The objective manifestations of acquired syphilis may appear at any period in its course and, while no strict consecutive order is always observed, each lesion has its favorite period of occurrence. Thus, the chancre and associated constitutional symptoms present the primary stage; the macular, papular, vesicular and pustular eruption before to the secondary period; while the tubercular, bullous, gummatoma and ulcerative are found in the tertiary or late stage.
General Symptomatic and Diagnostic Features of the Syphilids. – The pathological processes of hyperemia and cell-infiltration alone or together with various subsequently induced changes give origin to numerous and varied forms of eruption; so much so that some one has described syphilis as an “imitator of other diseases.” The resemblance to other eruptive diseases is natural, inasmuch as the same anatomical parts are involved in all by an inflammatory process, but which in the case of syphilis (and some others) is dominated by a specific cause. The imitation therefore is hardly more than objective and is usually over-balanced by distinct differences. These characteristic differences, each alone of little diagnostic value, together form a significant clinical group, and may be studied in general contrast with the simple eruptions of other diseases.
Course and Duration – Syphilids of the active or secondary stage usually appear rapidly and attain full development in one or two weeks, although it is not uncommon for a few new lesions to show irregularity for some time afterward. Their disappearance is gradual although the papular eruption is prone to relapses for some months. On the other hand, the tertiary eruption shows little tendency to spontaneous disappearance. The eruption of the early period may be attended with pronounced systemic reaction, fever and may closely resemble an exanthem, but the apparent acuteness of the syphilid soon resolves itself into a subacute course. The behavior of these lesions may be hastened, interrupted or modified by some intercurrent disease which, however, will not affect the syphilitic diathesis sufficiently to prevent ultimate relapses. Acute febrile attacks of various origin, scabies, miliaria, furuncles, eczema, seborrhea, a scrofulous and lymphatic tendency, idiosyncrasy, age, climate, alcoholism and, in fact, any general or personal defects in hygiene, all of which influences are not peculiar or syphilis, may act to modify or aggravate the behavior of the syphilids. These influences give individuality to each case and afford a scientific basis for treatment.
The polymorphism of the early syphilids is present in the majority of cases and the lesions have a tendency to appear in crops. Thus, macules, papules, pustules and scaly lesions may coexist in all stages of evolution and decline or successively exhibit their varied phases in the transit of one form into another.
The order of evolution of the syphilids, while not absolute, is quite characteristic and the skin is usually involved from without inward or from the more superficial to the deeper parts. At an interval averaging about six weeks after the development of the characteristic primary sore, the so-called secondary eruptions begin to appear. Of these the macular is the first to occur, followed by the deeper but still superficial papular, then by the pustular and so on until the lesions present are indicative of the age of the disease. The papule is the most typical of the secondary lesions and occurs in varied forms to be described later. Although the eruptions of this period are supposed to last about two years, they are seldom constant because of the tendency to disappearance and recurrence at variable intervals. The tubercle is the typical lesion of tertiary syphilis, as the papule is of the earlier stage. The tertiary syphilids in comparison with the so-called secondary lesions are characterized as follows: they are much less constant in occurrence; when they do appear they are without order of succession; they are asymmetrical, localized, deep-seated, tend to persist and spread; cause local destruction of the tissue and leave permanent scars; and, though they may recur during the patient’s life, they are at no time contagious or inoculable.
The foregoing applies more or less accurately to the typical evolution of the syphilids, which may vary greatly in intensity, in the number and extent of the lesions and in their duration and succession. Rarely there may be an almost complete reversal of the law of syphilitic evolution and deeper lesions common to the tertiary period may antedate the superficial eruptions of the secondary period (retrogressive syphilis). In another irregular type, the usually late and deeper tubercles and gummata develop before the early and superficial macules, papules and pustules subside, or follow closely their decline and pursue an acute non-destructive course (rapid benign syphilis). Less often the rapid, violent and extensive involvement of the deeper tissues assumes a malignant destructive course (precocious malignant syphilis). Lesser irregularities in the evolution of the syphilids, such as the predominance or persistence of one kind of lesion, the moderate occurrence of the early lesions in the late or tertiary period, or lesions of the latter within the limits of the secondary stage, are not uncommon.
Distribution – In location, the syphilitic eruptions of the secondary stage resemble the exanthemata in being more or less generalized and symmetrical, but they seldom appear in such areas as the sternal, supra and infraclavicular regions, or on the dorsal surfaces of the wrists, hands and feet where other inflammatory eruptions are common. The form of the eruption determines the location to some extent. Thus, the macular syphilid is commonly found on the chest, trunk and flexor surfaces, while the papular favors the forehead, margin of the hairy scalp, neck, extremities and trunk. The pustular is often found on the hairy parts of the scalp, face and other regions well supplied with sebaceous glands and hair-follicles. Moist papules or mucous patches occur almost exclusively on the warm or moist regions of the body. The later syphilids, including the tubercular, may develop almost anywhere but the erythematous and rupial forms, like non-specific lesions of the same type, prefer the legs.
Configuration – The early eruptions exhibit little if any tendency toward special grouping or configuration but are usually round or oval and occasionally irregular. However, maculopapular lesions, especially in negroes, around the mouth, lower part of the face and neck are often distinctly annular. The later secondary outbreaks may show segmental, circinate or serpiginous tendencies, but ordinarily these manifestations are found in the later or tertiary period where this tendency is almost diagnostic.
The color and pigmentation of the syphilids are not so characteristic as generally supposed. At first the lesions are a bright pinkish-red and later they become a brownish-red, yellowish-brown or coppery tint which finally amounts to a brownish pigmentation which may eventually disappear. Rarely, pigmentation occurs independently of other lesions (pigmentary syphilid).
The scales of syphilis are thin, superficial, scant, dull white or yellowish in color and non-adherent compared with the same product of similar eruptions.
The crusts of syphilitic pustules and ulcers are quite distinctive. They are grayish, brownish or greenish-black in color, rest upon an indurated base and are easily detached; they are thicker than the crusts of simple lesions and are built up in layers from the secretions formed beneath. If of large size (ecthymatous or rupial), they may seem to almost float upon a base of liquid pus; and the conical, laminated brownish-black crusts of the rupial type, which may slowly attain a large size, are pathognomonic. The brownish-black, rough, dirty, oyster-shell-like crusts of late syphilitic ulcers are also characteristic.
Fig. 148 – Annulopapular syphilid, a common form in Negroes (courtesy of Dr. Howard Fox).
The ulcers of syphilis may be round, oval, crescentic or horseshoe in shape, due to both the enlargement and the subsequent healing which takes place more or less regularly form within outwardly. Hence, the margins are generally regular and their edges perpendicular. The floors may be grayish or present a membranous appearance, bathed with a sanious pus. The ulcer is often bordered by a reddish areola.
Fig. 149 -Syphilitic scars following a pustular syphilid.
The scars of syphilis are often diagnostic. They are distinctly round or oval in shape; at first reddish brown in color, they gradually fade from the center to the periphery until when mature there is left a white, smooth, shining, more or less depressed pliable surface, bounded by a narrow areola of brown pigmentation, which is usually persistent. Near the joints syphilitic scars may be traversed by fibrous bands, more often they are smooth or only minutely perforated at the follicular opening.
Subjective Symptoms – A marked negative feature of the syphilids is the absence of pain or itching during their entire course. In fact, the patients may be unaware of their existence until they are accidentally seen. However, the lesions may rarely be sensitive and painful if the process is unusually acute or they are subjected to external irritation. Itching may be present in the warm or moist regions of the skin, especially if an eczematous condition coexists.
Concomitant Symptoms – In the primary stage a chancre may be seen or its mark or scar may be noted and a train of constitutional disturbances, varying in intensity, may appear toward the end of the primary or the onset of the secondary period. These include general adenopathy, sore throat with mucous patches or superficial ulcers in the mouth and pharynx, iritis, headache, bone pains, moderate fever reaching 100* to 101* F in the evening, lassitude, general anemia, alopecia, onychia and paronychia. It is rare, however, that all of these symptoms are observed in one case. Frequently, only one or two will be noted. In the third period, accompanying symptoms may be wholly absent although scars, the effects of iritis, together with bone pains, alopecia, leucoplakin and superficial glossitis may be present.
Syphilitic alopecia may be more or less general in the secondary period, due probably to the changed condition of the blood and consequent deficient nutrition of the hair. This early loss of hair may occur in the third month or later and usually consists of a general though irregular thinning of the hair on the head, thinning or notching of the eyebrows, eyelashes, mustache and beard, and sometimes of the pubic and axillary regions. The loss of hair may be very moderate or pronounced and, in aggravated cases, may be nearly or quite complete over the whole body. Occasionally the hair falls out in patches resembling alopecia areata or at the site of lesions involving the follicles. Early alopecia from syphilis may be aggravated by a coexisting seborrhea and tend to be more persistent but usually the loss of hair is temporary and is more restored spontaneously or by specific treatment within six months. In the tertiary period localized permanent baldness may result from destructive lesions, whether they end in absorption or ulceration. The remaining hair in case of advanced syphilis is apt to be dry and harsh. The irregular and incomplete nature of the alopecia and the notched eyebrow is always suggestive of syphilis. The more distinct patchy variety may be distinguished from the round or oval patches of alopecia areata by their irregular shape, tendency to be symmetrical and the presence of other signs of syphilis. The localized and permanent forms of alopecia due to the destructive lesions of late syphilis may be differentiated from circumscribed baldness due to lupus, favus, chronic eczema, etc., by the clinical history and the quality of the cicatrices.
Fig. 150 – Syphilitic alopecia, showing a general but irregular thinning of the hair.
Syphilitic involvement of the nails may differ little from that caused by chronic psoriasis or eczema, and may lead to similar atrophic or hypertrophic changes, for a description of which the reader is referred to the section on diseases of the nails. They occur generally in the secondary period but may appear later. One nail only may be attacked or several at the same time, or more often successively. Onychia more commonly affects the nails of the hands and runs a mild course. Perionychia attacks the fingers and toes about equally, but those exposed to injury from use or accident are more liable. It may be acute in process and chronic in course and, in the ulcerative form, may destroy more or less completely the matrix and other parts of the nails. When the ulceration is confined to the sides or the free part of the nail a regrowth of a perfect nail may be expected and even prolonged ulceration of the base may not prevent the regeneration of a fairly good nail. Considerable pain and annoyance may attend ulcerative perionychia and the outcome is always uncertain; non- ulcerative forms give little trouble and are easily cured. A history of syphilis and the presence of existing lesions should distinguish a syphilitic affection of the nails from conditions due to eczema or psoriasis.
Laboratory Diagnosis – A positive diagnosis of syphilis is established by finding the spirocheta pallida. “It is easy,” says Schaudinn, “after a certain amount of diligence, to differentiate the treponema pallidum from other types of spirochetas in fresh preparations. the fineness and feeble refractibility of this spirochete, the constant, close, deep and regular character of its spirals, which are numerous (10 to 20), render it impossible to confuse it with other microorganisms of the same type. Its chief characteristic, however, lies in the fact that it retains its spiral arrangement not only during motion but also in the state of rest while the spirals of most other spirochetes disappear when they are in a condition of repose.” There are on the market condensers, adjustable to the best makes of microscopes that converge the light obliquely on the specimen. By means of such condensers the spirochetes appear as bright refractive bodies on a dark background and are readily recognized by their regular and closely set spirals. Serum from the margin of chancres or secondary lesions is the best material in which to look for spirochetes.
When it is impossible to diagnose syphilis by finding the spirocheta pallida or by a clear clinical history, the Wassermann reaction affords a reliable diagnostic test as well as a therapeutic index. This biologic test was introduced by Wassermann, Neisser and Bruck in May, 1906. It is based upon the well-known principle of the power of the serum of one animal to dissolve the red corpuscles in the serum of another species. This process is known as hemolysis and is dependent upon three substances: a complement, always present in any blood serum; an anti-body, resulting from the reaction of the injected animal against the injected red blood cells; and an antigen, in this instance the injected blood corpuscles. It is the union of these three elements which constitutes the hemolytic system. If the corpuscles settle at the bottom of the test tube the test is positive (hemolysis restrained), demonstrating the presence of living spirochetes. When the test is negative (hemolysis), the fluid in the tube is diffusely stained with free hemoglobin and it is presumptive, but not absolute, evidence of the absence of syphilis.
Positive reactions have been obtained in other diseases than syphilis, such as yaws, scarlet fever, malaria, leprosy and pellagra. Numerous modifications of the original test, notably that of Noguchi, have been evolved and, inasmuch as they simplify the technic and make the procedure more certain, it may be expected eventually that this test will become a more reliable diagnostic method. Some constitutions react against infections much more forcibly and quickly than others and therefore there is some variance in the time of appearance of the first positive Wassermann in cases of primary syphilis. It has been proven that the ingestion of mercury or a recent alcoholic debauch tends to the production of a negative or partially negative reaction in one that would otherwise be positive. Thus, a negative Wassermann report does not eliminate syphilis if there is a history of recent mercurial or alcoholic ingestion. Appreciating these exceptions, a positive Wassermann may be expected in nearly all cases of secondary syphilis and in a majority of the tertiary cases. Repeated negative Wassermanns indicate the cure of the disease.
Numerous classifications of the syphilids have been proposed but the simplest is, to all intents and purposes, the best and the characteristic forms of the macular, papular, vesicular, pustular, bullous, tubercular, gummatous and pigmentary types with their subdivisions will now be considered. Besides the general diagnostic points already touched upon, a differential diagnosis of these forms from diseases presenting similar lesions will be discussed under their respective headings.
Macular Syphilid (syphilitic roseola; exanthematous syphilid; erythema syphiliticum; erythematous syphilid). – This is the commonest form of the early syphilitic eruptions and occurs in the sixth to eighth week, rarely earlier or later, after the primary sore, and is usually preceded by moderate fever. The eruption occurs as round, oval or irregular, ill-defined, pea- to fingernail- sized macules. At first a bright pink or rose-red disappearing on pressure, in a few days to four weeks they appear bluish, grayish-brown or coppery-colored, little changed by tint, the color being so faint that it is hardly noticeable. Single patches develop in about two days and the whole eruption may be complete in seven to ten days. It occurs earliest and most abundantly over the chest and abdomen, less often upon the upper extremities, neck and back. Rarely, it is widely or generally distributed though it seldom invades the face or the dorsal surface of the hands and feet. The lesions may be few or many, as a rule showing no tendency to become elevated, coalescent, circular or scaly. The eruption lasts for one to four weeks, leaving behind a brownish-gray stain. Relapses sometimes occur during the first year and small circinate lesions may be present in the second or third years, or even later, and prove rebellious to treatment.
In some instances there is a tendency toward the maculopapular type and although this may embrace the entire eruption, it is quite common to find a few of these lesions on the palms and soles and about the genitals and anus, even when they are not found elsewhere. This form may have a tendency to develop into circles or parts of circles. It is apt to be preceded by more severe constitutional symptoms, is more rapid in its invasion, more persistent in its course and exhibits more desquamation than the purely macular type.