Manual of Surgery/Chapter IX
Definition.--Virus.--ACQUIRED SYPHILIS--Primary period:
_Incubation, primary chancre, glandular enlargement_; _Extra-genital chancres_--Treatment--Secondary period: _General symptoms, skin affections, mucous patches, affections of bones, joints, eyes_, etc.--Treatment: _Salvarsan_--_Methods of administering mercury_--Syphilis and marriage--Intermediate stage--_Reminders_--Tertiary period: _General symptoms_, _gummata_, _tertiary ulcers_, _tertiary lesions of skin, mucous membrane, bones, joints_, etc.--Second attacks.--INHERITED SYPHILIS--Transmission--_Clinical features in infancy, in later life_--Contagiousness--Treatment.
Syphilis is an infective disease due to the entrance into the body of a specific virus. It is nearly always communicated from one individual to another by contact infection, the discharge from a syphilitic lesion being the medium through which the virus is transmitted, and the seat of inoculation is almost invariably a surface covered by squamous epithelium. The disease was unknown in Europe before the year 1493, when it was introduced into Spain by Columbus' crew, who were infected in Haiti, where the disease had been endemic from time immemorial (Bloch).
The granulation tissue which forms as a result of the reaction of the tissues to the presence of the virus is chiefly composed of lymphocytes and plasma cells, along with an abundant new formation of capillary blood vessels. Giant cells are not uncommon, but the endothelioid cells, which are so marked a feature of tuberculous granulation tissue, are practically absent.
When syphilis is communicated from one individual to another by contact infection, the condition is spoken of as _acquired syphilis_, and the first visible sign of the disease appears at the site of inoculation, and is known as _the primary lesion_. Those who have thus acquired the disease may transmit it to their offspring, who are then said to suffer from _inherited syphilis_.
- The Virus of Syphilis.#--The cause of syphilis, whether acquired or
inherited, is the organism, described by Schaudinn and Hoffman, in 1905, under the name of _spirochaeta pallida_ or _spironema pallidum_. It is a delicate, thread-like spirilla, in length averaging from 8 to 10 [micron] and in width about 0.25 [micron], and is distinguished from other spirochaetes by its delicate shape, its dead-white appearance, together with its closely twisted spiral form, with numerous undulations (10 to 26), which are perfectly regular, and are characteristic in that they remain the same during rest and in active movement (Fig. 36). In a fresh specimen, such as a scraping from a hard chancre suspended in a little salt solution, it shows active movements. The organism is readily destroyed by heat, and perishes in the absence of moisture. It has been proved experimentally that it remains infective only up to six hours after its removal from the body. Noguchi has succeeded in obtaining pure cultures from the infected tissues of the rabbit.
[Illustration: FIG. 36.--Spirochaeta pallida from scraping of hard Chancre of Prepuce. x 1000 diam. Burri method.]
The spirochaete may be recognised in films made by scraping the deeper parts of the primary lesion, from papules on the skin, or from blisters artificially raised on lesions of the skin or on the immediately adjacent portion of healthy skin. It is readily found in the mucous patches and condylomata of the secondary period. It is best stained by Giemsa's method, and its recognition is greatly aided by the use of the ultra-microscope.
The spirochaete has been demonstrated in every form of syphilitic lesion, and has been isolated from the blood--with difficulty--and from lymph withdrawn by a hollow needle from enlarged lymph glands. The saliva of persons suffering from syphilitic lesions of the mouth also contains the organism.
[Illustration: FIG. 37.--Spirochaeta refrigerans from scraping of Vagina. x 1000 diam. Burri method.]
In tertiary lesions there is greater difficulty in demonstrating the spirochaete, but small numbers have been found in the peripheral parts of gummata and in the thickened patches in syphilitic disease of the aorta. Noguchi and Moore have discovered the spirochaete in the brain in a number of cases of general paralysis of the insane. The spirochaete may persist in the body for a long time after infection; its presence has been demonstrated as long as sixteen years after the original acquisition of the disease.
In inherited syphilis the spirochaete is present in enormous numbers throughout all the organs and fluids of the body.
Considerable interest attaches to the observations of Metchnikoff, Roux, and Neisser, who have succeeded in conveying syphilis to the chimpanzee and other members of the ape tribe, obtaining primary and secondary lesions similar to those observed in man, and also containing the spirochaete. In animals the disease has been transmitted by material from all kinds of syphilitic lesions, including even the blood in the secondary and tertiary stages of the disease. The primary lesion is in the form of an indurated papule, in every respect resembling the corresponding lesion in man, and associated with enlargement and induration of the lymph glands. The primary lesion usually appears about thirty days after inoculation, to be followed, in about half the cases, by secondary manifestations, which are usually of a mild character; in no instance has any tertiary lesion been observed. The severity of the affection amongst apes would appear to be in proportion to the nearness of the relationship of the animal to the human subject. The eye of the rabbit is also susceptible to inoculation from syphilitic lesions; the material in a finely divided state is introduced into the anterior chamber of the eye.
Attempts to immunise against the disease have so far proved negative, but Metchnikoff has shown that the inunction of the part inoculated with an ointment containing 33 per cent. of calomel, within one hour of infection, suffices to neutralise the virus in man, and up to eighteen hours in monkeys. He recommends the adoption of this procedure in the prophylaxis of syphilis.
Noguchi has made an emulsion of dead spirochaetes which he calls _luetin_, and which gives a specific reaction resembling that of tuberculin in tuberculosis, a papule or a pustule forming at the site of the intra-dermal injection. It is said to be most efficacious in the tertiary and latent forms of syphilis, which are precisely those forms in which the diagnosis is surrounded with difficulties.
In the vast majority of cases, infection takes place during the congress of the sexes. Delicate, easily abraded surfaces are then brought into contact, and the discharge from lesions containing the virus is placed under favourable conditions for conveying the disease from one person to the other. In the male the possibility of infection taking place is increased if the virus is retained under cover of a long and tight prepuce, and if there are abrasions on the surface with which it comes in contact. The frequency with which infection takes place on the genitals during sexual intercourse warrants syphilis being considered a venereal disease, although there are other ways in which it may be contracted.
Some of these imply direct contact--such, for example, as kissing, the digital examination of syphilitic patients by doctors or nurses, or infection of the surgeon's fingers while operating upon a syphilitic patient. In suckling, a syphilitic wet nurse may infect a healthy infant, or a syphilitic infant may infect a healthy wet nurse. In other cases the infection is by indirect contact, the virus being conveyed through the medium of articles contaminated by a syphilitic patient--such, for example, as surgical instruments, tobacco pipes, wind instruments, table utensils, towels, or underclothing. Physiological secretions, such as saliva, milk, or tears, are not capable of communicating the disease unless contaminated by discharge from a syphilitic sore. While the saliva itself is innocuous, it can be, and often is, contaminated by the discharge from mucous patches or other syphilitic lesions in the mouth and throat, and is then a dangerous medium of infection. Unless these extra-genital sources of infection are borne in mind, there is a danger of failing to recognise the primary lesion of syphilis in unusual positions, such as the lip, finger, or nipple. When the disease is thus acquired by innocent transfer, it is known as _syphilis insontium_.
- Stages or Periods of Syphilis.#--Following the teaching of Ricord, it
is customary to divide the life-history of syphilis into three periods or stages, referred to, for convenience, as primary, secondary, and tertiary. This division is to some extent arbitrary and artificial, as the different stages overlap one another, and the lesions of one stage merge insensibly into those of another. Wide variations are met with in the manifestations of the secondary stage, and histologically there is no valid distinction to be drawn between secondary and tertiary lesions.
_The primary period_ embraces the interval that elapses between the initial infection and the first constitutional manifestations,--roughly, from four to eight weeks,--and includes the period of incubation, the development of the primary sore, and the enlargement of the nearest lymph glands.
_The secondary period_ varies in duration from one to two years, during which time the patient is liable to suffer from manifestations which are for the most part superficial in character, affecting the skin and its appendages, the mucous membranes, and the lymph glands.
_The tertiary period_ has no time-limit except that it follows upon the secondary, so that during the remainder of his life the patient is liable to suffer from manifestations which may affect the deeper tissues and internal organs as well as the skin and mucous membranes.
- Primary Syphilis.#--_The period of incubation_ represents the interval
that elapses between the occurrence of infection and the appearance of the primary lesion at the site of inoculation. Its limits may be stated as varying from two to six weeks, with an average of from twenty-one to twenty-eight days. While the disease is incubating, there is nothing to show that infection has occurred.
_The Primary Lesion._--The incubation period having elapsed, there appears at the site of inoculation a circumscribed area of infiltration which represents the reaction of the tissues to the entrance of the virus. The first appearance is that of a sharply defined papule, rarely larger than a split pea. Its surface is at first smooth and shiny, but as necrosis of the tissue elements takes place in the centre, it becomes concave, and in many cases the epithelium is shed, and an ulcer is formed. Such an ulcer has an elevated border, sharply cut edges, an indurated base, and exudes a scanty serous discharge; its surface is at first occupied by yellow necrosed tissue, but in time this is replaced by smooth, pale-pink granulation tissue; finally, epithelium may spread over the surface, and the ulcer heals. As a rule, the patient suffers little discomfort, and may even be ignorant of the existence of the lesion, unless, as a result of exposure to mechanical or septic irritation, ulceration ensues, and the sore becomes painful and tender, and yields a purulent discharge. The primary lesion may persist until the secondary manifestations make their appearance, that is, for several weeks.
It cannot be emphasised too strongly that the induration of the primary lesion, which has obtained for it the name of "hard chancre," is its most important characteristic. It is best appreciated when the sore is grasped from side to side between the finger and thumb. The sensation on grasping it has been aptly compared to that imparted by a nodule of cartilage, or by a button felt through a layer of cloth. The evidence obtained by touch is more valuable than that obtained by inspection, a fact which is made use of in the recognition of _concealed chancres_--that is, those which are hidden by a tight prepuce. The induration is due not only to the dense packing of the connective-tissue spaces with lymphocytes and plasma cells, but also to the formation of new connective-tissue elements. It is most marked in chancres situated in the furrow between the glans and the prepuce.
_In the male_, the primary lesion specially affects certain _situations_, and the appearances vary with these: (1) On the inner aspect of the prepuce, and in the fold between the prepuce and the glans; in the latter situation the induration imparts a "collar-like" rigidity to the prepuce, which is most apparent when it is rolled back over the corona. (2) At the orifice of the prepuce the primary lesion assumes the form of multiple linear ulcers or fissures, and as each of these is attended with infiltration, the prepuce cannot be pulled back--a condition known as _syphilitic phimosis_. (3) On the glans penis the infiltration may be so superficial that it resembles a layer of parchment, but if it invades the cavernous tissue there is a dense mass of induration. (4) On the external aspect of the prepuce or on the skin of the penis itself. (5) At either end of the torn fraenum, in the form of a diamond-shaped ulcer raised above the surroundings. (6) In relation to the meatus and canal of the urethra, in either of which situations the swelling and induration may lead to narrowing of the urethra, so that the urine is passed with pain and difficulty and in a minute stream; stricture results only in the exceptional cases in which the chancre has ulcerated and caused destruction of tissue. A chancre within the orifice of the urethra is rare, and, being concealed from view, it can only be recognised by the discharge from the meatus and by the induration felt between the finger and thumb on palpating the urethra.
_In the female_, the primary lesion is not so typical or so easily recognised as in men; it is usually met with on the labia; the induration is rarely characteristic and does not last so long. The primary lesion may take the form of condylomata. Indurated oedema, with brownish-red or livid discoloration of one or both labia, is diagnostic of syphilis.
The hard chancre is usually solitary, but sometimes there are two or more; when there are several, they are individually smaller than the solitary chancre.
It is the exception for a hard chancre to leave a visible scar, hence, in examining patients with a doubtful history of syphilis, little reliance can be placed on the presence or absence of a scar on the genitals. When the primary lesion has taken the form of an open ulcer with purulent discharge, or has sloughed, there is a permanent scar.
_Infection of the adjacent lymph glands_ is usually found to have taken place by the time the primary lesion has acquired its characteristic induration. Several of the glands along Poupart's ligament, on one or on both sides, become enlarged, rounded, and indurated; they are usually freely movable, and are rarely sensitive unless there is superadded septic infection. The term _bullet-bubo_ has been applied to them, and their presence is of great value in diagnosis. In a certain number of cases, one of the main _lymph vessels_ on the dorsum of the penis is transformed into a fibrous cord easily recognisable on palpation, and when grasped between the fingers appears to be in size and consistence not unlike the vas deferens.
_Concealed chancre_ is the term applied when one or more chancres are situated within the sac of a prepuce which cannot be retracted. If the induration is well marked, the chancre can be palpated through the prepuce, and is tender on pressure. As under these conditions it is impossible for the patient to keep the parts clean, septic infection becomes a prominent feature, the prepuce is oedematous and inflamed, and there is an abundant discharge of pus from its orifice. It occasionally happens that the infection assumes a virulent character and causes sloughing of the prepuce--a condition known as _phagedaena_. The discharge is then foul and blood-stained, and the prepuce becomes of a dusky red or purple colour, and may finally slough, exposing the glans.
_Extra-genital or Erratic Chancres_ (Fig. 38).--Erratic chancre is the term applied by Jonathan Hutchinson to the primary lesion of syphilis when it appears on parts of the body other than the genitals. It differs in some respects from the hard chancre as met with on the penis; it is usually larger, the induration is more diffused, and the enlarged glands are softer and more sensitive. The glands in nearest relation to the sore are those first affected, for example, the epitrochlear or axillary glands in chancre of the finger; the submaxillary glands in chancre of the lip or mouth; or the pre-auricular gland in chancre of the eyelid or forehead. In consequence of their divergence from the typical chancre, and of their being often met with in persons who, from age, surroundings, or moral character, are unlikely subjects of venereal disease, the true nature of erratic chancres is often overlooked until the persistence of the lesion, its want of resemblance to anything else, or the onset of constitutional symptoms, determines the diagnosis of syphilis. A solitary, indolent sore occurring on the lip, eyelid, finger, or nipple, which does not heal but tends to increase in size, and is associated with induration and enlargement of the adjacent glands, is most likely to be the primary lesion of syphilis.
[Illustration: FIG. 38.--Primary Lesion on Thumb, with Secondary Eruption on Forearm.]
 From _A System of Syphilis_, vol. ii., edited by D'Arcy Power and J. Keogh Murphy, Oxford Medical Publications.
- The Soft Sore, Soft Chancre, or Chancroid.#--The differential diagnosis
of syphilis necessitates the consideration of the _soft sore_, _soft chancre_, or _chancroid_, which is also a common form of venereal disease, and is due to infection with a virulent pus-forming bacillus, first described by Ducrey in 1889. Ducrey's bacillus occurs in the form of minute oval rods measuring about 1.5 [micron] in length, which stain readily with any basic aniline dye, but are quickly decolorised by Gram's method. They are found mixed with other organisms in the purulent discharge from the sore, and are chiefly arranged in small groups or in short chains. Soft sores are always contracted by direct contact from another individual, and the incubation period is a short one of from two to five days. They are usually situated in the vicinity of the fraenum, and, in women, about the labia minora or fourchette; they probably originate in abrasions in these situations. They appear as pustules, which are rapidly converted into small, acutely inflamed ulcers with sharply cut, irregular margins, which bleed easily and yield an abundant yellow purulent discharge. They are devoid of the induration of syphilis, are painful, and nearly always multiple, reproducing themselves in successive crops by auto-inoculation. Soft sores are often complicated by phimosis and balanitis, and they frequently lead to infection of the glands in the groin. The resulting bubo is ill-defined, painful, and tender, and suppuration occurs in about one-fourth of the cases. The overlying skin becomes adherent and red, and suppuration takes place either in the form of separate foci in the interior of the individual glands, or around them; in the latter case, on incision, the glands are found lying bathed in pus. Ducrey's bacillus is found in pure culture in the pus. Sometimes other pyogenic organisms are superadded. After the bubo has been opened the wound may take on the characters of a soft sore.
_Treatment._--Soft sores heal rapidly when kept clean. If concealed under a tight prepuce, an incision should be made along the dorsum to give access to the sores. They should be washed with eusol, and dusted with a mixture of one part iodoform and two parts boracic or salicylic acid, or, when the odour of iodoform is objected to, of equal parts of boracic acid and carbonate of zinc. Immersion of the penis in a bath of eusol for some hours daily is useful. The sore is then covered with a piece of gauze kept in position by drawing the prepuce over it, or by a few turns of a narrow bandage. Sublimed sulphur frequently rubbed into the sore is recommended by C. H. Mills. If the sores spread in spite of this, they should be painted with cocaine and then cauterised. When the glands in the groin are infected, the patient must be confined to bed, and a dressing impregnated with ichthyol and glycerin (10 per cent.) applied; the repeated use of a suction bell is of great service. Harrison recommends aspiration of a bubonic abscess, followed by injection of 1 in 20 solution of tincture of iodine into the cavity; this is in turn aspirated, and then 1 or 2 c.c. of the solution injected and left in. This is repeated as often as the cavity refills. It is sometimes necessary to let the pus out by one or more small incisions and continue the use of the suction bell.
_Diagnosis of Primary Syphilis._--In cases in which there is a history of an incubation period of from three to five weeks, when the sore is indurated, persistent, and indolent, and attended with bullet-buboes in the groin, the diagnosis of primary syphilis is not difficult. Owing, however, to the great importance of instituting treatment at the earliest possible stage of the infection, an effort should be made to establish the diagnosis without delay by demonstrating the spirochaete. Before any antiseptic is applied, the margin of the suspected sore is rubbed with gauze, and the serum that exudes on pressure is collected in a capillary tube and sent to a pathologist for microscopical examination. A better specimen can sometimes be obtained by puncturing an enlarged lymph gland with a hypodermic needle, injecting a few minims of sterile saline solution and then aspirating the blood-stained fluid.
The Wassermann test must not be relied upon for diagnosis in the early stage, as it does not appear until the disease has become generalised and the secondary manifestations are about to begin. The practice of waiting in doubtful cases before making a diagnosis until secondary manifestations appear is to be condemned.
Extra-genital chancres, _e.g._ sores on the fingers of doctors or nurses, are specially liable to be overlooked, if the possibility of syphilis is not kept in mind.
It is important to bear in mind _the possibility of a patient having acquired a mixed infection_ with the virus of soft chancre, which will manifest itself a few days after infection, and the virus of syphilis, which shows itself after an interval of several weeks. This occurrence was formerly the source of much confusion in diagnosis, and it was believed at one time that syphilis might result from soft sores, but it is now established that syphilis does not follow upon soft sores unless the virus of syphilis has been introduced at the same time. The practitioner must be on his guard, therefore, when a patient asks his advice concerning a venereal sore which has appeared within a few days of exposure to infection. Such a patient is naturally anxious to know whether he has contracted syphilis or not, but neither a positive nor a negative answer can be given--unless the spirochaete can be identified.
Syphilis is also to be diagnosed from _epithelioma_, the common form of cancer of the penis. It is especially in elderly patients with a tight prepuce that the induration of syphilis is liable to be mistaken for that associated with epithelioma. In difficult cases the prepuce must be slit open.
Difficulty may occur in the diagnosis of primary syphilis from _herpes_, as this may appear as late as ten days after connection; it commences as a group of vesicles which soon burst and leave shallow ulcers with a yellow floor; these disappear quickly on the use of an antiseptic dusting powder.
Apprehensive patients who have committed sexual indiscretions are apt to regard as syphilitic any lesion which happens to be located on the penis--for example, acne pustules, eczema, psoriasis papules, boils, balanitis, or venereal warts.
_The local treatment_ of the primary sore consists in attempting to destroy the organisms _in situ_. An ointment made up of calomel 33 parts, lanoline 67 parts, and vaseline 10 parts (Metchnikoff's cream) is rubbed into the sore several times a day. If the surface is unbroken, it may be dusted lightly with a powder composed of equal parts of calomel and carbonate of zinc. A gauze dressing is applied, and the penis and scrotum should be supported against the abdominal wall by a triangular handkerchief or bathing-drawers; if there is inflammatory oedema the patient should be confined to bed.
In _concealed chancres_ with phimosis, the sac of the prepuce should be slit up along the dorsum to admit of the ointment being applied. If phagedaena occurs, the prepuce must be slit open along the dorsum, or if sloughing, cut away, and the patient should have frequent sitz baths of weak sublimate lotion. When the chancre is within the meatus, iodoform bougies are inserted into the urethra, and the urine should be rendered bland by drinking large quantities of fluid.
General treatment is considered on p. 149.
- Secondary Syphilis.#--The following description of secondary syphilis
is based on the average course of the disease in untreated cases. The onset of constitutional symptoms occurs from six to twelve weeks after infection, and the manifestations are the result of the entrance of the virus into the general circulation, and its being carried to all parts of the body. The period during which the patient is liable to suffer from secondary symptoms ranges from six months to two years.
In some cases the general health is not disturbed; in others the patient is feverish and out of sorts, losing appetite, becoming pale and anaemic, complaining of lassitude, incapacity for exertion, headache, and pains of a rheumatic type referred to the bones. There is a moderate degree of leucocytosis, but the increase is due not to the polymorpho-nuclear leucocytes but to lymphocytes. In isolated cases the temperature rises to 101 or 102 F. and the patient loses flesh. The lymph glands, particularly those along the posterior border of the sterno-mastoid, become enlarged and slightly tender. The hair comes out, eruptions appear on the skin and mucous membranes, and the patient may suffer from sore throat and affections of the eyes. The local lesions are to be regarded as being of the nature of reactions against accumulations of the parasite, lymphocytes and plasma cells being the elements chiefly concerned in the reactive process.
_Affections of the Skin_ are among the most constant manifestations. An evanescent macular rash, not unlike that of measles--_roseola_--is the first to appear, usually in from six to eight weeks from the date of infection; it is widely diffused over the trunk, and the original dull rose-colour soon fades, leaving brownish stains, which in time disappear. It is usually followed by a _papular eruption_, the individual papules being raised above the surface of the skin, smooth or scaly, and as they are due to infiltration of the skin they are more persistent than the roseoles. They vary in size and distribution, being sometimes small, hard, polished, and closely aggregated like lichen, sometimes as large as a shilling-piece, with an accumulation of scales on the surface like that seen in psoriasis. The co-existence of scaly papules and faded roseoles is very suggestive of syphilis.
Other types of eruption are less common, and are met with from the third month onwards. A _pustular_ eruption, not unlike that of acne, is sometimes a prominent feature, but is not characteristic of syphilis unless it affects the scalp and forehead and is associated with the remains of the papular eruption. The term _ecthyma_ is applied when the pustules are of large size, and, after breaking on the surface, give rise to superficial ulcers; the discharge from the ulcer often dries up and forms a scab or crust which is continually added to from below as the ulcer extends in area and depth. The term _rupia_ is applied when the crusts are prominent, dark in colour, and conical in shape, roughly resembling the shell of a limpet. If the crust is detached, a sharply defined ulcer is exposed, and when this heals it leaves a scar which is usually circular, thin, white, shining like satin, and the surrounding skin is darkly pigmented; in the case of deep ulcers, the scar is depressed and adherent (Fig. 39).
[Illustration: FIG. 39.--Syphilitic Rupia, showing the limpet-shaped crusts or scabs.]
In the later stages there may occur a form of creeping or _spreading ulceration of the skin_ of the face, groin, or scrotum, healing at one edge and spreading at another like tuberculous lupus, but distinguished from this by its more rapid progress and by the pigmentation of the scar.
_Condylomata_ are more characteristic of syphilis than any other type of skin lesion. They are papules occurring on those parts of the body where the skin is habitually moist, and especially where two skin surfaces are in contact. They are chiefly met with on the external genitals, especially in women, around the anus, beneath large pendulous mammae, between the toes, and at the angles of the mouth, and in these situations their development is greatly favoured by neglect of cleanliness. They present the appearance of well-defined circular or ovoid areas in which the skin is thickened and raised above the surface; they are covered with a white sodden epidermis, and furnish a scanty but very infective discharge. Under the influence of irritation and want of rest, as at the anus or at the angle of the mouth, they are apt to become fissured and superficially ulcerated, and the discharge then becomes abundant and may crust on the surface, forming yellow scabs. At the angle of the mouth the condylomatous patches may spread to the cheek, and when they ulcerate may leave fissure-like scars radiating from the mouth--an appearance best seen in inherited syphilis (Fig. 44).
_The Appendages of the Skin._--The _hair_ loses its gloss, becomes dry and brittle, and readily falls out, either as an exaggeration of the normal shedding of the hair, or in scattered areas over the scalp (_syphilitic alopoecia_). The hair is not re-formed in the scars which result from ulcerated lesions of the scalp. The _nail-folds_ occasionally present a pustular eruption and superficial ulceration, to which the name _syphilitic onychia_ has been applied; more commonly the nails become brittle and ragged, and they may even be shed.
_The Mucous Membranes_, and especially those of the _mouth_ and _throat_, suffer from lesions similar to those met with on the skin. On a mucous surface the papular eruption assumes the form of _mucous patches_, which are areas with a congested base covered with a thin white film of sodden epithelium like wet tissue-paper. They are best seen on the inner aspect of the cheeks, the soft palate, uvula, pillars of the fauces, and tonsils. In addition to mucous patches, there may be a number of small, _superficial, kidney-shaped ulcers_, especially along the margins of the tongue and on the tonsils. In the absence of mucous patches and ulcers, the sore throat may be characterised by a bluish tinge of the inflamed mucous membrane and a thin film of shed epithelium on the surface. Sometimes there is an elongated sinuous film which has been likened to the track of a snail. In the _larynx_ the presence of congestion, oedema, and mucous patches may be the cause of persistent hoarseness. The _tongue_ often presents a combination of lesions, including ulcers, patches where the papillae are absent, fissures, and raised white papules resembling warts, especially towards the centre of the dorsum. These lesions are specially apt to occur in those who smoke, drink undiluted alcohol or spirits, or eat hot condiments to excess, or who have irregular, sharp-cornered teeth. At a later period, and in those who are broken down in health from intemperance or other cause, the sore throat may take the form of rapidly spreading, penetrating ulcers in the soft palate and pillars of the fauces, which may lead to extensive destruction of tissue, with subsequent scars and deformity highly characteristic of previous syphilis.
In the _Bones_, lesions occur which assume the clinical features of an evanescent periostitis, the patient complaining of nocturnal pains over the frontal bone, sternum, tibiae, and ulnae, and localised tenderness on tapping over these bones.
In the _Joints_, a serous synovitis or hydrops may occur, chiefly in the knee, on one or on both sides.
_The Affections of the Eyes_, although fortunately rare, are of great importance because of the serious results which may follow if they are not recognised and treated. _Iritis_ is the commonest of these, and may occur in one or in both eyes, one after the other, from three to eight months after infection. The patient complains of impairment of sight and of frontal or supraorbital pain. The eye waters and is hypersensitive, the iris is discoloured and reacts sluggishly to light, and there is a zone of ciliary congestion around the cornea. The appearance of minute white nodules or flakes of lymph at the margin of the pupil is especially characteristic of syphilitic iritis. When adhesions have formed between the iris and the structures in relation to it, the pupil dilates irregularly under atropin. Although complete recovery is to be expected under early and energetic treatment, if neglected, _iritis_ may result in occlusion of the pupil and permanent impairment or loss of sight.
The other lesions of the eye are much rarer, and can only be discovered on ophthalmoscopic examination.
The virus of syphilis exerts a special influence upon the _Blood Vessels_, exciting a proliferation of the endothelial lining which results in narrowing of their lumen, _endarteritis_, and a perivascular infiltration in the form of accumulations of plasma cells around the vessels and in the lymphatics that accompany them.
In the _Brain_, in the later periods of secondary and in tertiary syphilis, changes occur as a result of the narrowing of the lumen of the arteries, or of their complete obliteration by thrombosis. By interfering with the nutrition of those parts of the brain supplied by the affected arteries, these lesions give rise to clinical features of which severe headache and paralysis are the most prominent.
Affections of the _Spinal Cord_ are extremely rare, but paraplegia from myelitis has been observed.
Lastly, attention must be directed to the remarkable variations observed in different patients. Sometimes the virulent character of the disease can only be accounted for by an idiosyncrasy of the patient. Constitutional symptoms, particularly pyrexia and anaemia, are most often met with in young women. Patients over forty years of age have greater difficulty in overcoming the infection than younger adults. Malarial and other infections, and the conditions attending life in tropical countries, from the debility which they cause, tend to aggravate and prolong the disease, which then assumes the characters of what has been called _malignant syphilis_. All chronic ailments have a similar influence, and alcoholic intemperance is universally regarded as a serious aggravating factor.
_Diagnosis of Secondary Syphilis._--A routine examination should be made of the parts of the body which are most often affected in this disease--the scalp, mouth, throat, posterior cervical glands, and the trunk, the patient being stripped and examined by daylight. Among the _diagnostic features of the skin affections_ the following may be mentioned: They are frequently, and sometimes to a marked degree, symmetrical; more than one type of eruption--papules and pustules, for example--are present at the same time; there is little itching; they are at first a dull-red colour, but later present a brown pigmentation which has been likened to the colour of raw ham; they exhibit a predilection for those parts of the forehead and neck which are close to the roots of the hair; they tend to pass off spontaneously; and they disappear rapidly under treatment.
- Serum Diagnosis--Wassermann Reaction.#--Wassermann found that if an
extract of syphilitic liver rich in spirochaetes is mixed with the serum from a syphilitic patient, a large amount of complement is fixed. The application of the test is highly complicated and can only be carried out by an expert pathologist. For the purpose he is supplied with from 5 c.c. to 10 c.c. of the patient's blood, withdrawn under aseptic conditions from the median basilic vein by means of a serum syringe, and transferred to a clean and dry glass tube. There is abundant evidence that the Wassermann test is a reliable means of establishing a diagnosis of syphilis.
A definitely positive reaction can usually be obtained between the fifteenth and thirtieth day after the appearance of the primary lesion, and as time goes on it becomes more marked. During the secondary period the reaction is practically always positive. In the tertiary stage also it is positive except in so far as it is modified by the results of treatment. In para-syphilitic lesions such as general paralysis and tabes a positive reaction is almost always present. In inherited syphilis the reaction is positive in every case. A positive reaction may be present in other diseases, for example, frambesia, trypanosomiasis, and leprosy.
As the presence of the reaction is an evidence of the activity of the spirochaetes, repeated applications of the test furnish a valuable means of estimating the efficacy of treatment. The object aimed at is to change a persistently positive reaction to a permanently negative one.
- Treatment of Syphilis.#--In the treatment of syphilis the two main
objects are to maintain the general health at the highest possible standard, and to introduce into the system therapeutic agents which will inhibit or destroy the invading parasite.
The second of these objects has been achieved by the researches of Ehrlich, who, in conjunction with his pupil, Hata, has built up a compound, the dihydrochloride of dioxydiamido-arseno-benzol, popularly known as salvarsan or "606." Other preparations, such as kharsivan, arseno-billon, and diarsenol, are chemically equivalent to salvarsan, containing from 27 to 31 per cent. of arsenic, and are equally efficient. The full dose is 0.6 grm. All these members of the "606" group form an acid solution when dissolved in water, and must be rendered alkaline before being injected. As subcutaneous and intra-muscular injections cause considerable pain, and may cause sloughing of the tissues, "606" preparations must be injected intravenously. Ehrlich has devised a preparation--neo-salvarsan, or "914," which is more easily prepared and forms a neutral solution. It contains from 18 to 20 per cent. of arsenic. Neo-kharsivan, novo-arseno-billon, and neo-diarsenol belong to the "914" group, the full dosage of which is 0.9 grm. As subcutaneous and intra-muscular injections of the "914" group are not painful, and even more efficient than intravenous injections, the administration is simpler.
Galyl, luargol, and other preparations act in the same way as the "606" and "914" groups.
The "606" preparations may be introduced into the veins by injection or by means of an apparatus which allows the solution to flow in by gravity. The left median basilic vein is selected, and a platino-iridium needle with a short point and a bore larger than that of the ordinary hypodermic syringe is used. The needle is passed for a few millimetres along the vein, and the solution is then slowly introduced; before withdrawing the needle some saline is run in to diminish the risk of thrombosis.
The "914" preparations may be injected either into the subcutaneous tissue of the buttock or into the substance of the gluteus muscle. The part is then massaged for a few minutes, and the massage is repeated daily for a few days.
No hard-and-fast rules can be laid down as to what constitutes a complete course of treatment. Harrison recommends as a _minimum_ course of one of the "914" preparations in _early primary cases_ an initial dose of 0.45 grm. given intra-muscularly or into the deep subcutaneous tissue; the same dose a week later; 0.6 grm. the following week; then miss a week and give 9.6 grms. on two successive weeks; then miss two weeks and give 0.6 grm. on two more successive weeks.
When a _positive Wassermann reaction_ is present before treatment is commenced, the above course is prolonged as follows: for three weeks is given a course of potassium iodide, after which four more weekly injections of 0.6 grm. of "914" are given.
With each injection of "914" after the first, throughout the whole course 1 grain of mercury is injected intra-muscularly.
In the course of a few hours, there is usually some indisposition, with a feeling of chilliness and slight rise of temperature; these symptoms pass off within twenty-four hours, and in a few days there is a decided improvement of health. Three or four days after an intra-muscular injection there may be pain and stiffness in the gluteal region.
These preparations are the most efficient therapeutic agents that have yet been employed in the treatment of syphilis.
The manifestations of the disease disappear with remarkable rapidity. Observations show that the spirochaetes lose their capacity for movement within an hour or two of the administration, and usually disappear altogether in from twenty-four to thirty-six hours. Wassermann's reaction usually yields a negative result in from three weeks to two months, but later may again become positive. Subsequent doses of the arsenical preparation are therefore usually indicated, and should be given in from 7 to 21 days according to the dose.
When syphilis occurs in a _pregnant woman_, she should be given in the early months an ordinary course of "914," followed by 10-grain doses of potassium iodide twice daily. The injections may be repeated two months later, and during the remainder of the pregnancy 2-grain mercury pills are given twice daily (A. Campbell). The presence of albumen in the urine contra-indicates arsenical treatment.
It need scarcely be pointed out that the use of powerful drugs like "606" and "914" is not free from risk; it may be mentioned that each dose contains nearly three grains of arsenic. Before the administration the patient must be overhauled; its administration is contra-indicated in the presence of disease of the heart and blood vessels, especially a combination of syphilitic aortitis and sclerosis of the coronary arteries, with degeneration of the heart muscle; in affections of the central nervous system, especially advanced paralysis, and in such disturbances of metabolism as are associated with diabetes and Bright's disease. Its use is not contra-indicated in any lesion of active syphilis.
The administration is controlled by the systematic examination of the urine for arsenic.
_The Administration of Mercury._--The success of the arsenical preparations has diminished the importance of mercury in the treatment of syphilis, but it is still used to supplement the effect of the injections. The amount of mercury to be given in any case must be proportioned to the idiosyncrasies of the patient, and it is advisable, before commencing the treatment, to test his urine and record his body-weight. The small amount of mercury given at the outset is gradually increased. If the body-weight falls, or if the gums become sore and the breath foul, the mercury should be stopped for a time. If salivation occurs, the drinking of hot water and the taking of hot baths should be insisted upon, and half-dram doses of the alkaline sulphates prescribed.
_Methods of Administering Mercury._--(1) _By the Mouth._--This was for long the most popular method in this country, the preparation usually employed being grey powder, in pills or tablets, each of which contains one grain of the powder. Three of these are given daily in the first instance, and the daily dose is increased to five or even seven grains till the standard for the individual patient is arrived at. As the grey powder alone sometimes causes irritation of the bowels, it should be combined with iron, as in the following formula: Hydrarg. c. cret. gr. 1; ferri sulph. exsiccat. gr. 1 or 2.
(2) _By Inunction._--Inunction consists in rubbing into the pores of the skin an ointment composed of equal parts of 20 per cent. oleate of mercury and lanolin. Every night after a hot bath, a dram of the ointment (made up by the chemist in paper packets) is rubbed for fifteen minutes into the skin where it is soft and comparatively free from hairs. When the patient has been brought under the influence of the mercury, inunction may be replaced by one of the other methods, of administering the drug.
(3) _By Intra-muscular Injection._--This consists in introducing the drug by means of a hypodermic syringe into the substance of the gluteal muscles. The syringe is made of glass, and has a solid glass piston; the needle of platino-iridium should be 5 cm. long and of a larger calibre than the ordinary hypodermic needle. The preparation usually employed consists of: metallic mercury or calomel 1 dram, lanolin and olive oil each 2 drams; it must be warmed to allow of its passage through the needle. Five minims--containing one grain of metallic mercury--represent a dose, and this is injected into the muscles above and behind the great trochanter once a week. The contents of the syringe are slowly expressed, and, after withdrawing the needle, gentle massage of the buttock should be employed. Four courses each of ten injections are given the first year, three courses of the same number during the second and third years, and two courses during the fourth year (Lambkin).
_The General Health._--The patient must lead a regular life and cultivate the fresh-air habit, which is as beneficial in syphilis as in tuberculosis. Anaemia, malaria, and other sources of debility must receive appropriate treatment. The diet should be simple and easily digested, and should include a full supply of milk. Alcohol is prohibited. The excretory organs are encouraged to act by the liberal drinking of hot water between meals, say five or six tumblerfuls in the twenty-four hours. The functions of the skin are further aided by frequent hot baths, and by the wearing of warm underclothing. While the patient should avoid exposure to cold, and taxing his energies by undue exertion, he should be advised to take exercise in the open air. On account of the liability to lesions of the mouth and throat, he should use tobacco in moderation, his teeth should be thoroughly overhauled by the dentist, and he should brush them after every meal, using an antiseptic tooth powder or wash. The mouth and throat should be rinsed out night and morning with a solution of chlorate of potash and alum, or with peroxide of hydrogen.
_Treatment of the Local Manifestations._--_The skin lesions_ are treated on the same lines as similar eruptions of other origin. As local applications, preparations of mercury are usually selected, notably the ointments of the red oxide of mercury, ammoniated mercury, or oleate of mercury (5 per cent.), or the mercurial plaster introduced by Unna. In the treatment of condylomata the greatest attention must be paid to cleanliness and dryness. After washing and drying the affected patches, they are dusted with a powder consisting of equal parts of calomel and carbonate of zinc; and apposed skin surfaces, such as the nates or labia, are separated by sublimate wool. In the ulcers of later secondary syphilis, crusts are got rid of in the first instance by means of a boracic poultice, after which a piece of lint or gauze cut to the size of the ulcer and soaked in black wash is applied and covered with oil-silk. If the ulcer tends to spread in area or in depth, it should be scraped with a sharp spoon, and painted over with acid nitrate of mercury, or a local hyperaemia may be induced by Klapp's suction apparatus.
_In lesions of the mouth and throat_, the teeth should be attended to; the best local application is a solution of chromic acid--10 grains to the ounce--painted on with a brush once daily. If this fails, the lesions may be dusted with calomel the last thing at night. For deep ulcers of the throat the patient should gargle frequently with chlorine water or with perchloride of mercury (1 in 2000); if the ulcer continues to spread it should be painted with acid nitrate of mercury.
In the treatment of _iritis_ the eyes are shaded from the light and completely rested, and the pupil is well dilated by atropin to prevent adhesions. If there is much pain, a blister may be applied to the temple.
_The Relations of Syphilis to Marriage._--Before the introduction of the Ehrlich-Hata treatment no patient was allowed to marry until three years had elapsed after the disappearance of the last manifestation. While marriage might be entered upon under these conditions without risk of the husband infecting the wife, the possibility of his conveying the disease to the offspring cannot be absolutely excluded. It is recommended, as a precautionary measure, to give a further mercurial course of two or three months' duration before marriage, and an intravenous injection of an arsenical preparation.
- Intermediate Stage.#--After the dying away of the secondary
manifestations and before the appearance of tertiary lesions, the patient may present certain symptoms which Hutchinson called _reminders_. These usually consist of relapses of certain of the affections of the skin, mouth, or throat, already described. In the skin, they may assume the form of peeling patches in the palms, or may appear as spreading and confluent circles of a scaly papular eruption, which if neglected may lead to the formation of fissures and superficial ulcers. Less frequently there is a relapse of the eye affections, or of paralytic symptoms from disease of the cerebral arteries.
- Tertiary Syphilis.#--While the manifestations of primary and secondary
syphilis are common, those of the tertiary period are by comparison rare, and are observed chiefly in those who have either neglected treatment or who have had their powers of resistance lowered by privation, by alcoholic indulgence, or by tropical disease.
It is to be borne in mind that in a certain proportion of men and in a larger proportion of women, the patient has no knowledge of having suffered from syphilis. Certain slight but important signs may give the clue in a number of cases, such as irregularity of the pupils or failure to react to light, abnormality of the reflexes, and the discovery of patches of leucoplakia on the tongue, cheek, or palate.
The _general character of tertiary manifestations_ may be stated as follows: They attack by preference the tissues derived from the mesoblastic layer of the embryo--the cellular tissue, bones, muscles, and viscera. They are often localised to one particular tissue or organ, such, for example, as the subcutaneous cellular tissue, the bones, or the liver, and they are rarely symmetrical. They are usually aggressive and persistent, with little tendency to natural cure, and they may be dangerous to life, because of the destructive changes produced in such organs as the brain or the larynx. They are remarkably amenable to treatment if instituted before the stage which is attended with destruction of tissue is reached. Early tertiary lesions may be infective, and the disease may be transmitted by the discharges from them; but the later the lesions the less is the risk of their containing an infective virus.
The most prominent feature of tertiary syphilis consists in the formation of granulation tissue, and this takes place on a scale considerably larger than that observed in lesions of the secondary period. The granulation tissue frequently forms a definite swelling or tumour-like mass (syphiloma), which, from its peculiar elastic consistence, is known as a _gumma_. In its early stages a gumma is a firm, semi-translucent greyish or greyish-red mass of tissue; later it becomes opaque, yellow, and caseous, with a tendency to soften and liquefy. The gumma does harm by displacing and replacing the normal tissue elements of the part affected, and by involving these in the degenerative changes, of the nature of caseation and necrosis, which produce the destructive lesions of the skin, mucous membranes, and internal organs. This is true not only of the circumscribed gumma, but of the condition known as _gummatous infiltration_ or _syphilitic cirrhosis_, in which the granulation tissue is diffused throughout the connective-tissue framework of such organs as the tongue or liver. Both the gummatous lesions and the fibrosis of tertiary syphilis are directly excited by the spirochaetes.
The life-history of an untreated gumma varies with its environment. When protected from injury and irritation in the substance of an internal organ such as the liver, it may become encapsulated by fibrous tissue, and persist in this condition for an indefinite period, or it may be absorbed and leave in its place a fibrous cicatrix. In the interior of a long bone it may replace the rigid framework of the shaft to such an extent as to lead to pathological fracture. If it is near the surface of the body--as, for example, in the subcutaneous or submucous cellular tissue, or in the periosteum of a superficial bone, such as the palate, the skull, or the tibia--the tissue of which it is composed is apt to undergo necrosis, in which the overlying skin or mucous membrane frequently participates, the result being an ulcer--the tertiary syphilitic ulcer (Figs. 40 and 41).
_Tertiary Lesions of the Skin and Subcutaneous Cellular Tissue._--The clinical features of a _subcutaneous gumma_ are those of an indolent, painless, elastic swelling, varying in size from a pea to an almond or walnut. After a variable period it usually softens in the centre, the skin over it becomes livid and dusky, and finally separates as a slough, exposing the tissue of the gumma, which sometimes appears as a mucoid, yellowish, honey-like substance, more frequently as a sodden, caseated tissue resembling wash-leather. The caseated tissue of a gumma differs from that of a tuberculous lesion in being tough and firm, of a buff colour like wash-leather, or whitish, like boiled fish. The degenerated tissue separates slowly and gradually, and in untreated cases may be visible for weeks in the floor of the ulcer.
[Illustration: FIG. 40.--Ulcerating Gumma of Lips.
(From a photograph lent by Dr. Stopford Taylor and Dr. R. W. Mackenna.)]
_The tertiary ulcer_ may be situated anywhere, but is most frequently met with on the leg, especially in the region of the knee (Fig. 42) and over the calf. There may be one or more ulcers, and also scars of antecedent ulcers. The edges are sharply cut, as if punched out; the margins are rounded in outline, firm, and congested; the base is occupied by gummatous tissue, or, if this has already separated and sloughed out, by unhealthy granulations and a thick purulent discharge. When the ulcer has healed it leaves a scar which is depressed, and if over a bone, is adherent to it. The features of the tertiary ulcer, however, are not always so characteristic as the above description would imply. It is to be diagnosed from the "leg ulcer," which occurs almost exclusively on the lower third of the leg; from Bazin's disease (p. 74); from the ulcers that result from certain forms of malignant disease, such as rodent cancer, and from those met with in chronic glanders.
_Gummatous Infiltration of the Skin_ ("Syphilitic Lupus").--This is a lesion, met with chiefly on the face and in the region of the external genitals, in which the skin becomes infiltrated with granulation tissue so that it is thickened, raised above the surface, and of a brownish-red colour. It appears as isolated nodules, which may fuse together; the epidermis becomes scaly and is shed, giving rise to superficial ulcers which are usually covered by crusted discharge. The disease tends to spread, creeping over the skin with a serpiginous, crescentic, or horse-shoe margin, while the central portion may heal and leave a scar. From the fact of its healing in the centre while it spreads at the margin, it may resemble tuberculous disease of the skin. It can usually be differentiated by observing that the infiltration is on a larger scale; the progress is much more rapid, involving in the course of months an area which in the case of tuberculosis would require as many years; the scars are sounder and are less liable to break down again; and the disease rapidly yields to anti-syphilitic treatment.
[Illustration: FIG. 41.--Ulceration of nineteen year's duration in a woman aet. 24, the subject of inherited syphilis, showing active ulceration, cicatricial contraction, and sabre-blade deformity of tibiae.]
_Tertiary lesions of mucous membrane and of the submucous cellular tissue_ are met with chiefly in the tongue, nose, throat, larynx, and rectum. They originate as gummata or as gummatous infiltrations, which are liable to break down and lead to the formation of ulcers which may prove locally destructive, and, in such situations as the larynx, even dangerous to life. In the tongue the tertiary ulcer may prove the starting-point of cancer; and in the larynx or rectum the healing of the ulcer may lead to cicatricial stenosis.
Tertiary lesions of the _bones and joints_, of the _muscles_, and of the _internal organs_, will be described under these heads. The part played by syphilis in the production of disease of arteries and of aneurysm will be referred to along with diseases of blood vessels.
[Illustration: FIG. 42.--Tertiary Syphilitic Ulceration in region of Knee and on both Thumbs of woman aet. 37.]
_Treatment._--The most valuable drugs for the treatment of the manifestations of the tertiary period are the arsenical preparations and the iodides of sodium and potassium. On account of their depressing effects, the latter are frequently prescribed along with carbonate of ammonium. The dose is usually a matter of experiment in each individual case; 5 grains three times a day may suffice, or it may be necessary to increase each dose to 20 or 25 grains. The symptoms of iodism which may follow from the smaller doses usually disappear on giving a larger amount of the drug. It should be taken after meals, with abundant water or other fluid, especially if given in tablet form. It is advisable to continue the iodides for from one to three months after the lesions for which they are given have cleared up. If the potassium salt is not tolerated, it may be replaced by the ammonium or sodium iodide.
_Local Treatment._--The absorption of a subcutaneous gumma is often hastened by the application of a fly-blister. When a gumma has broken on the surface and caused an ulcer, this is treated on general principles, with a preference, however, for applications containing mercury or iodine, or both. If a wet dressing is required to cleanse the ulcer, black wash may be used; if a powder to promote dryness, one containing iodoform; if an ointment is indicated, the choice lies between the red oxide of mercury or the dilute nitrate of mercury ointment, and one consisting of equal parts of lanolin and vaselin with 2 per cent. of iodine. Deep ulcers, and obstinate lesions of the bones, larynx, and other parts may be treated by excision or scraping with the sharp spoon.
- Second Attacks of Syphilis.#--Instances of re-infection of syphilis
have been recorded with greater frequency since the more general introduction of arsenical treatment. A remarkable feature in such cases is the shortness of the interval between the original infection and the alleged re-infection; in a recent series of twenty-eight cases, this interval was less than a year. Another feature of interest is that when patients in the tertiary stage of syphilis are inoculated with the virus from lesions from these in the primary and secondary stage lesions of the tertiary type are produced.
Reference may be made to the #relapsing false indurated chancre#, described by Hutchinson and by Fournier, as it may be the source of difficulty in diagnosis. A patient who has had an infecting chancre one or more years before, may present a slightly raised induration on the penis at or close to the site of his original sore. This relapsed induration is often so like that of a primary chancre that it is impossible to distinguish between them, except by the history. If there has been a recent exposure to venereal infection, it is liable to be regarded as the primary lesion of a second attack of syphilis, but the further progress shows that neither bullet-buboes nor secondary manifestations develop. These facts, together with the disappearance of the induration under treatment, make it very likely that the lesion is really gummatous in character.
One of the most striking features of syphilis is that it may be transmitted from infected parents to their offspring, the children exhibiting the manifestations that characterise the acquired form of the disease.
The more recent the syphilis in the parent, the greater is the risk of the disease being communicated to the offspring; so that if either parent suffers from secondary syphilis the infection is almost inevitably transmitted.
While it is certain that either parent may be responsible for transmitting the disease to the next generation, the method of transmission is not known. In the case of a syphilitic mother it is most probable that the infection is conveyed to the foetus by the placental circulation. In the case of a syphilitic father, it is commonly believed that the infection is conveyed to the ovum through the seminal fluid at the moment of conception. If a series of children, one after the other, suffer from inherited syphilis, it is almost invariably the case that the mother has been infected.
In contrast to the acquired form, inherited syphilis is remarkable for the absence of any primary stage, the infection being a general one from the outset. The spirochaete is demonstrated in incredible numbers in the liver, spleen, lung, and other organs, and in the nasal secretion, and, from any of these, successful inoculations in monkeys can readily be made. The manifestations differ in degree rather than in kind from those of the acquired disease; the difference is partly due to the fact that the virus is attacking developing instead of fully formed tissues.
The virus exercises an injurious influence on the foetus, which in many cases dies during the early months of intra-uterine life, so that miscarriage results, and this may take place in repeated pregnancies, the date at which the miscarriage occurs becoming later as the virus in the mother becomes attenuated. Eventually a child is carried to full term, and it may be still-born, or, if born alive, may suffer from syphilitic manifestations. It is difficult to explain such vagaries of syphilitic inheritance as the infection of one twin and the escape of the other.
_Clinical Features._--We are not here concerned with the severe forms of the disease which prove fatal, but with the milder forms in which the infant is apparently healthy when born, but after from two to six weeks begins to show evidence of the syphilitic taint.
The usual phenomena are that the child ceases to thrive, becomes thin and sallow, and suffers from eruptions on the skin and mucous membranes. There is frequently a condition known as _snuffles_, in which the nasal passages are obstructed by an accumulation of thin muco-purulent discharge which causes the breathing to be noisy. It usually begins within a month after birth and before the eruptions on the skin appear. When long continued it is liable to interfere with the development of the nasal bones, so that when the child grows up there results a condition known as the "saddle-nose" deformity (Figs. 43 and 44).
[Illustration: FIG. 43.--Facies of Inherited Syphilis.
(From Dr. Byrom Bramwell's _Atlas of Clinical Medicine_.)]
_Affections of the Skin._--Although all types of skin affection are met with in the inherited disease, the most important is a _papular_ eruption, the papules being of large size, with a smooth shining top and of a reddish-brown colour. It affects chiefly the buttocks and thighs, the genitals, and other parts which are constantly moist. It is necessary to distinguish this specific eruption from a form of eczema which occurs in these situations in non-syphilitic children, the points that characterise the syphilitic condition being the infiltration of the skin and the coppery colour of the eruption. At the anus the papules acquire the characters of _condylomata_, also at the angles of the mouth, where they often ulcerate and leave radiating scars.
_Affections of the Mucous Membranes._--The inflammation of the nasal mucous membrane that causes snuffles has already been referred to. There may be mucous patches in the mouth, or a stomatitis which is of importance, because it results in interference with the development of the permanent teeth. The mucous membrane of the larynx may be the seat of mucous patches or of catarrh, and as a result the child's cry is hoarse.
_Affections of the Bones._--Swellings at the ends of the long bones, due to inflammation at the epiphysial junctions, are most often observed at the upper end of the humerus and in the bones in the region of the elbow. Partial displacement and mobility at the ossifying junction may be observed. The infant cries when the part is touched; and as it does not move the limb voluntarily, the condition is spoken of as _the pseudo-paralysis of syphilis_. Recovery takes place under anti-syphilitic treatment and immobilisation of the limb.
Diffuse thickening of the shafts of the long bones, due to a deposit of new bone by the periosteum, is sometimes met with.
[Illustration: FIG. 44.--Facies of Inherited Syphilis.]
The conditions of the skull known as Parrot's nodes or bosses, and craniotabes, were formerly believed to be characteristic of inherited syphilis, but they are now known to occur, particularly in rickety children, from other causes. The _bosses_ result from the heaping up of new spongy bone beneath the pericranium, and they may be grouped symmetrically around the anterior fontanelle, or may extend along either side of the sagittal suture, which appears as a deep groove--the "natiform skull." The bosses disappear in time, but the skull may remain permanently altered in shape, the frontal and parietal eminences appearing unduly prominent. The term _craniotabes_ is applied when the bone becomes thin and soft, reverting to its original membranous condition, so that the affected areas dimple under the finger like parchment or thin cardboard; its localisation in the posterior parts of the skull suggests that the disappearance of the osseous tissue is influenced by the pressure of the head on the pillow. Craniotabes is recovered from as the child improves in health.
Between the ages of three and six months, certain other phenomena may be met with, such as _effusion into the joints_, especially the knees; _iritis_, in one or in both eyes, and enlargement of the spleen and liver.
In the majority of cases the child recovers from these early manifestations, especially when efficiently treated, and may enjoy an indefinite period of good health. On the other hand, when it attains the age of from two to four years, it may begin to manifest lesions which correspond to those of the tertiary period of acquired syphilis.
- Later Lesions.#--In the skin and subcutaneous tissue, the later
manifestations may take the form of localised gummata, which tend to break down and form ulcers, on the leg for example, or of a spreading gummatous infiltration which is also liable to ulcerate, leaving disfiguring scars, especially on the face. The palate and fauces may be destroyed by ulceration. In the nose, especially when the ulcerative process is associated with a putrid discharge--ozaena--the destruction of tissue may be considerable and result in unsightly deformity. The entire palatal portions of the upper jaws, the vomer, turbinate, and other bones bounding the nasal and oral cavities, may disappear, so that on looking into the mouth the base of the skull is readily seen. Gummatous disease is frequently observed also in the flat bones of the skull, in the bones of the hand, as syphilitic dactylitis, and in the bones of the forearm and leg. When the tibia is affected the disease is frequently bilateral, and may assume the form of gummatous ulcers and sinuses. In later years the tibia may present alterations in shape resulting from antecedent gummatous disease--for example, nodular thickenings of the shaft, flattening of the crest, or a more uniform increase in thickness and length of the shaft of the bone, which, when it is curved in addition, is described as the "sabre-blade" deformity. Among lesions of the viscera, mention should be made of gumma of the testis, which causes the organ to become enlarged, uneven, and indurated. This has even been observed in infants a few months old.
Occasionally a syphilitic child suffers from a succession of these gummatous lesions with resulting ill-health, and, it may be, waxy disease of the internal organs; on the other hand, it may recover and present no further manifestations of the inherited taint.
_Affections of the Eyes._--At or near puberty there is frequently observed an affection of the eyes, known as _chronic interstitial keratitis_, the relationship of which to inherited syphilis was first established by Hutchinson. It occurs between the ages of six and sixteen years, and usually affects one eye before the other. It commences as a diffuse haziness or steaminess near the centre of the cornea, and as it spreads the entire cornea assumes the appearance of ground glass. The chief complaint is of dimness of sight, which may almost amount to blindness, but there is little pain or photophobia; a certain amount of conjunctival and ciliary congestion is usually present, and there may be _iritis_ in addition. The cornea, or parts of it, may become of a deep pink or salmon colour from the formation in it of new blood vessels. The affection may last for from eighteen months to two years. Complete recovery usually takes place, but slight opacities, especially in the site of former salmon patches, may persist, and the disease occasionally relapses. _Choroiditis_ and _retinitis_ may also occur, and leave permanent changes easily recognised on examination with the ophthalmoscope.
Among the rarer and more serious lesions of the inherited disease may be mentioned gummatous disease in the _larynx and trachea_, attended with ulceration and resulting in stenosis; and lesions of the _nervous system_ which may result in convulsions, paralysis, or dementia.
In a limited number of cases, about the period of puberty there may develop _deafness_, which is usually bilateral and may become absolute.
_Changes in the Permanent Teeth._--These affect specially the upper central incisors, which are dwarfed and stand somewhat apart in the gum, with their free edges converging towards one another. They are tapering or peg-shaped, and present at their cutting margin a deep semilunar notch. These appearances are commonly associated with the name of Hutchinson, who first described them. Affecting as they do the permanent teeth, they are not available for diagnosis until the child is over eight years of age. Henry Moon drew attention to a change in the first molars; these are reduced in size and dome-shaped through dwarfing of the central tubercle of each cusp.
- Diagnosis of Inherited Syphilis.#--When there is a typical eruption on
the buttocks and snuffles there is no difficulty in recognising the disease. When, however, the rash is scanty or is obscured by co-existing eczema, most reliance should be placed on the distribution of the eruption, on the brown stains which are left after it has passed off, on the presence of condylomata, and of fissuring and scarring at the angles of the mouth. The history of the mother relative to repeated miscarriages and still-born children may afford confirmatory evidence. In doubtful cases, the diagnosis may be aided by the Wassermann test and by noting the therapeutic effects of grey powder, which, in syphilitic infants, usually effects a marked and rapid improvement both in the symptoms and in the general health.
While a considerable number of syphilitic children grow up without showing any trace of their syphilitic inheritance, the majority retain throughout life one or more of the following characteristics, which may therefore be described as _permanent signs of the inherited disease_: Dwarfing of stature from interference with growth at the epiphysial junctions; the forehead low and vertical, and the parietal and frontal eminences unduly prominent; the bridge of the nose sunken and rounded; radiating scars at the angles of the mouth; perforation or destruction of the hard palate; Hutchinson's teeth; opacities of the cornea from antecedent keratitis; alterations in the fundus oculi from choroiditis; deafness; depressed scars or nodes on the bones from previous gummata; "sabre-blade" or other deformity of the tibiae.
- The Contagiousness of Inherited Syphilis.#--In 1837, Colles of Dublin
stated his belief that, while a syphilitic infant may convey the disease to a healthy wet nurse, it is incapable of infecting its own mother if nursed by her, even although she may never have shown symptoms of the disease. This doctrine, which is known as _Colles' law_, is generally accepted in spite of the alleged occurrence of occasional exceptions. The older the child, the less risk there is of its communicating the disease to others, until eventually the tendency dies out altogether, as it does in the tertiary period of acquired syphilis. It should be added, however, that the contagiousness of inherited syphilis is denied by some observers, who affirm that, when syphilitic infants prove infective, the disease has been really acquired at or soon after birth.
There is general agreement that the subjects of inherited syphilis cannot transmit the disease by inheritance to their offspring, and that, although they very rarely acquire the disease _de novo_, it is possible for them to do so.
- Prognosis of Inherited Syphilis.#--Although inherited syphilis is
responsible for a large but apparently diminishing mortality in infancy, the subjects of this disease may grow up to be as strong and healthy as their neighbours. Hutchinson insisted on the fact that there is little bad health in the general community that can be attributed to inherited syphilis.
- Treatment.#--Arsenical injections are as beneficial in the inherited as
in the acquired disease. An infant the subject of inherited syphilis should, if possible, be nursed by its mother, and failing this it should be fed by hand. In infants at the breast, the drug may be given to the mother; in others, it is administered in the same manner as already described--only in smaller doses. On the first appearance of syphilitic manifestations it should be given 0.05 grm, novarsenbillon, injected into the deep subcutaneous tissues every week for six weeks, followed by one year's mercurial inunction--a piece of mercurial ointment the size of a pea being inserted under the infant's binder. In older children the dose is proportionately increased. The general health should be improved in every possible direction; considerable benefit may be derived from the use of cod-liver oil, and from preparations containing iron and calcium. Surgical interference may be required in the destructive gummatous lesions of the nose, throat, larynx, and bones, either with the object of arresting the spread of the disease, or of removing or alleviating the resulting deformities. In children suffering from keratitis, the eyes should be protected from the light by smoked or coloured glasses, and the pupils should be dilated with atropin from time to time, especially in cases complicated with iritis.
- Acquired Syphilis in Infants and Young Children.#--When syphilis is met
with in infants and young children, it is apt to be taken for granted that the disease has been inherited. It is possible, however, for them to acquire the disease--as, for example, while passing through the maternal passages during birth, through being nursed or kissed by infected women, or through the rite of circumcision. The risk of infection which formerly existed by the arm-to-arm method of vaccination has been abolished by the use of calf lymph.
The clinical features of the acquired disease in infants and young children are similar to those observed in the adult, with a tendency, however, to be more severe, probably because the disease is often late in being recognised and treated.