The relative effect of paternal as compared with maternal influence may be considered after we have described the latter.
Descent from the mother may occur theoretically in consequence of—
1. Infection of the mother previous to conception.
2. Infection of the mother at the moment of conception.
3. Infection of the mother during the period of utero-gestation.
As to the first of these methods of transmitting the disease there is little if any difference of opinion. Even those who claim the most for paternal influence38 include among the conditions which may give rise to syphilis in the child disease of the ovule, and it may be stated as incontrovertible that recent or active syphilis in the mother at the time of conception will almost certainly be followed by syphilis in the child. As a rule, women who have borne syphilitic children, even when they do not give unmistakable evidence of the disease, fail in health, become anæmic, and often develop glandular or osseous swellings which, according to Zeissl,39 are only relieved by antisyphilitic treatment. The bearing of Colles's law upon the alleged immunity in many instances of the mothers of syphilitic children has already been shown (p. 264); and there is other evidence, not perhaps so conclusive, but strongly corroborative, of the same view—viz. that their escape is only apparent, and that syphilis, either latent or active, always affects such mothers.40 There can be no manner of doubt that in every instance at or about the time of childbirth there are not to be found pathognomonic lesions of syphilis, nor do such lesions always make their appearance in cases where the period of observation is a short one; but it is claimed with much show of truth that prolonged and patient inspection of such patients will in time result in the discovery of some symptom which betrays the presence of the disease.
38 Bumstead and Taylor, op. cit., p. 745. Hutchinson in Reynolds's System of Medicine, Am. ed., vol. i. p. 431: "In a large proportion of the cases met with in practice the taint is derived from the father only." (On this point see foot-note to p. 270.) If there were any doubt as to the fact that syphilis in the mother only may be transmitted to the child, it would be removed by the cases of Bardicet and others mentioned in Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 688. In these cases nurses who had contracted the disease from their sucklings subsequently became pregnant (without having infected their husbands) and gave birth to syphilitic children.
39 Jahrbuch, vol. ii. p. 303, 1872.
40 Zeissl, Sigmund, Oewre, Flindt, Woodman, and others are quoted by Hill and Cooper to this effect. Woodman, for other purposes, gave the histories of 200 cases of infantile syphilis. In all of these the mothers had suffered from typical secondary lesions.
The cases in which treatment of the father has resulted in healthy children, whereas without treatment he procreated only syphilitic children, the mother being without either symptom or treatment, have been urged as evidence of the direct descent of syphilis from the father to the child without the intervention or participation of the mother. Doubt has been thrown upon them by those who uphold the contrary view;41 but after looking into them carefully I am constrained to admit that some of them, notably those of Kassowitz42 and R. W. Taylor,43 are convincing of the facts—(1) that treatment of the father controlled the condition of the child, and (2) that there was no evidence that the mother had syphilis. But we have seen that the only proof of the universal infection of mothers of syphilitic children is the law of Colles, and that in many cases the disease is for a long time latent or unrecognizable. Let us admit that this was the condition in the cases in question; it does not at all follow, necessarily, that because the mother has latent or hidden syphilis she must infect her child. Every case even of active syphilis in the parents is not handed down to the children, a certain proportion of whom escape even when both parents are in the height of the secondary stage at the time of conception.44 But the activity of the disease in the children, and even more the likelihood of its reaching them, are in direct proportion to its activity in the parents.45 A mother, therefore, who under the influence of active syphilis in her husband has given birth to two or three syphilitic children, and has herself shown no symptoms of the disease, may nevertheless have it in the latent form and have no strong tendency to transmit it. Consequently, treatment of the father will result in the procreation of healthy children, because it removes the active and efficient cause of their infection. To take any other view of these cases is to assume that every syphilitic parent must hand down the disease to the children—an assumption which is not in consonance with numerous well-attested clinical facts.
41 Hill and Cooper, op. cit., pp. 52, 53.
42 "Die Vererbung der Syphilis," Stricker's Med. Jahrb., 1875, p. 391.
43 Archives of Clinical Surgery, New York, Sept., 1876.
44 Fournier, op. cit., pp. 35-37.
45 Hutchinson, op. cit., p. 431.
Leaving this interesting question, however, we may consider the other methods by which syphilis descends from the mother to the child, having seen already that it is beyond doubt that it may be handed down by disease of the ovule due to syphilis acquired previous to conception, and having seen that there is a high degree of probability that the mother herself rarely, if ever, escapes the disease. The second method, or that in which the mother becomes syphilitic at the moment of conception, has already been sufficiently discussed. It is really, strictly speaking, an example of paternal heredity, as the resulting germ is syphilitic—not because the ovule of the mother was infected, but on account of the disease of the spermatozoid of the father.
There remains for consideration the influence upon the child of a syphilis acquired by the mother during some period of utero-gestation. That under these circumstances the child can become infected has been and is still absolutely denied by some very respectable authorities.46 All that is necessary for proof of its occurrence is, however, (1) freedom of both parents from syphilis at the time of conception, or, in other words, syphilis must have been acquired by both—not alone by the mother—after the beginning of pregnancy; (2) that the syphilis of the child be unmistakably pre-natal—that is, not acquired by some accident during or after birth.
46 Bumstead and Taylor, op. cit., pp. 742, 744. They base their denial, first, on the physiological fact (?) that no interchange of cellular elements between mother and father is possible, and next on the absence of satisfactory evidence of the occurrence of infection during pregnancy. Zeissl's case seems sufficient answer to the last assertion, and there is strong evidence that the first is without good foundation. "The placenta is penetrated by the virus, and does not play the part of a filter for the elementary particles of matter which, so far as we know, represent the true active contagion of the disease. In the absence of direct experiments, which it would be almost impossible to institute, we may argue from the facts known to exist in certain acute infectious diseases in which there are very interesting points of resemblance.
"It has been known for a long time that small-pox occurring in the mother may be transmitted to the product of conception enclosed in the uterus, and it is supposed that the virulent particles traverse the walls of the maternal vessels in order to penetrate the circulating apparatus of the foetus. But what is only a supposition in the case of small-pox seems to have been actually demonstrated in symptomatic charbon (Arloing, Cornevin, and Thomas), bacteridian charbon, and recurrent fever, in which the poisonous element is easily recognized. The recent experiences of Strauss and Chamberland (1882) have shown that the foetus participates in the 'infection charbonneux' of the mother. Albrecht has shown (1880) the presence of numerous spirochoetæ in the blood of the heart of a child born at seven months of a woman with a second attack of relapsing fever. It may be supposed, therefore, though not demonstrated, that the transmission of syphilis takes place by the same method as that of relapsing typhus or of charbon" (Nouveau Dict. de Méd. et de Chir., pp. 682, 683).
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 old48 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.
47 Quoted by Hill and Cooper, op. cit., p. 60.
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 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
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 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.
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 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
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).
"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."
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 describes52 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.
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 syphilis53 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 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.
53 Fraenkel took as his criterion of syphilis the presence of the osteo-chondritis described by Wegner (see p. 286).
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.
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."
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 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.
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.
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.
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 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.
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.
61 If chancre were the first symptom of constitutional syphilis, why should it not appear in cases of hereditary syphilis?
62 Infantile Syphilis, p. 101
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).
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 denied65 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
65 Ariander, Mémoires de Méd. et d'Accouch., quoted by Diday.
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.
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."
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 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 mercury71 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
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."
70 Op. cit., pp. 70-77.
71 Depaul, Gaz. Méd. de Paris, 1851, p. 472, and Galligo, Gaz. Med. Toscana, 1852, p. 123.
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 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.
73 Cornil, op. cit., p. 203.