CONFLUENT VARIOLA (variola confluens).—This virulent form of 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.
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 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, 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.