Part 68

Variola is an acute, febrile, contagious, and systemic affection, preceded by an incubative period, characterized by the evolution of symptoms in a relatively determinate order, with a cutaneous efflorescence successively papular, vesicular, and pustular in type, followed by crusting, and terminating either fatally or by complete convalescence, with or without sequelæ in the form of multiple, circumscribed, and superficial cicatrices.

SYNONYMS.--Lat., Variola; Eng., Small-pox; Fr., Petite Vérole; Ger., Pocken; Ital., Vajuolo.

HISTORY.--Small-pox is a disease which, there is reason to believe, was first developed in the earliest ages of which the human family has record. Originating probably in China, India, and the adjacent countries of the Asiatic continent, its extension over Europe and America was, without question, in the line of progress pursued by the advancing centres of traffic and population. The earliest traces of its ravages can be dimly recognized in the descriptions of writers in the middle and latter parts of the sixth century. In the early years of the tenth century, however, a remarkably accurate picture of the disease was drawn by Rhazes, a physician of Bagdad. His treatise, translated by Greenhill for the London Pathological Society, sets forth the views of an Egyptian physician named Ahron, who wrote in the sixth century. After these dates the remarkable political and social changes in Europe, which are to be attributed either directly or remotely to the Crusades, contributed largely to the opportunities for the spread of the disease and to the occurrence later of those decimating epidemics which became veritable scourges. In the last century the resulting mortality in some of the countries of Europe was often equal to the entire population of one of their largest cities. If a modern traveller could find himself transported to the streets of the city of London as they appeared in the early part of the present century, it is probable that no peculiarities of architecture, dress, or behavior would be to him so strikingly conspicuous as the enormous number of pock-marked visages he would encounter among the people at every turn. In the face of all cavil and sophistry, medical science will always count among its greatest triumphs the modifications which variola has undergone since its preventive treatment was established upon a satisfactory basis by the discovery of the immortal Jenner.

{435} The bibliography of the disease is extensive, and the list of authors contributing to the subject is enriched by the names of such men as Boerhaave, Van Swieten, Sauvages, Willan, E. Wagner, Johanny Rendu, Hebra, and, more lately, Kaposi.

ETIOLOGY.--Respecting the etiology of variola, it can scarcely be affirmed that our knowledge has been greatly extended since the date of the experiments of Jenner. There is no historical knowledge of its generation de novo; and the earliest cases of the malady must therefore be classed with the exceedingly rare instances of spontaneous cow-pox which have proved such a boon to the vaccini-culturists. To-day every case of small-pox is justly regarded as having been directly or indirectly transmitted from one or more individuals affected with a similar disorder. It is thus recognized as specifically infectious, contagious, and inoculable, its transmission occurring, first, without contact, by atmospheric conduction of a volatile contagious principle of unknown nature; second, with contact either by (a) actual transference of dry or moist infectious secretions deposited upon a susceptible surface, immediately or through the medium of garments, bed-clothing, paper money, and similar material substances; or (b) by inoculation of unprotected persons with the pathological product of an infected organism. There is no doubt but that the contagious principle displays its greatest activities in connection with the contents of the lesions undergoing a change from the vesicular to the pustular phases, though from the beginning to the end of the disease it is probable that all the tissues and fluids of the infected body are in various degrees capable of producing the malady in those who are unprotected. Furthermore, whether associated or not with an organic substance, the contagium of the disease is known to preserve the power of reproducing itself for a period lasting for weeks, months, and even a longer time. A field for its activities once secured, there is a period of time during which few if any evidences of its progress are declared, this period being abruptly terminated by distinct and characteristic symptoms. This is known as the period of incubation.

The nature of the contagium in small-pox has been the subject of much speculation, careful investigation, and experiment, the results having established but few facts of any practical value. There is at present no proof that any bacteria, vegetable germs, or other minute organisms foreign to the human body are the essential causes of the disease. It is certain that in health the human body is completely enveloped in a volatile medium emanating from the secretions of the glands of the skin, which can be recognized by some of the keen-scented lower animals when it is wafted through the air at a distance of several hundred feet from a single individual. It is reasonable to conclude that not only in small-pox, but in other contagious and infectious diseases, these emanations possess a pathological character, and become capable of transmitting such maladies from diseased to healthy organisms. Certain also it is that when the subjects of these diseases are crowded together, as in prisons, hospitals and camps, this contagious element gathers an unwonted intensity. By far the larger number of all transmissions of variola occur after inhalation of the infective medium--in other words, by the avenue of the lungs. It is probably for the same reason that the disease spreads more widely and with greater virulence during the cold seasons of the {436} year, in this latitude especially from December to February--a time when the ventilation of inhabited dwelling-houses is usually much less perfect than in warmer weather.

The disease affects individuals of all ages and both sexes, not sparing the foetus in utero, and, in the case of the latter, occurring both with and without previous infection of the mother of the unborn child. Nowhere are its ravages so extensive and followed by such fatal results as among those who have long been unprotected by previous vaccination. Among the debilitated, as also among the very young and the very old, small-pox is liable to be followed by severe complications and a fatal result. Negroes, possibly in consequence of tendencies inherited through generations of unvaccinated ancestors, are particularly prone to the disease. Lastly, there is occasionally noted an individual idiosyncrasy, in consequence of which either a remarkable susceptibility to the disease exists or a no less singular immunity against its encroachment is conferred.

Thus, physicians, much exposed to its influences in the discharge of their professional duties, are known to be relatively exempt, while other individuals, few in number it must be admitted, have either had repeated attacks of the malady itself, or, after each exposure to its contagious principle, a recurrent illness of variable type. In the immense majority of all cases, however, one attack confers immunity upon the sufferer against subsequent invasion of the disease for the remainder of life. Upon a few occasions I have known variola to occur in individuals previously affected with cutaneous diseases, especially the eczematous--a fact which merely suggests that such pre-existing disorder of the integument conferred no immunity against infection.

SYMPTOMATOLOGY.--The earliest symptoms of small-pox may be occasionally recognized during the stage of incubation, which, as described above, embraces a period of from ten to fifteen days, though these limits are not absolutely fixed, since both shorter and longer incubative periods have been at times established. During the interval the patient may appear to enjoy perfect health, or, on the other hand, suffer from an ill-defined malaise, with anorexia, languor, insomnia, and allied symptoms. Close observation of the patient thus affected will often reveal the existence of a peculiar pallor of the face, accompanied by a skin-color which suggests a slight degree of sallowness of the complexion. These rather indeterminate symptoms are naturally most marked toward the completion of the period of incubation.

The latter terminated, the period of invasion follows, and extends from the conclusion of the incubative stage to the moment when the first cutaneous lesions of variola appear upon the surface. The symptoms which characterize the onset of this period of invasion are conspicuous and characteristic. There is often a sharp vespertine rigor or a more or less continuous chilliness, accompanied by sensations of "creeping" over the surface, lasting even for several hours. Meantime, the temperature rises to 103° or 105° F., the pulse running up to 120 or 130 beats per minute. In this febrile condition there is commonly complaint of a characteristic aching in the head and back, intense, scarcely intermittent, and so peculiar as to have frequently furnished a clue to the diagnosis of the approaching malady. These sensations are quite analogous to the substernal and other pains which frequently precede the first explosions {437} of syphilis, and are all, without question, due to the circulation of a poisoned blood, the influence of which is in this manner confessed by the nervous system. In the case of infants and young children the invasion of small-pox is frequently ushered in by delirium and convulsions--symptoms which are to be explained by the facts just named.

This complexus of febrile and nervous symptoms, varying somewhat in intensity and possibly interrupted by sensations of chilliness, may be recognized as continuing on the second and third days of the period of invasion. Meantime, there may be noted a dusky hyperæmia of the pharynx and tonsils, the surface of which may even display elevated points which develop later into papules. In exceptional instances the intensity of the poison is such that the system fails to rally before the violence of the onset, and a fatal result ensues before the characteristic exanthem appears upon the skin.

On the second and third days of the invasion stage of the disease, if they are displayed at all, the variolous rashes appear. Too much attention can scarcely be paid to the importance of their recognition on the part of the diagnostician. Often indeed have practitioners been deceived by their occurrence, having been either completely blinded to the serious nature of the malady in progress, or, as Bartholow has well shown, having supposed that they were dealing with a concurrence of variola and scarlatina or rubeola.

Hebra was the first to point out the significance of the rash known as roseola variolosa or erythema variolosa. Occurring at about the dates named above, it is in a few patients pronounced and vivid, even in solitary instances rivalling in severity the exanthem which succeeds it. In others, the majority of all patients in some epidemics, it may be entirely wanting. The writer has certainly observed its most typical development in women who were either menstruating or in the puerperal state. It is said also to be relatively frequent in subjects of a tender age. Kaposi has recognized it in all its manifestations at every age.

It appears in the form of puncta, striæ, or diffuse and uniform blushes covering extensive areas of the integument, livid red, purplish, or brownish-red in hue, paling under pressure, but never leaving upon the skin over which the finger-nail is quickly drawn the characteristic whitish streak by which many practitioners test the scarlatinal rash. The surfaces involved may be either not raised or slightly elevated above the general level of the skin, and are usually circumscribed. The regions chiefly involved have been carefully described by Th. Simon, and are hence sometimes called Simon's triangles. Thus the groin, the internal face of the thighs, and the hypogastric region may be involved at once (femoral triangle of Simon); the surface of the axilla, the pectoral region, and the inner face of the arm (brachial triangle of Simon), as also the extensor faces of the knees and the elbows, the dorsum of the feet, and indeed every portion of the surface of the body.

In the midst of these rash-covered areas may also appear petechial or hemorrhagic, dark-red, pin-head to bean-sized maculæ, which undergo color-changes both in lighter and deeper shades as the invasion period {438} lapses. In lieu of these, however, transient wheals may come and go over the surface, and even the erythema described above may assume an erratic phase and appear in one part only to disappear and recur at another. None of these flash-light warnings of the oncoming exanthem are proportioned to the latter in the matter of extent and intensity of development. They may be followed by grave or mild manifestations of the disease. The subsequent eruption may also be much more abundantly developed in regions where the invasion rashes have not appeared, and the latter completely fade before the former have advanced to occupy the field thus deserted.

The invasion stage of variola commonly occupies three days. Rarely it extends into the fourth, fifth, and even the sixth, day after the premonitory chill and fever.

Upon its subsidence the exanthem of the disease as a rule promptly appears. Simultaneously, the temperature abates, the rapidity of the pulse diminishes, and there is marked amelioration of the general symptoms. The patient, frequently deceived by the completeness of this defervescence, is apt to conclude that he is convalescent from his disorder, and is thus often astonished at the discovery of the exanthem upon the person, usually the face. In other cases, more commonly those of a grave character, there is failure of this defervescence, the febrile symptoms continuing or even increasing in severity.

The eruption first appears in the form of pin-head sized and larger, firm, conical, discrete, coherent or confluent, reddish papules, sometimes accompanied by mild sensations of a pricking or painful character, often exciting no subjective symptoms by which their presence could be declared. To the touch they are characteristically indurated, and suggest the hardness of small shot imbedded in the skin. They appear first and in greatest abundance upon the face and scalp, involving later and progressively the trunk, the extremities, and the palmar and plantar surfaces. It is at this moment that the eruption most resembles that to be recognized in measles (the distinction between the eruptive symptoms of the two diseases will be considered later). At times a reddish areola surrounds each lesion, especially those appearing upon the trunk. All are situated about the orifices of the follicles and glands of the skin.

On the first and second days of the eruption the papular lesions multiply in number, involve an increasingly large area, and individually augment in size; so they appear first upon the head, and are successively presented to the eye upon the lower portions of the body. The older lesions are usually recognized upon the scalp, face, neck, and shoulders; the more recent upon the extremities. By the third day of the eruptive stage there is usually evident at the apex of the older lesions a minute vesicle containing a drop of pellucid serum, which rapidly changes in character and size till a distinct vesicle is formed with cloudy or lactescent contents. Early in their career an apicial depression can be seen, which later deepens into a characteristic umbilication. This umbilication in the vesicular stage is somewhat peculiar. It is more than a mere depression of the summit, such as might be made by thrusting a blunt-pointed pin centrally and downward so as to carry the roof-wall before it. It is made clinically most distinct by the fluting or puckering of the peripheral part of the roof-wall, giving the lesion a crenated appearance which is not {439} assumed by any other cutaneous efflorescence of multiple development. It may be regarded as pathognomonic of variola.

The pock is usually mature by the sixth day of the eruption. It is pea-sized and globular in shape; its umbilication has been usually quite removed by the complete filling of its chamber with distinctly purulent contents; it is often surrounded by a halo due to hyperæmia or exudation; and, the total number of individual lesions being then fairly determined, it is often closely set against its fellows, islets of unaffected integument having meantime become fewer and more contracted. The face, covered with this eruption, then exhibits a typical aspect. The entire integument becomes swollen and brawny or oedematous. The eyes are thus closed by the tumid lids, which are separable with difficulty, and this, too, even though they be the seat of comparatively few lesions. The nose, lips, cheeks, and ears are by similar processes deformed and given a most repulsive unsightliness. Mucus and puriform secretions gather and dry about the mucous outlets. The skin of other parts of the body (hands, feet, genitalia, and the entire extremities) is in a similar condition, merely most noticeable in the exposed and disfigured visage.

The fever of maturation or suppuration, or, as it is often called, the secondary fever, is lighted to activity with the onset of the suppurative process. The temperature rises to a point ranging between 101° and 105° F., the pulse-rate simultaneously rising to 100 and even 150 in the minute, varying of course with the age of the patient and the severity of the attack. During its continuance, from the eighth or ninth to the eleventh or twelfth day of the disease, the victim of the malady is in a deplorable and critical condition. The intense grade of cutaneous inflammation, with its resulting subjective sensations of burning pain and tension, the soreness of the mouth (tongue, pharynx, inside of lips, and palate), due to the existence of pus-filled pocks upon the buccal membrane, and, for similar reasons, the dysphagia and irritation of the larynx and tracheal membrane, are all sufficient to account for the general condition. In cases of mild grade the patient lies conscious, but in a stolid apathy, listlessly accepting the services of his attendants. In others there is delirium of low or high grade, often sufficient to demand constant surveillance, lest in consequence the patient do serious injury to himself.

The behavior of the pustules which appear upon the mucous surfaces accessible to the eye is modified somewhat by the heat, moisture, and friction to which these surfaces are exposed. Typical, fully-distended pustules occasionally persist upon the soft palate and the inside of the lips. Soon, however, the macerated roof-wall yields, leaving a reddish floor where the mucous membrane is exposed, denuded of its epithelial layer or covered with a new tender and hyperæmic pellicle. In grave and severe cases these pustular lesions may extend deeply into the mucous tracts, involving the trachea, bronchi, or alimentary canal. In an autopsy made by the writer on the body of a male subject dead of unmodified variola, there was no portion of the alimentary canal from the mouth to the anus which was not studded by thickly-set pustules. The urethra, vagina, vulva, external auditory canal, and conjunctivæ are, in severe cases, similarly involved. According to Kaposi, the tympanum is usually exempt.

The period of desiccation begins usually on the thirteenth or fourteenth {440} day of the disease, and, according to the severity of the previous pathological processes, requires for its completion from one week to a fortnight. Its onset is characterized by a second marked but gradually developed defervescence. With a diurnal temperature successively less elevated above the normal standard there is a corresponding fall of the pulse-rate. As the disease has by this date taxed the vital resources of the system to the utmost limit, the exhaustion resulting may be declared by a pulse which is flagging, weak, and even in the matter of frequency much below the standard of health.

The cutaneous lesions now again undergo a change. Some of the pustules rupture, and their viscid contents, oozing forth, concrete into a yellowish crust which gradually assumes a brownish hue. Others desiccate en masse, the roof-wall first collapsing upon the contents, thus producing an appearance which again suggests umbilication of the lesions. This is sometimes termed a secondary umbilication. The desiccation en masse is doubtless due to the evaporation of a portion of the fluid exuded into the superficial strata of the integument, and the consequent inspissation of the pus. Often the face at this moment is totally concealed by a dense, dry, brownish or even blackish mask, composed of the crusts furnished by numerous individual lesions. At the same time the tumefaction of the skin subsides, and the subjective sensations to which it gave rise gradually disappear. Beneath the crusts cicatrization advances till the former are lessened, and finally, becoming detached, fall in quantity from the surfaces subjected to friction. Beneath them are seen brownish and violaceous blotches, the integument thus stained slowly losing its abnormal color. It is thus seen to be the seat of multiple, slightly depressed, shining scars of a dead white color, which in the course of time lose somewhat of their disfiguring prominence, but which when typically distinct persist for a lifetime. This exfoliation of crusts continues till the skin is completely rid of its pathological products, the process being completed with entire restoration to health about the conclusion of the fourth or fifth week of the disease. Meantime, in favorable cases, convalescence progresses pari passu. The patient has a returning appetite, decadence of symptoms originating in impairment of function of the mucous membranes, and gains in weight till the restoration to sound health is complete.

Such is the history in outline of what may be regarded as a typical form of uncomplicated variola. It should not be forgotten, however, that in different epidemics there are marked differences in the career and manifestations of the malady, and that even among the cases observed in a single locality visited by the disease the same divergence of symptoms is no less conspicuous. This diversity is due to several causes, irrespective of the remarkable modifications displayed in the variolous who have been previously vaccinated. Individual susceptibility is doubtless to be considered in this connection, as also the temperament, bodily vigor, and hygienic surroundings of those who are infected. It is possible also that the intensity of the poison may be subjected to occasional modifications in its transmission from individual to individual. In this way the following types of variola present themselves in clinical forms with divergent features:

CONFLUENT VARIOLA (variola confluens).--This virulent form of {441} small-pox is ushered in by a relatively short incubative period, followed by a severe invasion of the disease. The premonitory chill is violent; the cephalic and lumbar pains are excruciating; the fever, rising to a high grade, 106° to 110° F., with few and slight remissions, scarcely subsides, if at all, with the appearance of the eruption, the latter developing early, and, to borrow an expression from syphilographers, exploding with violence over large areas of the surface of the body. The initial lesions of the exanthem are dense and deeply-set papules, so closely coherent even at this moment that they scarcely leave between them interspaces of sound skin. During the vesiculo-pustular transformation which they promptly undergo on the second day there is a more or less complete coalescence of the elements of the eruption, which circumstance has given this form of the disease its name, confluent variola. This confluence is most conspicuous upon the face and hands, where large flat vesicles run together, form pus-filled bullæ, and finally convert the surface on which they rest into a single, large, many-chambered pustule. All this occurs upon an enormously swollen and inflamed skin, disfiguring every feature of the face and wellnigh obliterating every external distinction between the scalp, nose, eyes, and mouth. Here and there the mass is elevated by the quantity of exuded pus to a more notable projection from the surface. Pustules filled with blood may appear at several points. At others, the suppurative inflammation may be seen to have eroded the derma, which is covered with a diphtheritic membranous exudation similar to that covering the mucous membranes lining the mouth, nose, and ears. Naturally, the skin in its totality often yields to these destructive processes and in large patches falls into gangrene.

The confluence of the lesions is less marked in other parts of the body than the face and hands, yet the entire surface may be covered with a coherent exanthem which becomes elsewhere, in large areas, confluent. The writer has seen patients in whom the head of a pin could not be placed upon an unaffected patch of skin in any portion of the body. The parts subjected to pressure in the reclining posture, such as the back, shoulders, and buttocks, are especially liable to this coalescence of the pustular lesions.

In confluent variola too, as already intimated, the mucous surfaces suffer proportionately. Pasty accumulations of muco-pus and diphtheritic exudation, like macerated chamois leather, cover the tongue, which is often so enormously swollen as to bulge between the teeth and project from the mouth. These exudations line the mouth, pharynx, larynx, and even the bronchi. Beneath these masses the eroded mucous surface is dry, livid red in color, and has a varnished aspect. Gangrene here may lead to necrosis of the cartilages of the larynx. Aphonia is often complete, deglutition impossible, respiration difficult. The stench arising from the patient is intolerably fetid and pervading, and a single exhalation will poison the best-ventilated apartment. The submaxillary and sublingual glands are enlarged and the neighboring lymphatics swollen.