Part 69

The patient who is plunged into this grave condition is the victim of a fever which is unquestionably septicæmic in character; he has a small, frequent, and often fluttering pulse; his mental condition is betrayed by a delirium of varying grade or he lies comatose. In this state a fatal {442} result is often induced by either exhaustion of the vital forces or an intercurrent malady, such as pleurisy, pneumonia, cardiac inflammation, oedema of the glottis, or an uncontrollable diarrhoea. In yet other cases the patient falls into a typhoid state, and, after surviving for a fortnight or more with a low fever, a broncho-pneumonia, or a diarrhoea, succumbs to an inevitable exhaustion, the surface of his body being yet covered with a dry, blackish, and fetid crust.

The expression of an intense variolous poison is known as hemorrhagic variola; also as purpura variolosa and black pox. A large number of such cases have been designated and treated as black measles, the real nature of the malady having been mistaken.

The law readily observed by the diagnostician of diseases in general must here be recognized. There are no hard and fast lines in nature. Hemorrhagic variola occurs, without question, in different types. At the one extreme are classed the inevitably fatal cases, where the patient sinks smitten by the malady even before the exanthem is developed; at the other are found the cases of confluent variola, not necessarily fatal, in the course of which hemorrhagic lesions appear in variable number, blood either filling the pustules after the latter have arrived at maturity, or forming ab initio purpuric pocks intermingled with the typical lesions of the variolous exanthem. However ill-defined the limits between these classes may be, the symptoms of hemorrhagic variola are sufficiently characteristic to require separate description. According to Kaposi, it occurs in the two following types:

The first form is termed variolic purpura. Its incubative period is brief and distinguished by unusual conditions of malaise and lumbar pain. On the fourth day there is an intense fever with rapid pulse, and this is speedily followed by a deep purplish-red staining of the face, neck, trunk, and extremities, the skin thus affected being slightly tumid and quite dry. Minute maculo-papules can be distinguished here and there over the surface, often closely set together, and presenting the characteristic color described above. At this stage of the disease the eruption greatly suggests an intense rubeolous exanthem, and has been, as a result, repeatedly mistaken for the so-called black measles. But the excruciating pains persist, there is often coincident delirium, and the pin-head sized maculo-papules noted above become lenticular in shape, cease to lose their color under the pressure of the finger, extend peripherally even in a few hours, flatten and become purpuric patches of a bluish-black shade, palm-sized and even larger, covering extensive areas of the integument, new lesions forming in unaffected islets of the skin; conjunctival ecchymoses appear at the angles formed by the lids, and finally encircle the cornea with an annular purplish-black cushion. The mucous surfaces become dry, crack, and bleed where the epithelium is torn, and become covered with offensive crusts. The odor exhaled by the patient is intolerably fetid. He lies stupid as the march to a fatal issue is hourly hastened. Hemorrhages occur from the larynx, bronchial membrane, intestinal surfaces, and even into the parenchyma of the viscera, the muscles, serous membranes, periosteum, and neurilemma. The urine is retained in the bladder; the respirations rapidly increase in frequency; the pulse flutters; and death closes the scene between one and two days after the onset of the malady. In several cases observed by the writer, {443} occurring in infants and children, the entire course of the malady was completed in twelve hours.

In the second and much rarer form of hemorrhagic variola there are the usual unfavorable portents of intense prodromic symptoms. On the fourth day the skin is swollen and indurated in consequence of the development within its structure of numerous firm, roundish, slightly acuminate papules, so thickly set together that it is wellnigh impossible to distinguish between them. These are early in betraying the bluish-black hue significant of hemorrhage into their mass. They multiply in number and increase in size, while their hemorrhagic stains widen and sweep from each as a centre, like the waves that spread from a pebble thrown into smooth water. In these cases, more often than in those first described, pus-filled pocks may develop over some portions of the surface, while in others a species of gangrene occurs in consequence of the separation of the derma from the subcutaneous tissues by effused blood. At times pustules of somewhat typical aspect are formed and subsequently filled with blood by a hemorrhage from below. The accompanying symptoms are grave, but less rapidly fatal than in the other types of the disease. Delirium, stupor, an intense fever, and a rapid, feeble pulse are commonly noted. A fatal result is usually reached in from four to five days.

Hemorrhagic lesions, isolated or confluent, are seen also in severe forms of variola, not of the two types described above. Thus, in confluent small-pox, especially when occurring among the unvaccinated, some of the pustules on the face, the back, or possibly the legs, where varicosities of the veins permit a passive engorgement of the tissues with blood, may become the seat of a hemorrhage. For these local causes are often etiologically effective. In other cases the appearance of the hemorrhagic lesions seems to be due to a dyscrasia, such as that recognized in phthisis, chronic alcoholism, and hæmophilia.

Aside from the trivial accidents to which the exanthem may be subject, the hemorrhagic types of variola may be regarded as necessarily grave and in a large proportion of cases inevitably fatal. That they are all truly the results of variolous poisoning is shown, first, by the occurrence of intermediate forms; second, by the occasional transmission of the disease in its typical aspects to the partially protected.

VARIOLOID is that form of variola in which the disease is modified, either in its course, duration, or intensity of symptoms, such modification usually resulting, directly or indirectly, from the protective influence of vaccination or from a previous attack of variola.

The symptoms of the class of patients commonly regarded as suffering from varioloid are all those of variola, modified, however, in the direction of a mitigation of their intensity and dangerous character. It is thus evident that there is no strict line of demarcation between the very mildest physical expression of the variolous poison and that variola vera which presents atypically benign symptoms in any stage of its career. Within this wide range of possibilities cases of varioloid occur which certainly differ from each other by very marked degrees.

The invasion stage of varioloid may be shorter or longer than that occurring in variola vera, and may be insignificant or intensely marked as regards the severity of its symptoms. According to Bartholow the {444} invasion rashes are here of common occurrence; and the more extensive the latter, the less copious the subsequent eruption. It must be admitted that a personal experience has not confirmed us in this view.

After the high fever and severe cephalic and lumbar pains of this stage there may follow, in the case of varioloid, a complete defervescence and the appearance of a very copious exanthem. With this, however, the apogee of the disease may be reached, and the subsequent symptoms be altogether insufficient in comparison with those which have preceded. Thus, the maculo-papules may never reach a vesicular stage, or, having attained this, the vesicles may not be umbilicated, or may shrivel after their contents have assumed a lactescent color, and be succeeded by light superficial crusts which in a few days fall. Or, again, the pustular stage of the lesions may be fully developed, even with the production of a halo about the pocks, while yet there is no swelling of the skin and but trifling subjective sensations experienced by the patient. The pustules in the course of from four days to a week desiccate and are shed, leaving behind them violaceous pigmentations of the surface without persistent cicatricial sequelæ.

Other cases, again, instead of producing the impression upon an observer of being illustrations of a malady aborted or cut short at some period of its career, seem to exhibit merely a modification in the intensity or distribution of symptoms betrayed in a wellnigh typical career. Thus, there may be a total absence or insignificant reminder of the septic fever usually known as the secondary fever of variola, and the elements of the eruption may be few or appear in scanty number upon the face and more copiously elsewhere. The latter may, however, pursue a perfectly typical career and be followed by characteristic scars.

There is yet another type of varioloid with which many practitioners become familiar who have experience in epidemics of small-pox. The patient exhibits distinct symptoms of malaise in the period of incubation. The fever of invasion, with its characteristic pains and nausea, is equally well marked. Defervescence occurs with a trifling eruption of maculo-papules, which in two days have wellnigh completely disappeared. There is no secondary fever, but the patient is far from well. There is a period of anæmia, mental depression, marked languor, and unmistakable evidences of physical prostration out of all proportion to the precedent symptoms. In these cases it may well be believed that the poison has at last produced a strong impression upon the nervous centres. The most characteristic feature of these cases is the tedious convalescence from an apparently trifling form of the malady.

The identity of varioloid with variola is abundantly shown--first, by the occurrence of intermediate forms of every grade, from the mildest evidence of variolous poisoning to typically developed cases of variola vera; second, by the fact that patients affected with varioloid are capable of transmitting variola to the unprotected; third, by the anatomico-pathological fact that the structure of the pock, when it appears, is the same in all.

A variation as to the form and contents of the lesion of modified variola occasionally occurs as a consequence of individual peculiarities or of the special surroundings of the patient. A number of useless terms have been employed to designate these peculiarities, the most of which {445} are relics of the superstitions of the past. In variola siliquosa the pocks are said to contain air only; in v. pemphicosa, bullous lesions predominate; in v. verrucosa, the papules, after partial evolution and involution, leave minute wart-like papillary masses upon the face; in v. crystallina, there are superficial vesicles only filled with clear serum, which somewhat resemble those recognized as sudamina. The older English writers with as little reason described cases of horn-pox, swine-pox, etc., differing only from those of variola by the anomalous behavior of the exanthem in the course of its evolution.

COMPLICATIONS AND SEQUELÆ.--The complications and sequelæ of variola are fewer in number and more restricted in range than those of many other maladies. This results from the remarkable unity of the disease as it occurs in its several manifestations among the unprotected, its relatively rapid progress, and its absolute disappearance on the completion of its curriculum. There is no chronic form of variola lingering for weeks and months after the violence of the fever has abated.

Furuncles and abscesses occasionally result during or after the pustular stage of the disease has been reached, sometimes of such extent as to give exit to large quantities of an ill-conditioned pus. The tissues, weakened by the suppurative process which the skin has undergone, may then necrose, and thus lay bare periosteum, cartilage, or bone. Erysipelas, especially about the face, may close the eyes, encroach upon the scalp, or spread extensively over other regions. Muscular paralyses, hemiplegic and paraplegic attacks, albuminuria, diarrhoea, and the inflammations of chronic type affecting the thoracic organs may each supervene, and either greatly prolong convalescence or precipitate a fatal issue. None of them is perhaps more common than a low typhoid and febrile state, in which the patient lies after his variola is practically ended, his skin struggling to regain its normal tone, a fever of remittent type taxing his energies, his bowels in frequent movements discharging a thin and fetid feculent matter, while a low delirium renders him insensible to the gravity of the situation.

Reference has been made above to the implication of the eyes of the variolous, and the possibility of the disorder terminating, after an otherwise favorable convalescence, in total blindness, should not be forgotten. The cornea may be the seat of pustules or a diffuse puriform infiltration resulting in ulceration, and eventually perforation with hernia of the iris. At times it is merely macerated by the pus continually covering it, and in that condition yields to even moderate pressure. At others the deeper portions of the globe fall into inflammation, and there is a resulting cyclitis, irido-cyclitis, or parophthalmia.

In the nose severe destructive effects may follow the pustular involvement of the Schneiderian membrane, including necrosis of the nasal bones and profuse epistaxis.

In a similar way, the external ear may be involved, the tympanum disappear, a severe otitis media supervene, and the mastoid cells become filled with pus and detritus of necrosed tissue.

{446} In the larynx, which may be well lined with pustules, as indicated above, complications may arise in the shape of oedema of the ary-epiglottic folds, laryngo-oesophageal abscess and various diphtheritic deposits lining every portion of the mucous membrane.

Other disorders noted as complicating variola are hydrocele and orchitis in the male, ovaritis in the female, gangrene of scrotum or labia, hæmaturia, peritonitis, adenopathy and lymphangitis and arthritis, as well as peri-arthritic suppurative inflammation.

PATHOLOGY AND MORBID ANATOMY.--Ours is a day in which bacteria, special to each of a number of infectious diseases (lepra, pemphigus, tuberculosis, etc.), are constantly reported as coming to light under the persuasive influence of modern staining solutions. With respect to variola, it may be said that while Cohn, Klebs, Weigert, and others have, without question, recognized microsphæra, micrococci, and similar organisms in variolous pus, their causative relation to the pathological process has certainly not yet been demonstrated.

The pathological anatomy of the cutaneous lesions of variola has been very carefully studied by Auspitz and Basch, and Heitzmann. The following is a condensed account of the results reached by these observers:

First appear circumscribed patches of hyperæmia, in which the papillary layer of the corium is concerned, and which is followed by some thickening of the rete, the epithelia involved becoming coarsely granular. This granular condition is due to an increase of living matter within the protoplasmic bodies, evident at the points of intersection of the reticulum of which they are composed, the nuclei becoming solid and shining, and the threads traversing this cement-substance between them becoming also increased in thickness. The papillæ beneath increase in size in consequence of their vascular engorgement, and in consequence of the change experienced by the connective-tissue bundles, which are partly transformed into protoplasm, while the protoplasm between them increases also. There is, in brief, a liquefaction of the glue-giving basis-substance, which makes visible the reticulum of living matter formerly hidden within it. In this way the epidermis is raised into the flat solid papules which are the early lesions of the disease.

Then follows an exudation of a serous fluid at one or more points in the papule, the meshes of the reticulum being so stretched and torn that small chambers are formed filled with the liquid exudate containing granules. Between these chambers the separating strata of epithelia are compressed so as to form septa or partition walls. The neighboring epithelia become granular, divested of their cement envelope, and transformed into protoplasmic clusters still connected with the living reticulum by slender threads. An irregular cavity is thus formed in the thickened rete traversed by septa, the contained exudation being filled with granules, coagulated fibrin, and lymph. A few protoplasmic bodies are here also distinguishable, which Heitzmann regards as either débris of destroyed epithelia or colorless blood-corpuscles.

In these changes the connective-tissue beneath participates. The papillæ eventually disappear, the superior portion of the corium being replaced by {447} clusters of medullary or inflammatory elements uninterruptedly connected by threads of living matter.

The pus-corpuscles which eventually appear originate mainly from transformed epithelia. In the process of transformation the increased protoplasm of the epithelia first exhibits shining homogeneous lumps, which, after an intermediate stage of vacuolation, undergo an endogenous metamorphosis into nucleated bodies with a reticulum in each. To the number of these there is possibly an addition by the immigration from below (diapedesis) of leucocytes.