(Congenital syphilis; Infantile syphilis)
Syphilis may be transmitted by either father or mother to their offspring and, if the fetus has escaped abortion or still-birth, it may be born with or without the presence of visible syphilitic symptoms. In fact, in a large majority of cases, the infant presents every surface indication of a normal child and the disease may not develop for a few weeks or months. However, the majority of infected infants will manifest an eruption within the first month and nearly all exhibit the secondary lesions before the end of the second month. A child may usually be said to have escaped infection if these do not appear within the first six months.
The many symptoms which a syphilitic infant may present, besides the cutaneous lesions, will only briefly be considered. They vary with the effects of the disease upon the different organs or tissues but usually such symptoms as pyrexia, pallor and peevishness first appear, followed by local disturbances of the mucous membranes of the nose, throat, larynx and mouth, of which inflammation of the lining of the nose or “snuffles” is the most common. In some cases, the liver, spleen, eyes and bones may be attacked and such conditions as notched or Hutchinson’s teeth, interstitial keratitis, disturbances of hearing, nodosities on the skull, dactylitis, onychia, paronychia, exostoses and even resulting pseudoparalysis may be noted.
Lesions of the skin may precede the catarrhal symptoms but usually they follow the latter. In a short time the subcutaneous fat is absorbed, the skin becomes loose and wrinkled, and the face sallow and careworn as from worry and age. Added to these surface changes, may be stains of early eruptions and later lesions undergoing evolution and involution. It is my purpose to mention briefly the surface eruptions or syphilids of the hereditary form. These occur chiefly in the earlier period of congenital syphilis and are generally of little importance in the advanced stage when lesions of the deeper tissues are apt to develop, perhaps independently of any cutaneous eruption. The common eruptions of hereditary syphilis are the macular, papular and bullous.
The macular syphilid, like other form of cutaneous syphilis in infants, often appears on the buttocks and about the anus. The lesions vary in size, are not always defined and frequently coalesce to form large areas of coppery or yellowish-red skin. Sometimes the eruption extends down the inner part of the thighs, up the back, and in rare instances becomes general, even spreading to the soles of the feet. There is likely to be more or less desquamation on the dry parts, exfoliation of the surface of the soles, and between the nates, separation of the macerated scales may leave the surface raw or glazed like erythema intertrigo. A less common form of erythema has been observed in syphilitic infants, often in association with ulcerating lesions of the mucous surfaces of the mouth. This eruption occurs in irregular, bright to copper to coppery-red patches of 1/2 inch or more in diameter and is most abundant on the abdomen, lower portion of the chest and inner region of the legs.
The papular syphilid usually develops from the macular and, combined with it, constitutes the commonest syphilid observed in an infant. The lesions may appear as small or large papules in conic, acuminated or flat shapes, pea- to finger-nail-sized, smooth, glistening and of a dull red color. The papulosquamous variety is more common than the pure papular form and, occurring in the folds of the skin where the surfaces are in contact, often develops into moist papules. Moist papules are even more frequent in their relative occurrence and more numerous in their points of distribution than the same lesions in the acquired syphilis. They may develop on any warm or moist lesion of the skin and mucous membrane, especially at the anus and corners of the mouth, often being associated with fissures.
Bullous syphilids are more common in the hereditary than in acquired syphilis and always indicate a condition of marked severity. The prognosis of these cases is particularly bad when the blebs are purulent or rapidly become so. Bullous eruptions are often present at birth or appear in the first ten days of life. They may be tense or flaccid according to the quantity of serum, pus or blood held in them. Their sites of predilection are the palms, soles, nail bed and the lower part of the face, although other parts may be invaded in severe or exceptional cases. Around the lesions a dark red areola is seen and, when they rupture or dry up, light to dark green crusts form which cover an extending ulcer. When the nail bed is the seat, of a syphilitic bleb, the nail often turns black and is finally cast off; in milder cases it may be only distorted in shape, especially at the free border.
Primary vesicular syphilids are as rare in the hereditary as in the acquired disease and usually develop into bullae. But as has been stated before, vesicles may cap small papules, a condition sometimes considered as an intermediate stage.
The pustular syphilids of hereditary syphilis, while more common than the vesicular, are still rare. They may occur upon the apices of small papules or be present as ecthymatous, rupial or furuncular-like lesions. Aside from the first mentioned form they are apt to be associated with profound cachexia.
Tubercular and gummatous syphilids, when present, are a later manifestation and are usually seen in adult life. While they are not so extensive as in the acquired disease, they are like the latter in appearance, involution and evolution and need no separate description here. The brownish-red, dry, fissured and glistening appearance of the palms as a late manifestation is regarded by many as especially characteristic.
The diagnosis of the hereditary syphilid is comparatively easy, especially when the general symptoms, aside from those of the skin, are present. Any diagnosis based upon the latter only is essentially the same as described in acquired syphilis.
Etiology of Syphilis – Syphilis is due to a specific infective parasite, the spirocheta pallida, now classified as the treponema pallidum. It was discovered in 1950 by Schaudinn and E. Hoffmann whose epoch-making researches have since been repeatedly verified by other investigators. Notable among these are metchnikoff, Roux, Lassar and Neisser who have demonstrated that syphilis can be transmitted to chimpanzees and other apes by inoculation. It is needless to discuss the subject of the etiology of hereditary syphilis beyond the fact that it may be transmitted by either or both parents while subject to the disease in its contagious stage.
The acquired form is always due to infection, directly or indirectly, from some person suffering with the active disease and is at the onset a purely local process analogous in many respects to diphtheria, tuberculosis, glanders and leprosy, or diseases in the lesions of which micro-organisms have been proved to be constantly present. In other ways the likeness, especially in the existence of a period of incubation, the outbreak of cutaneous efflorescence and a certain immunity from other attacks lies with the exanthemata or diseases which are markedly contagious and supposedly of microbic origin.
The most common method of infection is through the sexual act by the transmission of the poison from an existing specific lesion on the genitalia. Prostitutes are naturally common carriers in this respect. This principally applies to the genital chancres because unnatural sexual relations are not usually responsible for the extragenital sores. The latter, while not uncommon, are often due to accidental and innocent inoculation, such as might occur by the act of kissing, through eating and drinking utensils used in common, communion cups, toilet articles or barber shop instruments. In professional work the poison may be communicated through instruments employed in circumcision, vaccination and dentistry, although fortunately these cases are rare with the improved technic now in vogue. There are many opportunities for infection in the industrial world but the danger of contagion though possible is slight. Physicians, dentists, nurse and other hospital attendants are not infrequently infected, especially on the fingers, from coming in contact with the virus.
Toward the development of the syphilids, other factors, besides the specific virus, contribute and account in a large degree for their varying course. These are not peculiar to syphilis and are contributing rather than predisposing. Constitutional impairment due to malaria, scrofula, alcoholism, infancy and old age and often some unknown cause not necessarily apparent in any marked disturbance of health, contribute to the insidious spread throughout the system of the parasite or its toxins. The symptoms produced by these contributory conditions, together with the variable potency or attenuation of the syphilitic poison inoculated, give individuality to each case of the disease. Another set of causes, chiefly external, beyond the normal difference existent in the skin of different individuals, may operate to modify the development and course of some of the syphilids. The presence of other diseases such as eczema or seborrhea may modify or aggravate the behavior of the secondary eruptions. Slight injuries of the skin may determine the seat of lesions, more especially those of the tertiary period. Lachesis of cleanliness may contribute to secondary infection from pus cocci or other microorganisms.
Pathology of Syphilis – The microscopic anatomy of syphilis is essentially that of an infective granulomata and consists of round-cell infiltration surrounding the blood vessels and lymphatics. The papular, tubercular and gummatous lesions show a variable but scanty number of giant cells. While the initial changes are in the upper part of the corium, the deeper structures are sooner or later involved in the process. Syphilis differs from other new-growths by the absence of any tendency toward organization, the retrogressive steps occurring from fatty degeneration and absorption or from necrosis and ulceration. Renaut claims that the different syphilitic lesions are structurally the same from an anatomical standpoint and that they show a reactionary defence against the pathogenic agent, causing an endarteritis of a special kind, with slow obliteration and a primary tendency to excite hypertrophy of the tissues about the causal agent.
Prognosis of Syphilis – The large majority of acquired cases respond to treatment. Rarely a long neglected or malignant type proves fatal but such an outcome may be expected in many infantile hereditary cases. The chances for a speedy and complete cure of syphilis rest on several factors, namely, the willingness or ability of the patient to lead an approved hygienic life; the possession of the average degree of resistance to syphilitic invasion; and conscientious attention to continued treatment until successive Wassermann tests are invariably negative. Neither the number or size of the lesions, nor their tendency to suppurate, nor the degree of constitutional disturbance is any indication of the future course of the disease. Frequently those who have only slightly annoying secondaries have troublesome tertiary manifestations and vice versa. Secondary syphilids are usually benign and self-limited in duration and the importance of their treatment consists largely in the prevention of later lesions; in fact, inefficient care of this stage is the chief etiological factor in the appearance of tertiary manifestations. In the tertiary period the earlier the recognition of the lesions and the application of treatment, the more favorable the prognosis.
TReatment of Syphilis – Effective public supervision of syphilis seems impossible and impracticable, owing to ignorance or mistaken sentiments. This is remarkable in view of the number of innocent victims of the disease and the dire effects which may follow the mildest primary manifestations. Syphilis should be classed with other contagious diseases and systematic protective regulations enforced for the benefit of the people. Prevention at present rests in the hands of the individual practitioner who can only advise and insist that his patient take proper means to prevent the infection of others.
Primary syphilis can be managed in various ways, depending on equipment and experience. Where the physician is not equipped with the facilities for examining serum smears for the presence of the spirochete pallida and thus making an immediate and positive diagnosis, the treatment of a suspected case consists of local cleanliness and general hygienic measures such as regular exercise, sleep and avoidance of dietary excesses. Cleanliness of the suspicious lesion may be accomplished by thorough bathing of the parts at least three times a day with a solution of mercuric chlorid (1:5000), the lesion in the meantime being covered with an antiseptic powder like boric acid, iodol or calomel and protected by a loose covering of sterile gauze. Excision of the initial lesion is warmly commended by some authors because it rids the patient of the greatest focus of infection. Without specifying the real nature of the disease, the patient should be warned of the possibility of infecting others and be kept under observation until the diagnosis is verified by the advent of such manifestations as general adenopathy, fever, headache and erythema, which occur within six weeks after the appearance of the initial lesion, if the suspected diagnosis proves correct. It is argued that syphilis should not be allowed to progress thus far without treatment and, while I acknowledge the soundness of this argument, I advise against it, for it is far better that a man who has syphilis should run a few days without treatment than that a mistake should be made and a patient condemned to suffer for a lifetime the torments of a subjective syphilis.
The constitutional treatment of active syphilis embraces all the factors that contribute to improve the patient’s hygiene and make for healthy surroundings such as change of air, simple diet including abstinence from alcohol and stimulating, spiced or rich foods, moderate use of tobacco, out of door life, regular sleep and general tonic treatment if anemia be present.
Internal Treatment – Just as soon as secondary symptoms appear or as soon as a positive diagnosis can be made by the finding of the spirochetes in the serum of the initial lesion, active internal treatment should be commenced. The advent of the Wassermann test and its modifications and the introduction of salvarsan and neosalvarsan have revolutionized the treatment of syphilis but it is well to remember that we have other specific means which for a long time have proven efficacious. It will be some years before positive data concerning the permanent effects of salvarsan can be known and at this time our enthusiasm over its action should be tempered by its conservative application.
Mercury is deservedly popular having been successfully used for centuries and while thousands have been cured by its use, some individuals cannot be reached by doses that can be tolerated. It may be administered by mouth, by inunction, by fumigation and by hypodermic and intravenous injections.
The relation of the action of mercury and its salts to the syphilitic processes is most readily understood by comparing the manifestations of secondary syphilis with the provings of mercury as verified and arranged in Allen’s Handbook of Materia Medica. It is the proneness to suppurative and ulcerative destruction of the surface tissues in the action of mercury which points to its applicability in the treatment of syphilids because it is this tendency we most desire to prevent and combat and over which mercury often exhibits a magical influence. A mercurial may be given with the beginning of the secondary symptoms or eruptions in frequent and fairly large doses of the lower decimal attenuations, but never to the extent of producing salivation or for an unlimited time, lest the general health of the patient suffer or a mercury-fast strain of spirochete pallida be developed. Mercury may be omitted every few weeks and another indicated remedy prescribed in the interim of days or weeks, but this alternating mercurial treatment should be continued for two or three years with a gradual lessening of the dose and lengthening of intervals in the last half of the course. However, it is rare that such a course is pursued nowadays because the newer methods of treatment are so well combined with early mercurial medication. I see no reason to change my opinion that the selection of the mercurial should be based upon its symptomatic relations so choice may be made of Mercurius sol. (1x), Mercurius dulc. (1x), Mercurius cor. (2x or 3x), Mercurius protoiodid (1x or 2x), Mercurius biniodid (1x or 2x) and rarely Cinnabar in the lower potencies. According to the needs of the case, the interval of dosage may vary from two to six hours and the one grain decimal tablet triturates are the most convenient form to use.
Inunctions produce a rapid effect and are correspondingly efficient. The process consists in rubbing into a portion of the skin, selected and prepared for the purpose, 20 to 60 grains of a 25 to 50 per cent. mercurial ointment made with fresh lard. It is best to arrange to give inunctions in courses of six. The surface of the body is divided into six portions, (1) right arm, (2) left arm, (3) right leg, (4) left leg, (5) the back, (6) the abdomen and chest. It is not important to include the head, neck or hands but, if inunctions are made in these exposed regions, a white precipitate or calomel ointment may be substituted for the ordinary blue ointment. Before an inunction the portion of the surface to be rubbed should be scrubbed with a lather of soap, washed off with hot water, dried and then wiped over with alcohol. If the operator’s hands are sound he need only protect them with an application of oil or soap before, and thoroughly wash them at the end of the rubbing; if abrasions or cracks exist rubber gloves may be worn. About half an hour is required to make a satisfactory inunction, and it is usually repeated every night, until a course of six has been given. Later another course may be started if needed, or other methods of treatment substituted. The patient should always be examined before each inunction and if any tendency to salivation appears, the treatment should be suspended for a suitable time. It is generally better to wash off the remains of one application just before another is made in a different region, thus keeping only one area anointed at one time.
Fumigations with mercurial vapor are occasionally used, especially for persistent and localized eruptions and for short periods of treatment but they are not suitable for debilitated subjects. They are given in the same manner as the domestic hot air bath. The patient, after thoroughly washing the skin, is seated naked on a cane-bottom chair, blankets thrown about him and a special vaporizing lamp containing thirty grains of calomel or forty grains of cinnabar is lighted and placed underneath the chair. Very soon free perspiration begins; in fifteen to twenty minutes the drugs are entirely evaporated. The lamp is then removed and after the patient is cooled off a little, he retires to bed with the same blankets wrapped about him. On the following day the patient should be warmly clad, wearing flannels next to the skin and take care not go get chilled. The bath can be repeated two or three times a week according to the effect desired or obtained, but it should rarely be employed for more than four weeks and should be discontinued at any time if it produces any ill effects.
Hypodermic injections – The administration of mercury hypodermatically is deservedly the most popular method. It allows of definite doses entirely under the control of the physician and neither irritates the stomach nor the skin. Against the hypodermic method there stands the element of pain and the possibility of abscess formation. These can be obviated by careful technic. If the inside of the needles and syringes are thoroughly cleansed before being boiled and the skin at the site of injection is rubbed with a sponge saturated with ether, dried and painted with tincture of iodin and the needle thrust deeply into the muscle, there will be no abscesses unless one is careless and contaminates the needle while giving the hypodermic. Both the soluble and insoluble mercurial salts are used but the latter produce greater and more persistent pain and are more liable to abscess formation because of their slower absorption. Among the soluble preparations, mercuric chlorid, in doses of 1/12 o 3/8 of a grain,is usually advocated. This may be deeply injected on alternate days, into the gluteous or trapezius muscles. Soluble mercurials such as the succinamid, albuminate, peptonate, carbolate, benzoate, sozoiodolate and others have been used as well as such insoluble compounds as the salicylates of mercury, calomel and metallic mercury (oleum cinereum). The salicylate is probably the most popular of the last group. Intravenous injections of mercuric chlorid (15 drops of a 1 to 1000 strength in normal salt solution is the usual dose) have been recommended. While the effect is rapid and may be painless, it presents little advantage over the intramuscular method.
The iodids of potassium, sodium or ammonium may be needed in any period of syphilis but ordinarily they are most useful in the late secondary or tertiary periods. Usually the first named salt is prescribed in 5 to 15 grain doses in some liquid medium, three times a day after meals. But when special organs are involved, it may be necessary to rapidly increase the dose to 60 or more grains three times a day. The action of potassium iodid is to subdue rather than cure syphilis and, in the secondary stage of the disease, it should be discontinued when it has accomplished its special work. In the tertiary period when there is a continuation or revival of cell products from syphilis without contagion, the iodid should be given longer to subdue the slower but more dense and persistent tendency to infiltration of the skin and other tissues. In tertiary syphilis mercury is still needed to complete a cure and the biniodid or bichlorid can be administered with the potassium iodid (mixed treatment) or better still, in alternation with the latter. The tendency of to-day is to treat all late secondary and many tertiary lesions of syphilis with salvarsan. Until more is known of the ultimate course of syphilis treated only with salvarsan, it is wisest to take advantage of its immediate action but not to rely upon it for the cure of syphilis.
Salvarsan, often known by its laboratory number “606” is a synthetic arsenical compound bearing the chemic name of dioxydiamidoarsenobenzol, introduced by Ehrlich in 1910. The form in which it is dispensed, a yellowish powder, contains 34 per cent. of arsenic but owing to its peculiar molecular form, arsenical poisoning is not developed. While this remedy may be used advantageously in any form of syphilis, at this early date it may be asserted that it is especially suitable to those cases that mercury has not relived nor prevented relapses, in the malignant and rapidly destructive forms, in severe ulcerations of the mucous membranes, in visceral, nerve and hereditary forms, in those cases presenting severe syphilitic cachexia, in latent cases in which the Wassermann test is persistently positive, in the early stages of tabes dorsalis and paresis, and in the earliest period to abort the regular course of syphilis. It can likewise be stated that salvarsan should not be employed in cases of severe non-syphilitic cachexia, myocarditis or other equally grave heart disease, advanced nephritis, or pronounced disease of the central nervous system.
There is much difference of opinion as to what will eventually prove the best method of salvarsan administration. The subcutaneous method may be dismissed by saying that it is the least efficacious mode. The intramuscular injection is commonly used because it is easier, demands less technical skill and incapacitates the patient less than the most successful method, the intravenous. The former is usually injected deep into the gluteal region while the latter is inserted in a distended vein of the forearm. The usual dose is 0.6 gram of salvarsan but it may be expedient to give fractional doses of 0.1 gram or more at intervals of three to six days rather than the full dose. The vehicles used in the intramuscular procedure are sterile water or a thin sterile oil while choice may be had of warm sterile water or warm normal salt solution for the intravenous method. The latter should be employed when possible because it exerts the most rapid effect on existing lesions, is more quickly eliminated from the system, is practically painless and does not produce tumefactions, but it should be done with all the skill, technic and surroundings of a surgical operation; in fact the patient should remain in the hospital two days so that the reactive symptoms of chill, fever, nausea, Diarrhoea, etc., which may appear in from one to six hours after the injection and which are more apt to follow the intravenous method, may come and go before his discharge.
Neosalvarsan whose laboratory number is “914” is a later preparation and more soluble. It is obtained by the addition of formaldehyd sulphoxylate of soda and while as efficient as salvarsan, is more useful because larger doses can be used at much shorter intervals besides permitting a much simpler technic. Hectin, containing 21 per cent. of arsenic, and sodium cacodylate, containing 35 per cent. of arsenic, have been used hypodermically with good success in a number of reported cases, including a few of my own.
Judging from my own experience salvarsan or neosalvarsan plus mercury and occasionally the iodids, presents the ideal treatment for syphilis. It seems as if the combination of arsenic and mercury affects the blood reaction more effectually than either remedy used alone. It is impossible to say with any exaction how many doses are necessary but ordinarily in the early period, three to five, at week intervals, plus a mercurial are sufficient, while in the later periods, three to eight, plus several mercurial courses may be essential. However, a single injection will often benefit if properly supported by other treatment.
External treatment – The unbroken lesions of the secondary period require no local treatment other than systematic cleanliness. For pustular eruptions and moist papules the use of boric acid or sublimate soap is advisable for local or general bathing. Ulcers resulting from pustular lesions may be induced to heal more rapidly by washing them with a mercuric chlorid solution (1:2000) and applying boric acid, calomel or aristol powders or an ointment consisting of calomel 33 per cent., lanolin 57 per cent. and vaseline 10 per cent. I have used mild fulguration and 10 second applications of solidified carbon dioxid with good results in many applications of solidified carbon dioxid with good results in many instances of sluggish ulceration of the late secondary or tertiary periods, combined with the mode of treatment mentioned just previously. The presence of syphilitic ulcers shows insufficient treatment. It may be that more mercury should be given or that mercury has been given and is ineffective. Such cases call for salvarsan, after which it is not uncommon to find the mercury- fast condition corrected.