synopsis of six cases of George M. Lefferts, reported in Bumstead and Taylor, _op. cit._, p. 754.]

Bronchial catarrh, giving rise to cough, and sometimes to considerable embarrassment of respiration, is a not infrequent complication of laryngeal syphilis.[197]

[Footnote 197: Schnitzler, _Die Lungen Syphilis, etc._, 1880, S. 41.]

Later troubles of the larynx in connection with inherited syphilis have not yet been carefully enough studied to warrant us in drawing any distinction between them and the usual symptoms seen in the acquired disease.

Syphilis of the testicles has been studied by Henoch,[198] Cornil,[199] Parrot,[200] Hutinel,[201] North,[202] Bryant,[203] and others. It is found to consist of a true interstitial orchitis, very closely resembling that seen in the syphilitic testicles of adults. Hutinel's investigations, based on ten cases, showed the testicles slightly enlarged and harder than normal, the scrotum pendent, the epididymis normal, the tension of the tunica vaginalis and tunica albuginea slight. The basis of the lesion is in a collection of small round embryonal cells resembling lymph-cells, arranged in the connective tissue around the arterioles which come from the tunica albuginea. This may be accompanied by a more or less marked diffused interstitial orchitis, or there may be only a thickening from the new formation of small round cells on the connective tissues of the testicles. Cornil found the seminal ducts separated by numerous round or fusiform cells. The disease usually occurs at from two months to three years of age; both testicles are generally involved, and are enlarged, hard, inelastic, and frequently nodulated. Mercurial treatment generally causes a marked improvement unless the inflammation has already resulted in the development of a new fibroid formation, in which case it would be likely to remain unaffected by any form of treatment. Inunctions with diluted mercurial ointment, iodoform, etc. are useful adjuvants.

[Footnote 198: _Schmidt's Jahrbuch_, 178, No. 4.]

[Footnote 199: _Op. cit._, p. 420.]

[Footnote 200: _Rev. mens. de Méd. et de Chir._, Paris, Feb., 1878.]

[Footnote 201: _Ibid._]

[Footnote 202: _Med. Times and Gaz._, Lond., 1862, vol. i. p. 403.]

[Footnote 203: _Ibid._, Dec., 1863.]

The kidneys are not infrequently involved in inherited syphilis. Parrot reports the pathological change to consist of a proliferation of small round cells in the intertubular connective tissue, followed by contraction, obliteration of the tubules, and degeneration of their epithelium.

Bradley has reported[204] the case of a child aged four months in whom a well-marked syphilitic eruption and an attack of acute Bright's disease were coincident. Mercurial treatment for three weeks cured both.

[Footnote 204: _British Med. Journ._, Feb. 4, 1876.]

Coupland has reported two cases of parenchymatous nephritis associated with inherited syphilis, but advances no proof that it was not an accident. {309} Gummata have been from time to time found in the kidneys of very young children who have died from their effects and from other visceral lesions due to syphilis.[205] Cases of enlargement, of fibroid, fatty, and gelatinous degenerations of the suprarenal capsules, have been recorded.

[Footnote 205: See discussion in Clinical Soc. of London, Jan., 1880; "Remarks on Visceral, and especially on Renal, Syphilis," by Barthelémy, _Annales de Derm. et Syph._, April, 1881.]

The thymus gland is occasionally found in syphilis to have undergone alterations claimed by Dubois, Depaul, and others to be syphilitic in their nature, but ascribed by Parrot simply to degenerative changes due to malnutrition. The gland does not appear to undergo any marked alteration in size, color, or consistency, but is found after death to contain a small quantity of purulent matter.

The tendency of syphilis is certainly not, as a rule, to the formation of pus. Nearly all the lesions we have studied with the exception of breaking-down gummata have consisted in various forms of cell-proliferation or accumulation, and not in the formation of abscesses, and it is not probable that this is an exception. I doubt very much the syphilitic character of these changes.[206]

[Footnote 206: Lancereaux believed that it was due to the breaking down of a gummy deposit, but that seems to be entirely hypothetical, none having been discovered. Weisflag (quoted by Bumstead) arrives at the following conclusions after studying the lesion and the literature of the subject: 1. This thymus abscess does exist. 2. When associated with other signs of congenital syphilis it indicates that the father or mother of an infant suffers or has suffered from syphilis. 3. It is possible, but not proved, that this affection may exist in children in whom there are no symptoms of syphilis, but its existence renders the diagnosis of hereditary syphilis probable, even if the disease of the parent is not proved. 4. Such is the great similarity in the appearance of pus and of the secretion of the thymus that they cannot always be distinguished.]

THE DIAGNOSIS AND PROGNOSIS OF INHERITED SYPHILIS.--In reviewing the general course of a case of inherited syphilis it seems evident that the differences between it and the acquired disease which have been so much dwelt upon are apparent rather than real.[207] The primary stage is of course missing, and on any theory of the essential nature of syphilis this is readily comprehensible. Whether the chancre is the first symptom of a constitutional disease, or, as I believe to be the case, is the simple accumulation at the point of original inoculation of the cells which constitute the syphilitic virus--or are at any rate its carriers--it would naturally be in the first case undiscoverable, in the second nonexistent.

[Footnote 207: "That the noteworthy differences between chancre-syphilis and the inherited disease are to be interpreted by considerations of the tissues of the growing child and the adult, is made very probable by what is observed when a mother near the end of pregnancy becomes infected with primary disease. In such a case the foetus nearly full grown acquires the disease, without a chancre, directly from the maternal blood. It is acquisition, not inheritance, for at the date of conception both the paternal and maternal elements were free from taint, and during the first six, seven, or even eight months of intra-uterine life the foetus remained healthy. Yet, as I have proved elsewhere by citation of cases, syphilis obtained in this peculiar method resembles exactly that which comes by true inheritance, and not that which follows a chancre. This important fact goes, with many others, in support of the belief that the poison of syphilis remains identical, however obtained, and that the differences which are so patent in its manifestations are due to differences in the state of its recipient" (Mr. Hutchinson, article on "Transmission of Syphilis," _Brit. and For. Med.-Chir. Rev._, Oct., 1877, p. 475).

"It is not true that the diversity of symptoms presented by infants authorizes us to admit a congenital and an hereditary syphilis. Whatever the mode of infection, it is impossible to make this distinction" (Ricord, note to _John Hunter's Works_, 1883).]

The secondary stage, characterized in the acquired form chiefly by {310} lymphatic engorgement and symmetrical, widely-spread, polymorphic cutaneous and mucous eruptions, and pathologically by a marked tendency to the proliferation of certain new small round nucleated cells, upon the presence of which depend all the manifestations of the disease, is in inherited syphilis strictly analogous. Eruptions of the same character make their appearance, differing only in minor points, as in a greater tendency to become moist or ulcerated, due to the more delicate texture of the infantile epidermis. To the same cause must be assigned the macroscopic peculiarities of the only syphiloderm said to be peculiar to infantile syphilis--pemphigus--which has been shown, however, to have a papular basis, and in that way to conform to all the other secondary eruptions.

The lymphatic engorgement either exists in the infant as in the adult or has its analogue in the enlargement of the spleen and liver--especially the former, which is almost as constant a phenomenon as is general glandular enlargement in acquired syphilis. The same pathological changes occur, the same infiltration of cells producing, according to their situation, papular, pustular, or mucous patches, or inflammation of such structures as the iris, choroid, or retina.

The tertiary stage, except in the fact that its phenomena may appear unusually early and may be commingled with those of the secondary period,[208] does not widely differ in the hereditary from that of the acquired disease. It affects the same tissues, results in the same pathological formations, and is preceded by the same period of latency or quiescence of variable duration. There is no reliable evidence with which I am familiar to show that in this stage inherited syphilis is either contagious or transmissible--another point of close resemblance between the two varieties under consideration.

[Footnote 208: This is by no means unknown even in the acquired form; frequent examples of it have been recorded, and it can be readily explained either on the theory of relapses in parts previously diseased (Hutchinson), or on that of obliteration of lymphatic trunks and accumulation of nutritive waste (Otis).]

In considering the question of diagnosis, therefore, we have an excellent guide in the fact that the disease conforms in most respects to the general laws of acquired syphilis, and that our knowledge of the latter affection will be a valuable aid to recognition of the former.

The chief elements of diagnosis and prognosis of inherited syphilis in its various stages may then be summarized as follows:

A history of syphilis in either parent is important just in proportion to the shortness of the interval between the time of infection and the date of conception. In other words, the shorter that interval the more likely (_a_) that the child will have syphilis, (_b_) that it will have it in a severe or fatal form. If the mother has been syphilitic and the father healthy--which is rare--it is perhaps more likely that the child will be diseased than when the reverse is the case. If both parents were syphilitic at or before the time of conception, the probability that the disease will be transmitted, and in a severe form, is much increased. There is no evidence to show that inheritance from one parent results in a graver variety of the disease than when it is derived from the other.

A history of abortion or miscarriage on the part of the mother should have weight in the determination of any given case, and if such accidents {311} have been very frequent their diagnostic importance is greatly increased. The loss of elder brothers or sisters and the causes of death, with the precedent symptoms, should be carefully inquired into. The nearer either of these occurrences--abortion or death of elder children, if there is a fair presumption that they were due to syphilis--has been to the birth of the patient in question, the greater the likelihood that the latter has been infected.

Upon examining the product of abortion or stillbirth the most easily observable symptoms will be those of the skin. Maceration and elevation of the epidermis into bullæ are in themselves hardly characteristic, though they may--especially the latter--be regarded as suspicious. If the cutaneous lesions are, however, distinctly papular or pustular or ulcerative, or if the bullæ have all the characteristics of syphilitic pemphigus, the diagnosis is assured.[209]

[Footnote 209: "It is probable that very early abortions are less rare than statistics indicate, but are often unsuspected."

"It is impossible to demonstrate the existence of syphilitic lesions in foetuses expelled during the first months of pregnancy. Later, the signs which have the greatest value are the lesions of the epiphyses of the long bones. When the foetus has nearly arrived at full term, and is not macerated, visceral and cutaneous lesions may be observed. According to Mewis, the skin eruptions cannot be seen before the eighth month, and are only recognizable on foetuses whose death has been very recent or who are born living. Pulmonary lesions may be determined at the end of the sixth month. Those of the pancreas are met with in about half the foetuses which perish a little before or a little after birth. The lesions of the liver, the spleen, and the bones may be recognized even in macerated foetuses, this frequency increasing from month to month" (_Nouv. Dict. de Méd. et Chir._, vol. xxxiv. p. 864).]

The most distinctive symptom--one which may really be considered as pathognomonic, is, however, the inflammation of the diaphyso-epiphysial articulations, with or without their disjunction. Distinct enlargement of the spleen or liver, and arachnitis with hydrocephalus, are valuable diagnostic points, and the presence of gummata--not very infrequent--would of course be conclusive.

At birth the syphilitic child may be small, stunted, emaciated, weazened, senile in appearance; this would properly give rise to suspicion, but may be associated with any disorder of nutrition on the part of child or mother. It may also disclose cutaneous or mucous eruptions evidently specific in character. The most common of these at this early date is the bullous eruption affecting the palms and soles, sometimes distributed over the whole body, and, as it indicates a feeble resistance of the tissues to the tendency to exudation and cell-growth, is usually a precursor of an early and fatal termination. In any event, marked symptoms at time of birth render the prognosis highly unfavorable.

It is quite as common, however--perhaps more so--for the subject of hereditary syphilis to give no evidence of the disease at birth, but even to appear healthy and well-nourished. In such cases the first symptoms of the disease appear, on an average, in from six weeks to two or three months, and consist principally of coryza (snuffles), hoarseness of voice, and syphilodermata. The latter may be macular, papular, pustular, or bullous. They are usually polymorphous, irregular in shape, dark coppery-red in color, with sometimes a glazed or crusted, but oftener a moist or ulcerating, surface, with a strong tendency to coalesce into large patches, or to form irregular serpiginous ulcers, or to take on hypertrophic growth {312} and develop into condylomata. Eruptions which are squamous and are situated about the mouth and chin and on the body, the legs, or the soles of the feet, though exceptional, are of more value than those on the nates, where the results of irritation from urine and feces may closely simulate syphilodermata.

Mucous patches on the tongue, cheeks, tonsils, and pharynx are common, often extending to the larynx, increasing the hoarseness, and to the nasal cavities, aggravating the snuffles. Both of these occurrences, by interfering with the respiration of the child and rendering its nursing interrupted and insufficient, greatly add to the gravity of the case. Enlargement of the spleen (common), enlargement of the liver (less so), and iritis (rare), may be mentioned among the phenomena of this stage, often associated with the skin eruptions.

About the time of the subsidence of the rash there may be developed the specific inflammation at the junction of epiphyses and diaphyses which produces a swelling of the long bones near their ends. The child will be noticed to cry a little when, for example, the wrist or elbow on one side is washed, and not to use these joints as much as the corresponding ones on the other side. The parts are not hot, only slightly tender, and as yet there is but little swelling. Later, the droop and the disuse of the affected limb become more noticeable and simulate infantile paralysis. There is, however, no wasting, no alteration of reaction by faradism, no real loss of power, so that the term pseudo-paralysis is an appropriate one. In a week or two similar symptoms will occur in the bone on the opposite side, and finally the ends of all the long bones may be affected; ordinarily the elbows, wrists, knees, and shoulders are the joints involved. Suppuration is rare, disjunction of the epiphysis from the diaphysis common. Recovery is apt to take place spontaneously within a month. The associated changes are chiefly endosteal at the junction of the shaft with the epiphysis, but there is also a little periostitis or perichondritis, which is the principal cause of the external swelling. Moderate deformity may ensue.[210]

[Footnote 210: For the diagnosis from rickets see p. 290.]

Similar changes occurring in the cranial bones give rise to what has been called the natiform skull. During the first year it is very common for syphilitic children to develop a number of lenticular swellings on the cranium, which appear symmetrically around the anterior fontanel, but at a little distance from it; _i.e._ one on each frontal and one on each parietal bone. These are said to be "bossed." They are at first circumscribed, and in a child nine or ten months old often measure three-quarters of an inch to an inch in diameter. They are at first circular, afterward more irregular, and finally tend to organize, becoming diffused and massive and causing a permanent thickening of the skull.

These symptoms which have been described are the prominent ones occurring during the first six or eight or twelve months of life. If they do not manifest themselves before the eighth month, it is highly probable, even in a case with a syphilitic parental history, that the child will either escape altogether or that the secondary stage has been very slight and altogether intra-uterine and unattended with noticeable phenomena. If during this first year the child's cachexia is marked, if there are any intercurrent diseases, if the symptoms show themselves early, if the nasal or laryngeal affection is severe, if the eruptions are markedly bullar or {313} pustular or ulcerative, if the enlargement of the spleen is great or the osseous lesions precocious or grave, and if, especially, there is any intermingling of tertiary symptoms, gummata, nodes, etc.,--the prognosis will be unfavorable.

From adolescence on through adult life the diagnosis of inherited syphilis will depend on the following points: First, of course, the history of parental or of infantile syphilis, or of both. Then a group of physical and physiognomical peculiarities, which are not definitely characteristic, and are of little value when taken separately, but are of considerable importance when all or a majority are present in any given case. These are low stature or puny development proportionate to the severity of the intra-uterine and infantile symptoms; a pasty, leaden, or earthy complexion,[211] a relic of previous syphilodermata, probably also a result of malnutrition; a prominent forehead, bulging in the middle line at and within the frontal eminence, and due either to thickening of the skull or to a previous arachnitis and hydrocephalus before the ossification of the fontanels; a flat, sunken bridge to the nose, due to the coryza of infancy extending to the periosteum of the delicate nasal bones, and either interfering with their nutrition or partially destroying them; dryness and thinness of the hair, with brittleness and splitting of the nails; synechiæ and dulness of the iris (rare); ulcerations of the hard palate;[212] and periosteal thickenings or enlargements of the shafts of the long bones near the ends, or slight angular deformity, results of the osteo-chondritis of infancy.

[Footnote 211: Trousseau (_Clinical Lectures_, vol. ii. p. 588, Philada., 1873), after calling attention to this peculiar hue of the face, says: "It not unfrequently happens that the physician, taught by long familiarity with this appearance, will almost at once diagnose syphilis after having simply seen the child's face, although the peculiar hue can be but vaguely described in words. The visage presents a special shade of bistre; it looks as if it had been lightly smeared with coffee-grounds or a very dilute aqueous solution of soot. There is neither the pallor, the icteric hue, nor the straw-yellow tinge of skin seen in other cachectic affections; the tinge is not nearly so deep, but is almost like that of the countenance of a recently-delivered woman, and either does not extend at all, or only partially, to the rest of the body. I know no disease except syphilis in which a child's skin has this peculiar color; and consequently, when it is well marked, it has more diagnostic value than any other symptom."]

[Footnote 212: Mr. Oakley Coles reports (_Proc. of Path. Society of Great Britain_, vol. vii. p. 5) several cases of inherited syphilis in which there was wide separation of the jaws in the median line. In one family one member had typical teeth and wide separation; three others had the same separation, but not the characteristic teeth. It was suggested that in such cases the teeth were in size far below the average, and that the condition was that often observed where the jaws are in development in excess of the teeth which they contain. I. E. Atkinson details some interesting cases of this lesion in late hereditary syphilis, and attributes to it considerable diagnostic importance (_American Journal of the Medical Sciences_, New Series, vol. lxxvii., Jan., 1879, p. 71).]

A much more valuable group of symptoms, however, are the following, which are mentioned in the order of their importance, any one of the first three being almost or quite conclusive: