Chapter XVII: Front Matter (17)

The following case,[47] reported by a most accurate observer, seems to combine both these requisites. Zeissl the younger reports that O. X., thirty-six years old, never having had syphilis, left his wife, to whom he had been married two years, to go a journey on July 15, 1877. The wife was then in the second month of her first pregnancy. On July 24th O. X. had extra-marital intercourse. About twenty-one days after this coitus he observed a small lump on the inner surface of the foreskin, and on Aug. 22d he consulted Zeissl the elder. On Sept. 23d a maculo-papular eruption of the skin with erythema faucium appeared. Under treatment these symptoms completely disappeared. On Oct. 29th he went home to fetch his wife to Vienna for her lying-in, and had intercourse with her soon after his return, notwithstanding Zeissl's strict prohibition. At the beginning of December a hard sore developed on the left nympha of the wife, who was then in the seventh calendar month of her pregnancy. At the end of December a maculo-papular eruption spread over the body and was treated with mercury. On Feb. 14, 1878, a well-grown and apparently healthy female child was born at full term. When eleven days old[48] a pustulo-scaly eruption came out on the child's soles and toes, and soon afterward a maculo-papular eruption over the body generally. A few days later the child died. No post-mortem examination was permitted. In July, 1878, the wife had iritis, and after that gummata on the leg. She miscarried in July, 1878, at the third month, and again in February, 1879, at the second month.

[Footnote 47: Quoted by Hill and Cooper, _op. cit._, p. 60.]

[Footnote 48: Of course much too early for constitutional symptoms if the disease had been acquired during or after birth.]

There seems to be no reasonable escape, after reading this carefully, from the conclusion that in some manner the poison of syphilis found its way from the mother to the child. The old idea that the latter was directly infected in utero from the semen of the father is altogether without foundation. Other cases equally satisfactory and complete have been reported, and, unless the intelligence or the truthfulness of the observers be impugned, establish without doubt the possibility of infection during utero-gestation.

In the above case the contagion of the mother occurred in the seventh {269} month of pregnancy; and this, I believe, is as late as it has ever been known to be communicated to the child. The exact date at which it becomes impossible so to transmit it is unknown, but as a general rule it may be said that the earlier a mother is infected during gestation the less likely is it that the child will escape. Treatment of the mother--as of the father in cases where he is at fault--very greatly modifies the whole problem and adds immensely to the chances that the child will not be infected.

Direct infection of the child during birth could not properly come under the head of hereditary syphilis. There is no possible reason why, when the mother has contagious lesions of the genitals, acquired too late to infect the child in utero, this should not occur, but as a matter of fact no such case has ever been recorded. One explanation of this circumstance may be found in the protective covering of vernix and mucus which coats the infant's body and lessens greatly the risk of absorption. This hardly accounts satisfactorily, however, for the entire absence of such cases from medical literature, and it is fair to suppose that in all but those cases in which the primary sore is acquired during the last month of gestation--which for obvious reasons are excessively rare--the infant acquires some immunity which protects it from its mother, and is similar to that which, under Colles's law, operates in her favor. In other words, even though apparently free from syphilis at birth--a not uncommon event, as we shall see--it has a latent or modified syphilis which protects it from contagion.

We may now briefly restate the conclusions at which we have thus far arrived:

1. After a certain interval, not less than four years, and after thorough specific treatment, a person who has contracted a syphilis not especially severe or malignant in its type may be permitted to marry. The assent to marriage will then be based on a belief in the curability of syphilis or the cessation of its contagiousness, its inoculability, and, in the vast majority of cases, its transmissive power at the end of the secondary stage.

2. It may be inherited from either parent or from both, and the probability that this will occur increases in a direct ratio with the nearness of the time of conception to the date of their infection with the disease. The severity of the inherited disease in the child increases in the same proportion.

3. It is undoubted that, the father being healthy and the mother syphilitic, the child may, and in all probability will, have the disease.[49]

[Footnote 49: Dr. Sturgis, who disbelieves altogether in the possibility of paternal heredity, concludes, after examining the subject carefully, that (1) a mother begets non-syphilitic children as long as she is not infected, even though the father is syphilitic; and (2) the moment she is diseased the children are inevitably so (Paper on "The Etiology of Hereditary Syphilis," _New York Medical Journal_, July, 1871). This doctrine was previously supported by M. Cullerier, whose views gave rise to the remarks of M. Voillemier (quoted by Fournier) that if they were accepted "the father would be only the accidental occasion of a child; one would be, in reality, the child of his mother only." Cullerier's cases are invalidated by the fact that the syphilitic fathers who had healthy children had been subjected to mercurial treatment (_Mém. de la Société de Chirurgie_, Paris, 1854, quoted by Taylor in _Archives of Clin. Surg._, vol. i. p. 83). The theory is a very old one. Vassal has sustained this idea as long ago as the end of the last century. Kostum (1804), and after him Hufeland, were of the same opinion. Cullerier (1857) wrote: "In order that a child acquire syphilis hereditarily it is necessary that the mother is or has been {270} syphilitic." Notta, Follin, Charrier, Mireur (1867), and Langlebert (1873) support this theory more or less earnestly. Oewre wrote (1873): "Paternal influence is nil as regards hereditary syphilis." Isseff (1879) wrote: "Where a man suffers or has suffered from syphilis he cannot transmit the disease to his descendants without infecting his wife; that is to say, in fewer words, there is no infection from the father." Sigmund says: "The heredity of syphilis is derived in its last analysis from the mother" (_Nouveau Dict. de Méd. et Chir._, vol. xxxiv. p. 689).]

4. It is probable, but less so,[50] that, the mother being healthy and the father syphilitic, the child will be infected.

[Footnote 50: This refers simply to the comparative probability of infection, and does not conflict with the statistical fact expressed by Hutchinson (Reynolds's _System of Medicine_, vol. i. p. 431) in his words: "In the large proportion of cases met with in practice the taint is derived from the father only." This numerical predominance of paternal influence is very readily explained. There are many more syphilitic men than syphilitic women, and especially among the couples who contract fertile marriages the number of women who are infected before becoming mothers is inconsiderable. On the other hand, it frequently happens that men who have had syphilis, but have been without symptoms for a longer or shorter interval, marry and transmit to a series of children a disease which has ceased to be directly contagious to their wives, the transmissive power continuing after the possibility of ordinary contagion has disappeared. As in the majority of such women the disease is latent, and may be only displayed in their immunity from infection, it becomes evident that, history and symptoms both being wanting on their part, the conditions justify the assertion of Mr. Hutchinson. (See _Nouveau Dict. de Médecine et Chirurgie_, p. 684.)

That assertion (quoted above) has, however, been thought by several writers to indicate his belief in the escape of the mother. That I have not misinterpreted him is evident from the following extract from an article on "The Transmission of Syphilis," written by him (_Brit. and For. Med.-Chir. Rev._, Oct., 1877): "I take it for granted (although I know that there are still some who doubt) that it is possible for a father to transmit the taint, the mother being at the time of conception wholly free. I believe, indeed, that in practice this is by far the most common way in which syphilis is transmitted. Whether in these cases it is correct to speak of the inheritance being paternal only is, as we have just seen, another matter, since it is possible that in every instance the mother derives an infection from the father, and may thus in turn influence it."]

5. It is highly probable, though it can hardly be considered as proven, that in all cases where a child becomes syphilitic through paternal influence the mother is also the subject of syphilis, which may, however, assume a latent form, the only evidence of its presence in a few cases being the protection which it affords against contagion through the medium of the child.

6. Syphilis may be transmitted from mother to child even when it is acquired by the former as late as the seventh month of utero-gestation.

Since writing the above the thirty-fourth volume of the _Nouveau Dictionnaire de Médecine et de Chirurgie_ has been published. In the article on syphilis seventeen pages are devoted to the question of heredity, which is reviewed in a most thorough manner and finally summed up as follows (p. 698):

"The most definite views which we possess on the subject of the hereditary transmission of syphilis may be thus expressed:

"Children may be infected by heredity, not only when the two parents are syphilitic, but also when only one, either the father or mother, is diseased at the time of conception.

"When both parents are diseased at that time there is more certainty that the child will be infected, and infected gravely, than if only one of them has the pox.

"The hereditary disease is not always fatal, even when both progenitors have actual specific symptoms. The more recent the disease of the parents the greater the chances of their transmitting the disease and of its assuming {271} a serious form. There is no proof that inherited syphilis is more grave when derived from the father than when coming from the mother.

"It is altogether exceptional for the mother, healthy at the moment of conception, not to participate in the disease transmitted by the father to the child. If she escapes direct contagion--which is rare when the disease of the father is active--she undergoes a species of infection from contact with the contaminated foetus.

"Syphilis by conception, which is thus transmitted from the foetus to the mother, may present the usual characters and evolutions of the acquired disease; frequently, also, it is latent, and is betrayed only by the existence of immunity from further contagion on the part of the mother. It may finally manifest itself by tertiary symptoms or by systemic troubles without specific characters.[51]

"The power of transmitting syphilis hereditarily decreases spontaneously as the disease of the parent becomes older. The influence of treatment is no less certain than that of time. When the two progenitors are at the moment of conception free from syphilis, the foetus may still be infected if the mother acquire the disease during her pregnancy."

[Footnote 51: "This form of syphilis shows itself in the mother in three ways:

"1st. By the usual signs of syphilis by contagion, with the exception of the primary sore, appearing about the sixty-fifth day after conception.

"2d. Appearing at a later date as secondary or even as tertiary symptoms, and preceded merely by a little disturbance of the general health, unhealthy appearance of skin, falling of hair, etc., but nothing truly specific.

"3d. Showing itself some years afterward in a tertiary form, having in the mean while given no indication of its existence save only in the protection it afforded against contagion from the child" (_Nouveau Dict. de Méd. et Chir._, vol. xxxiv. p. 696).]

* * * * *

Syphilis of the placenta is of especial interest in its relation to the abortions and stillbirths so frequent in syphilis. Until the elaborate paper of Fraenkel upon this subject (see foot-note, p. 265) almost nothing was known about it. He describes[52] the macroscopic changes as consisting of increased size and weight of the placenta, closer and firmer texture of the placental tissue, the presence of old and recent extravasations of blood in all stages, opacity and thickening of the decidual covering and of the amnion and chorion, which are in places adherent to each other. Microscopically, it was found that the placental villi were filled with small nucleated cells, which were especially abundant in the centre of the villous spaces along the axis where the vessels usually take their course. The ends of the villi were enlarged with knob-like processes.

[Footnote 52: I condense here from the translation of Fraenkel's paper, which constitutes the bulk of chap. xxvii. of Bumstead and Taylor's excellent work.]

Fraenkel's explanation of these changes is as follows: Under the influence of syphilis[53] cell-proliferation begins in the villi, which are, normally, only sparingly supplied with cells. These new cells excite proliferation of the cells of the connective-tissue stroma and of the epithelium. This proceeds to such an extent that it leads to compression of the vessels,[54] interfering with the circulation, and finally obliterates them. The {272} vascular spaces into which the villi dip become filled up and narrowed, and often disappear. In this way, and also by reason of the thickening of the epithelium, the interchange between the maternal and foetal blood is interfered with, and at last is prevented.

[Footnote 53: Fraenkel took as his criterion of syphilis the presence of the osteo-chondritis described by Wegner (see p. 286).]

[Footnote 54: "Hennig was the first who called attention to the intimate relation of the cell-growths to the vessels. The obliteration or compression of numerous vessels of the villi interferes with the mutual interchange of gases between the maternal and foetal blood, causing fatty degeneration of the villi and, if the process is extensive, the death of the foetus" (article "Syphilis," _Ziemssen's Cyclopædia_, vol. iii. p. 237).]

If this process is spread over the whole placenta, the foetus perishes before it is complete. If it is limited to circumscribed areas, it may continue to live for a shorter or longer period.

These observations require to be repeated and confirmed, but they have served to open up a most interesting branch of syphilitic pathology.

Syphilis in the parents will manifest itself in the children in one of several ways, which are determined chiefly by two factors--viz. first, the length of the interval between the infection of the parent and the date of conception; and, second, the thoroughness of the treatment of the parents during that interval. To these may be added as subsidiary, but still of definite importance, a third, the type of disease which has affected the father or mother, whether mild or severe, benign or malignant.

From what has already been said in reference to the question of marriage, it will be at once understood that the danger to the offspring in untreated cases, and in those where conception has occurred during the early secondary period of the disease, is of the most extreme gravity.[55] In such cases the usual result of pregnancy is abortion at from the first to the fifth or sixth month,[56] the foetus sometimes exhibiting the evidences of the disease in the shape of large bullæ upon the palms and soles, or in the presence of characteristic visceral lesions, but quite as often showing nothing distinctive. It has generally undergone more or less maceration, and the skin, which is readily detachable, is of a congested, purplish color.

[Footnote 55: According to Kassowitz, in women who are not treated all pregnancies occurring within the first three years of their infection terminate either in abortion or in the birth of children who survive for only a few weeks or months.

Weber (quoted by Parrot, _Le Progrès Médicale_, Nov. 24, 1877, p. 882) treated thirty-five pregnant women by mercurial inunction, and they all went to full term under normal conditions. Among those treated with mercury and iodide of potassium, but who, by reason of intolerance of the former drug, took chiefly the latter, 20 per cent. aborted; when the mixed treatment was carried out regularly 15 per cent. aborted; and when only iodide of potassium was given 36 per cent. aborted. His studies were made on 109 syphilitic pregnant women. Parrot himself says (_ibid._) that "abortion occurs in about one-third of all syphilitic women. Syphilis should always be suspected when this accident occurs several times successively." "The date of abortion depends on the age of the syphilis of the woman. It is most to be feared during the evolution of secondary symptoms, particularly when they are grave. There is more probability that it will occur when infection has preceded pregnancy than when it occurs during its course. Nothing is so much to be feared as contamination of the ovule. The nearer the date of infection of the mother approaches to full term, the fewer the chances of abortion. It is not likely to occur even when the mother is infected at five months."]

[Footnote 56: Many observers think that abortion results directly from the death of the foetus. Babington (notes to Hunter's _Treatise on Venereal_), Trousseau, and Von Baerensprung were of this opinion.]

Dr. Cory thinks that in many cases it is possible that the effect of syphilis may be to effect so early an abortion that the case is simply regarded as one of delayed menstruation or of menorrhagia. Such a conception would, however, be competent to infect the mother, and might seem to explain cases otherwise involved in obscurity.[57] At least one-third {273} of all syphilitic children are dead born.[58] As time goes on, however, and the intensity of the parental disease is lessened, or in cases where appropriate treatment has been applied, either the abortion occurs at a later period of pregnancy or the children are brought alive into the world. Even then, however, and although at birth they may show no evidences of the disease, their chance of escape is but small. One-fourth of them die within the first six months. If they survive that period, the chances for life are slightly in their favor, but those for health or freedom from deformity and disease are still overwhelmingly against them.

[Footnote 57: As, for instance, when a woman married to a syphilitic man, but without issue, remarries a man with no history of syphilis, and yet gives birth to a syphilitic child. In such a case there would be no history of direct infection and none of pregnancy, the only two modes by which she could have contracted the disease, and the father--the second husband--might be unjustly suspected.]

[Footnote 58: Kassowitz, _op. cit._]

The course of inherited syphilis differs strikingly from that of the acquired disease. It will hardly be necessary to do more than remind the reader of the ordinary stages of the latter affection--the primary, which includes the period of the chancre and of lymphatic engorgement, lasting about sixty to seventy days; the secondary, or exanthematic, characterized by copious cutaneous eruptions and extensive involvement of mucous surfaces, lasting from one to three years; the intermediate,[59] or the stage of latency and relapses, lasting for a very variable period, from three to ten years, but under proper treatment very much reduced or altogether abolished; and finally, the tertiary period, beginning four or five years after contagion, extending indefinitely throughout life, but often in cases properly treated absent altogether.

[Footnote 59: So designated by Mr. Hutchinson, and for clinical purposes a very valuable addition to the periods of syphilis. He describes it as follows: The patient may be either wholly free from symptoms and in good health, or he may remain pale and rather feeble, and liable from time to time to slight returns of eruption on the skin, sores on the mucous membranes, condylomata, etc. He is protected as regards fresh contagion, and should he beget children they are almost certain to suffer. The relapses during this stage are usually easy to be distinguished from true secondary symptoms. There is little or no febrile disturbance, the rash is not copious, and often not symmetrical. Acute iritis, retinitis, etc. never occur for the first time, though they may do so in the form of relapses.]

For purposes of description and of contrast we may similarly divide the whole period of evolution of a case of inherited syphilis,[60] omitting the primary stage, which has never been found to exist in true cases of hereditary syphilis. Of course in congenital or infantile syphilis, in which by direct contagion, either from the mother or from any one else, the disease was acquired by the child, the course would not differ materially from that observed in the adult. But as this stage in all probability corresponds to the period during which the poison is already finding its way into the system through the lymphatics, of course it is not found in the child who is infected from the moment of conception or who receives the poison from the mother directly into the circulation.[61] For from one to three weeks the infants often show no symptoms of the disease. In 158 cases collected by Diday, 86 manifested symptoms of the disease before the expiration of the first month, and 60 of the remainder before the end of the third month.[62] When to these are added the {274} statistics of Roger, we find that of a total of 172 cases, 159 showed syphilitic symptoms before the end of the third month.[63] When the symptoms are present at birth, they consist largely in a general withered, atrophied, weazened appearance of the child; a hoarse cry, due to swelling, with subacute inflammation, or even ulceration, of the laryngeal mucous membrane; a coryza,[64] due to a similar condition of the Schneiderian membrane; and certain cutaneous eruptions, the most common of which at this early date is the large vesicular or bullous eruption known as syphilitic pemphigus.

[Footnote 60: The idea that the character of the symptoms which first appeared depended upon and corresponded with the stage of the disease in the parent has now, I believe, no supporters. It was once thought, at least by some syphilographers, that if the parents were in the tertiary stage at the time of conception the child would develop tertiary symptoms, omitting the other stages.]

[Footnote 61: If chancre were the first symptom of constitutional syphilis, why should it not appear in cases of hereditary syphilis?]

[Footnote 62: _Infantile Syphilis_, p. 101]

[Footnote 63: About 1823, from 16,000 to 17,000 children were admitted annually to the wards devoted to foundlings at Lyons. Clièt, recording his experience with this disease, says that syphilis is one of the most common of their maladies, but that "it exceedingly seldom shows itself at birth by evident signs" (_Compte-Rendu Méd. Chir. des Observations à l'Hôpital général de la Charité de Lyon_, 1823). Cristoferi, physician to the foundling hospital at Bologna, says that syphilis generally manifests itself between the ages of one and three months. Never once, he states, was a newly-born infant admitted with the disease unquestionably developed (_Gazette Medica di Milano_, 1844). Trousseau says that it "rarely appears before the second week, and very exceptionally after the eighth month" (_Lectures on Clin. Med._, vol. iv. p. 331, London, 1871, ed. of New Sydenham Society).]

[Footnote 64: Usually appears later, but exceptionally at birth.]

Pemphigus.--With regard to the specific or non-specific character of this eruption there has been much difference of opinion, and, as it is often the earliest distinctive expression of syphilis, a diagnosis of which could hardly be founded on the general appearance of the child, or even on the hoarse cry and the coryza, it becomes important to have definite ideas upon the subject. Nearly a century ago (in 1794) it was denied[65] that this eruption was a manifestation of venereal disease; and this view has been supported by many able and accurate observers down to the present day. In 1851 a discussion upon the subject took place in the French Academy of Medicine, which elicited the opinions of the majority of those members who were entitled to speak with authority in the matter, and which has since been referred to by most writers. Cazeaux upheld the non-syphilitic hypothesis on the ground (1st) that the so-called syphilitic pemphigus of children does not differ from the simple pemphigus of adults, presenting none of the physical characters which distinguish the specific cutaneous eruptions; (2d) that it appears at birth or immediately after, while the symptoms of hereditary syphilis generally show themselves later;[66] and (3d) that at that time there had been seen no cases of pemphigus at the Lourcine Hospital, where so many syphilitic children were born.[67] In this view he was supported by Trousseau, Lasègue, Gibert, Bazin, and other obstetricians and syphilographers of note.[68]

[Footnote 65: Ariander, _Mémoires de Méd. et d'Accouch._, quoted by Diday.]

[Footnote 66: This is now known to be an unreliable distinction, expressing perhaps a general rule, but one with so many exceptions as to render it void of diagnostic significance.]

[Footnote 67: This may have been true at that time, but has certainly not continued to be so. Cornil says: "We often see at the Lourcine children born prematurely or at the full term with pemphigus, either fully developed at the moment of birth or appearing a few days afterward, and who commonly die with syphilitic cachexia, the sad heritage derived from their maternal parents."]

[Footnote 68: Pemphigus may indeed be a specific affection, but no characteristic sign has been discovered sufficient to distinguish it from the ordinary form of pemphigus. "On the other hand, there is no inconsistency in admitting that syphilis, which so deeply impairs the constitution of the parent, may act like any other common cause and excite non-specific pemphigus; for an infant is badly lodged and poorly nourished in the womb of an enfeebled mother, apart from the influence of the virus" (Ricord, note to _John Hunter's Works_, 1853).]

On the other hand, Dubois claimed a specific character for the affection {275} on the ground (1st) of the fact that in the majority of cases there was a syphilitic history in the parents; and (2d) that the eruption often coexists with well-known syphilitic lesions. This was supported by Cazenave, Danyan, Bouchut, Vidal, Ollivier, and others.[69] Diday, who devotes several pages of his interesting work on _Infantile Syphilis_ to this subject, regards the eruption as simply a manifestation of a cachexia produced by syphilis,[70] founding this opinion on (1st) the absence of specific characters in the eruption; and (2d) that syphilitic pemphigus is a rare affection in the adult, if it occurs at all, so that to recognize it in the child would be to make a single exception to the general rule that "all the syphilitic eruptions of new-born children have their equivalents in those of adults." He explained the two cases which were then (1858) recorded of cures of pemphigus by mercury[71] by saying that it was the treatment of the diathesis, not of the disease, which caused the improvement. He acknowledges, however, the very frequent association of pemphigus in the child with syphilis in the parent, and says that it springs from the latter affection, "specially, but not specifically"--a rather wire-drawn distinction.[72]

[Footnote 69: Jullien (_op. cit._, p. 1005), after considering the opposing views as to the character of this eruption, says: "We have no hesitation in declaring ourselves in accord with Roger, Ollivier, Ranvier, Parrot, and others, and in distinctly separating from the specific affection the rare eruption known as simple pemphigus, sometimes epidemic, occasionally febrile, and appearing most frequently about three months after birth. We consider likewise that an evidence of congenital syphilis which is by no means doubtful is found in the bullous eruption seen at birth or within the first two weeks, comparatively frequent, and involving by preference the palms and soles. This opinion is based upon (1st) its appearance in children whose parents are known to be syphilitic; (2d) its association with syphilitic lesions of the lungs, liver, kidneys, thymus gland, etc.; (3d) its partial disappearance under mercurial treatment, and its reappearance when that treatment is discontinued."]

[Footnote 70: _Op. cit._, pp. 70-77.]

[Footnote 71: Depaul, _Gaz. Méd. de Paris_, 1851, p. 472, and Galligo, _Gaz. Med. Toscana_, 1852, p. 123.]

[Footnote 72: Trousseau (_Clinical Lecture on Syphilis in Infants_), after detailing a case in which there was some doubt as to the existence of hereditary syphilis in a child born alive, and in which case the previous pregnancy had resulted in a stillborn child at seven months, the body of the latter having been preserved in alcohol and exhibiting numerous traces of pemphigus, says: "So far as I was concerned, this demonstration did not amount to more than the establishing of a probability, and several physicians who participated in this indecision finally accepted a compromise. They considered that maternal syphilis had determined a sort of cachexia in the foetus which had led to an eruption of bullæ which was not specific. By accepting this too-facile hypothesis you will imprudently open a door which you will with difficulty be able to close."]

As these differences of opinion have been perpetuated to the present day, it has seemed to me proper to make this reference to their history, although I am strongly convinced that the progress of clinical and pathological knowledge enables us now to assert that although, as an exception, bullæ may be due to a profound cachexia not dependent on syphilis, yet that in the large majority of cases they are specific in their character.

The argument which always seemed to me the strongest, the fact that a similar eruption is almost--or quite--unknown in the adult, has been removed by the observations of Cornil, who has shown that it belongs properly with the papular rather than with the bullous eruptions, and should be classed with the roseola and papules of early syphilis--just where, from its clinical history, we should expect to find it. The raising of the epidermic layers is due chiefly to their delicacy, their slight resistance, and their previous immersion in the amniotic fluid--_i.e._ to {276} conditions which are peculiar to the skin shortly after birth.[73] He founds these very important opinions upon the autopsy of a child stillborn a little before full term, the mother being in the height of secondary syphilis. The child presented characteristic bullæ on the soles and palms. After hardening these were found to consist of the two layers of epidermis placed one above the other. Fig. 6 represents a bulla about one centimeter in diameter which was situated on the plantar surface of the great toe.

[Footnote 73: Cornil, _op. cit._, p. 203.]

If, then, we find an infant at birth or immediately after[74] presenting on the soles, the palms, the fingers and toes, or on the limbs, an eruption consisting of blebs more or less perfectly distended with a liquid which may be clear, cloudy, or bloody, circular or oval in shape, sometimes irregular, seated on inflamed, reddish skin, and surrounded by trifling areolæ, we may strongly suspect the presence of syphilis in an active and most menacing form. And this suspicion becomes a certainty if, in combination with such an eruption, the general cutaneous surface is yellowish or muddy in hue, is hard, dry, wrinkled, without elasticity or softness--owing to the absence of subcutaneous fat--and, for the same reason, is furrowed and wrinkled about the face, imparting an appearance of senility; if the child has a hoarse cry, a discharge from the {277} nostrils; and, of course, if there are at the same time other and unmistakable syphilodermata. This eruption is specially important, however, because upon the recognition of its specific character in cases of stillbirth, or in those in which the child survives only a few days--not long enough for the development of further symptoms--will depend the opinion as to the cause of death, which, whether expressed or not, will determine the future treatment of both parents during the interval and of the mother during the next pregnancy.

[Footnote 74: Non-syphilitic pemphigus is said to be never present at birth, nor until the child has become considerably exhausted by wasting from some defect of nutrition. It therefore does not appear until it is several weeks old. It then attacks the trunk in preference to the palms and soles.]

We may now consider the other symptoms of the secondary period in the child.

Coryza is one of the most characteristic, and at the same time one of the most important, of these in its influence on the health of the child. It is due to the same condition of the mucous membrane lining the nasal fossæ as manifests itself simultaneously or soon afterward on the skin in the shape of erythema, roseola, or papules; in other words, it is a hyperæmia with papillary infiltration. Now, on the skin this condition, except in so far as it indicates the presence of a grave constitutional disease, is of no special importance. In the nostrils of a sucking infant, already debilitated and impoverished by the anæmia of syphilis, and depending upon its nutrition for the continuance of the miserable flickering life which was its original endowment, the same condition assumes the gravest significance.

The excessive supply of blood to the parts induces a catarrhal condition which shows itself in a thin, watery discharge, which, as the child during sucking is compelled to breathe through the nose, is rapidly dried into crusts. These become adherent, fill up and lessen the channel for the passage of air, and in so doing add to the rapidity and force of the respiration through the nose, and thus increase the tendency to the deposit of these crusts. The peculiar nasal, noisy respiration of the child has given the affection the popular name of snuffles. As the child can no longer breathe, or can breathe only with great difficulty, while sucking, it takes the breast only to drop it again immediately on account of impending suffocation.[75] As the disease progresses ulceration occurs beneath the crusts, and often involves the entire thickness of the delicate mucous and periosteal layers underlying the thin bones of the nose; perforation of these bones results, sometimes with caries to such an extent as to cause an entire loss of the nasal septum, with flattening of the nose--a symptom comparable to one which sometimes occurs in the tertiary period of adults, but produced, as we have seen, by other causes. In adults syphilitic caries and necrosis are usually due to lesions seated primarily in the osseous or subperiosteal tissues; in the child, at least in this instance, these tissues are involved secondarily.

[Footnote 75: For an admirable description of the mechanism of this and other symptoms of coryza see Diday, _op. cit._, pp. 78-83.]

Erythema, or roseola as it is differently called, is apt to present itself about the second or third week[76] after birth. As in the adult, it begins upon the abdomen in the form of little oval, circular, or irregular spots, dull red in color and disappearing upon pressure. Later the color becomes deeper, the eruption extends to the trunk and limbs, and, as exudation and cell-proliferation succeed to simple capillary stains, it {278} ceases to disappear when pressed upon. It is often moist, owing to the thinness of the epidermis, sometimes excoriated. Occasionally it is confluent, and covers large areas with an almost unbroken sheet of deep-red color.

[Footnote 76: Bassereau gives an instance of its occurrence within three days.]

The diagnosis in the early stage is often difficult on account of the resemblance to the simple erythema of infancy. As the disease progresses, however, maculæ form here and there; the cell-infiltration involves the papillæ, several of which coalesce, forming flat papules; the nutrition of the superficial layers of the epiderm is interfered with, especially where it is thick, as on the palms and soles, and the eruption in those regions becomes scaly, and then the diagnosis is not difficult.

Papules and Mucous Patches.--In the ordinary evolution of the disease the next manifestation is usually the development of papules upon the general cutaneous surface and of mucous patches on the tongue, lips, and cheeks--probably also on other mucous membranes not exposed to examination. The papules are apt, for the reason already mentioned--the thinness and moisture of the skin--to be of the broad, flat kind, especially, as in the adult, in those regions where the elements of warmth and friction are superadded to the moisture, as in the folds of the skin about the genitalia, the neck, the flexures of the joints, etc. They are then moist, covered with a grayish secretion or a thin crust, and are in reality mucous patches. Occasionally they take on a little hypertrophy and develop condylomatous excrescences which closely resemble the simple acute condylomata of infants. In syphilis, however, the growth springs from a previously existing papule, which is not apt to be solitary, there being others in the neighborhood which will probably establish the diagnosis. The syphilitic condylomata also have a peculiar fetid discharge, resembling that of mucous patches and more or less characteristic.[77]

[Footnote 77: Van Harlingen, article "Syphilis" in the _International Encyclopædia of Surgery_, vol. ii. p. 560.]

Mucous patches in the infant are among the most important of the early syphilitic lesions--not to the child itself, because they do not materially affect its health, save in those exceptional instances where they are accompanied by a marked degree of stomatitis, and thus interfere with its nursing. Their importance is due to the fact that they are almost constantly present, and they are thus by far the most frequent vehicle of contagion from the child to its nurse or to others with whom it may come in contact. At times they do not differ materially from the same lesion occurring in the adult, but lose much sooner their epithelial investment (on account of the delicacy and comparatively slight attachment of the epithelium at this stage), and they then appear as oval or irregular red, slightly depressed spots, distinct or coalescing, ulcerating or oftener covered by a false membrane. They especially affect the angles of the mouth and the sides and dorsum of the tongue; and indeed their disposition to select the former situation constitutes a diagnostic difference between them and non-specific stomatitis which is to be found in the sulci between the gums and cheeks and on the gums themselves--locations rarely invaded by mucous patches.[78] When the latter are ulcerating or are concealed by diphtheritic membrane, and are situated on the tongue, they may be mistaken for either simple or parasitic stomatitis. The {279} diagnosis can often be made by the presence of other syphilitic symptoms--coryza, erythema, and especially papules. In their absence, however, it must be remembered that in simple stomatitis, the inflammation not being limited to special areas, the whole tongue is apt to be involved or a much larger portion of the buccal mucous membrane; and as there is no marked tendency to cell-proliferation in these cases, the accompanying exudation is apt to be serous or watery and to result in vesiculation--a condition never seen in syphilitic stomatitis. In the parasitic disease, too, the inflammation is less localized, there is more swelling and congestion, and the false membrane is said to be of a whiter color.

[Footnote 78: Bumstead and Taylor, _op. cit._, p. 750.]

No child that has even been suspected of having a taint of hereditary syphilis should be permitted to nurse at the breast of any one but the mother, to share its cup or nursing-bottle with other children, to receive the caresses of relatives or friends; and in this last restriction we would include the father, even if the suggestion[79] be true, that in the case of syphilitic children the protection from contagion probably extends to the male as well as the female parent. Paternity is sometimes a more doubtful problem than would seem probable, and even if the father were protected the husband might not be. The mucous patches, if any are found to exist, should be actively treated both locally and constitutionally, and during their demonstrable presence a most rigorous quarantine should be observed.

[Footnote 79: Hyde, _op. cit._ See p. 264.]

Syphilitic condylomata are due to hypertrophic changes in the papules, which under the influence of heat and moisture in certain regions coalesce and become more elevated. They vary in size from an eighth of an inch to a quarter or even a half of an inch in diameter. Their surface is flat and covered by a crust or by an offensive secretion. They are found most commonly about the anus or at the angles of the mouth.

Pustular Syphilides.--A little later in the secondary period, usually at about the sixth week, but sometimes much earlier, the papules become transformed into pustules, the change taking place slowly, so that if examined at any time after it has begun the child will present an eruption which is markedly polymorphic, showing here and there yellowish or reddish-yellow maculæ left after the absorption of the cell-element of certain papules, at other places beefy-red papules at the height of their development, or papules crowned by a ring of desiccated and desquamating epidermic scales, and in still other regions pustules in various stages of formation. Or the various formative stages of the pustules may be passed through so quickly that the eruption will be almost entirely pustular, few if any unmodified papules being discovered. The pustules may remain distended with pus for a considerable time, after which they may wither and slowly disappear or may rupture and leave ulcerated surfaces. A number of these ulcers sometimes run together and make extensive patches covered with thick, dark-colored crusts. These patches may resemble areas of impetigo or of impetiginous eczema, but in those affections the crusts are usually thinner and of a lighter color, and the skin beneath them is generally on a level with the surrounding surface, bright red and glazed; while under the crusts of the syphilide will be found a more or less depressed or excavated ulcer, often covered with pus. The diagnosis may indeed often be made by gently detaching and {280} raising one of the crusts and noting the character of the surface beneath. The erosion under the crusts of eczema heals over more readily and without leaving a cicatrix.

A so-called furuncular eruption[80] is said to appear at variable periods between the sixth month and the third year, but does not appear to me to be clearly differentiated from the large pustular syphilides with thickened and elevated bases on the one hand, or the ulcerating tubercular eruption on the other.[81] They are all so rare in hereditary syphilis, at any rate, as to have little clinical importance.

[Footnote 80: Bumstead and Taylor, _op. cit._, p. 750.]

[Footnote 81: The distinction between the two forms is usually manifest if the development of the lesions has been observed; but even this fails in regard to the tubercular eruption. They both occur at the same period; they both begin similarly, the furuncles as "small nodules in the corium," the tubercles as "deeply-seated papules or nodules;" they both run on to ulceration and pursue a chronic course (Van Harlingen, _op. cit._, p. 561).]

Iritis.--Another symptom of the secondary period, but of later development and of rarer occurrence than the syphilodermata which have been described, is iritis. In spite of its rarity this is extremely important, because it is frequently overlooked until it has reached such a stage that occlusion of the pupil results, and also because when it is recognized it constitutes an almost pathognomonic sign of syphilis.[82] This statement may now be made unhesitatingly, although for many years it was contended that iritis, and even the still more characteristic symptom keratitis, were only associated with syphilis as coincidences, the constitutional disease, when hereditary, having no causative relation to the local condition.

[Footnote 82: "When primary iritis occurs in syphilis in young children it is almost always due to syphilis" (Soelberg Wells, _Treatise on Diseases of the Eye_, Philada., 1873, p. 173).]

To Mr. Hutchinson belongs the credit of having first clearly developed the specific character of this trouble,[83] which, on account of the mildness of the attendant symptoms, is often overlooked. The sclerotic zone of congestion so marked in the adult, and therefore so valuable a diagnostic sign to the general practitioner, is very slight, sometimes absent; and as a consequence the attention of neither parent nor physician is attracted to the condition until in the more serious cases it has done irreparable mischief. In milder cases, particularly where the child is under mercurial treatment for concomitant symptoms of syphilis, it may run its course and escape notice altogether;[84] and it is possible that owing to this fact the rarity of the affection has been overestimated. It is also possible that in such cases changes occurring at this time may in some instances lay the foundation for some of the deeper-seated ocular troubles of later life.

[Footnote 83: _Med. Times and Gazette_, 1860, July 14; _Ophthalmic Hospital Reports_, vol. i. pp. 191, 226; _A Clinical Memoir on Certain Diseases of the Eye and Ear consequent on Inherited Syphilis_, London, 1863. In the introduction to this volume Mr. Hutchinson states that acute iritis dependent on hereditary syphilis was first described in connection with its true cause by Mr. Lawrence, but, as from the date of that gentleman's first case (1830) up to 1863 but six cases had been recorded, the announcement had made but little impression on the profession.]

[Footnote 84: "In the cases of this form of iritis which are seen in ordinary eye-practice much damage has often been done by occlusion of the pupil and deeper mischief. Probably many of the slighter cases escape the notice of the parents and are not brought to the surgeon" (Mr. Edward Nettleship. See Hill and Cooper, p. 271).]

If, however, attention has been attracted to the eyes, the diagnosis is not usually difficult. The pupil is irregular, especially under atropia; {281} there are streaks of lymph, dulness, swelling, change of color, and on very careful inspection a faint pink zone may be seen in the sclerotic. The conjunctiva and cornea are generally clear.

Mr. Hutchinson's analysis of the twenty-three cases reported by him is still of interest as furnishing reliable data for prognosis. The average age at the time the iritis commenced was five months and a half. The oldest was sixteen months at the time of the outbreak, the youngest six weeks. In twelve cases but one eye suffered; in eleven both were affected. In seven cases (ten eyes) the cure was complete; in two or three other cases very slender adhesions remained; in twelve cases, in nearly all of which the patients came under care only at a late period of the disease, one pupil was permanently occluded by organized false membrane. In nearly all, coincident symptoms of syphilis of the skin or mucous membranes were present. Of the thirteen cases in which alone a history of the family is recorded, the affected infant was the only living child of his parents in twelve instances. In the only case in the whole series in which it is stated that there were other living children the mother had lost four infants out of seven live births.

The prognosis depends on the stage at which they come under treatment. The lymph if recent, no matter in what quantity, will probably be absorbed under mercurial treatment, which will often be of great benefit even in those cases in which a certain amount of organization has occurred.[85]

[Footnote 85: It will not be uninteresting, perhaps, to append the aphorisms regarding iritis in infants which Mr. Hutchinson at that time enunciated: 1. The subjects of infantile iritis are much more frequently of the female than the male sex. 2. The age of five months is the period of life at or about which syphilitic infants are most liable to suffer from iritis. 3. Syphilitic iritis in infants is often symmetrical, but quite as frequently not so. (In his article in Reynolds's _System of Medicine_, written in 1866, three years later, but revised in 1870, he describes it as "usually symmetrical," vol. i. p. 444.) 4. Iritis, as it occurs in infants, is seldom complicated, and is attended by but few of the more severe symptoms which characterize the disease in the adult. 5. Notwithstanding the absence of phenomena of acute inflammation, the effusion of lymph and the danger of occlusion of the pupil are usually very great. 6. Mercurial treatment is most signally efficacious in curing the disease, and, if recent, in procuring the complete absorption of the effused lymph. 7. Mercurial treatment previously adopted does not prevent the occurrence of this form of iritis. 8. The subjects of infantile iritis, though often puny and cachectic, are also often apparently in good condition. 9. Infants suffering from iritis should always show one or other of the well-recognized symptoms of hereditary taint. 10. Most of those who suffer from syphilitic iritis are infants born within a short period of the date of the primary disease in their parents.]

We have now a group of symptoms characteristic of the secondary period of syphilis, or that extending from birth, or much more commonly from the age of three or four weeks to about the end of the first year. The syphilitic child during this time has several or all of the following symptoms: Coryza with snuffles; an erythematous, papular, or pustular eruption on the skin; mucous patches on the lips, tongue, cheeks, etc.; a marked tendency to general wasting; a hoarse cry or cough; senility of aspect; iritis. The majority of syphilitic children born alive die during this stage.

Before its termination, sometimes even at birth, other lesions have been noticed (especially those affecting the liver), which, however, may better be described in connection with the special organ or organs involved.

Succeeding this stage--_i.e._ beginning in about a year or eighteen {282} months--comes an intermediate period, which extends to second dentition, to puberty, or even much later, and which is characterized rather negatively--that is, by the absence of symptoms--than otherwise. The evidence of the general diathesis will of course be present in the shape possibly of malnutrition, stunted growth, or retarded development, perhaps shown in the weazened or withered face, the sunken nose, the pallor of the skin, the premature loss of the upper incisor teeth or the malformation of the others if they have erupted.

There is but little tendency to recurrence or relapse of any of the secondary symptoms; and in certain cases, not a very small proportion, in which these symptoms have been light and have been well and thoroughly treated, this stage extends throughout life; or, in other words, as is frequently the case with the adult who has followed a proper course of treatment, the disease appears to terminate with the secondary stage. In other cases, however, it recurs, and the symptoms which it then presents may be taken up in connection with the different organs or tissues involved.

Syphilis of the ear is for obvious reasons not often discoverable until the patient has reached an age at which interference with the function of hearing becomes a noticeable phenomena. The only symptom likely to attract attention during the stage of inherited syphilis which we are now considering is a catarrh of the middle ear, which may have for its starting-point some inflammation, ulceration, or mucous patch of the pharynx, causing a temporary or permanent occlusion of the orifices of the Eustachian tubes.[86] This may lead to perforation of the membrana tympani, purulent infiltration of the mastoid cells, etc., and when accompanied by an otorrhoea which attracts attention to the ear will be easily discovered by the physician. These cases are, however, exceptional, otorrhoea only being present in nine out of Hutchinson and Jackson's[87] one hundred cases of inherited syphilis, and consequently but little is known about the frequency or gravity of lesions of the auditory apparatus in the secondary stage of this form of syphilis.[88] The changes which occur later on are chiefly those which involve either the nerves themselves or their distribution in the labyrinth.

[Footnote 86: Bäumler, _Ziemssen's Cyclopædia_, vol. iii. p. 226.]

[Footnote 87: Hutchinson and Hughlings Jackson, _Med. Times and Gaz._, Nov. 23, 1861.]

[Footnote 88: Schwartze (quoted by Hill and Cooper) found also that otorrhoea was a rare complication in deafness from syphilis.]