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.
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[4] 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.
[Footnote 4: _Loc. cit._]
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.