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.[5]
[Footnote 5: Besides the terms given above, Hebra gives the following
list of Latin adjectives which have been employed to describe special
varieties of small-pox, none of which requires special explanation:
variola papulosa, conica, acuminata, globosa, globulosa, tuberculosa,
cornea, fimbriata, miliaris, lymphatica, vesiculosa, pustularis,
rosea, morbillosa, carbunculosa, etc.]
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,[6] laryngo-oesophageal abscess and various
diphtheritic deposits lining every portion of the mucous membrane.
[Footnote 6: J. William White, "Surgical Aspects of Small-Pox,"
_Medical News_, March 4, 1882, p. 241.]
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,[7] and Heitzmann.[8] The
following is a condensed account of the results reached by these
observers:
[Footnote 7: _Virch. Archiv_, Bd. 28.]
[Footnote 8: _Trans. of Amer. Derm. Ass._, Aug., 1879.]
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.
The question of repair with or without the production of cicatrices
rests upon the behavior of the connective-tissue elements. If these
are not torn asunder, but remain in connection with each other, the
re-formation of a glue-giving basis-substance is possible, and new
bundles of fibrous connective-tissue take the place of the old. If, on
the contrary, the latter are completely destroyed, their place is
filled with the cicatricial new growth. The pigmentation, which is
such a common transitory sequela of the skin lesions, is due both to
the imbibition of the coloring matter of the blood by the epithelia
and by direct hemorrhagic exudation into both the rete and derma.
The umbilication of the mature pock is doubtless due to the situation
of such lesions at the orifices of the excretory ducts of the
skin-glands. The epidermis, in one or more of its strata, dips
downward to form a living investment for such glands, and in this
situation ties down the centre of the roof-wall of the pustules.
Eventually, it too, as a result of the maceration and tension
incidental to the complete filling of the pock with pus-elements, is
ruptured or stretched, and the umbilication of the pustule disappears.
The anatomy of the exanthematous lesions in hemorrhagic variola is not
different from that described above. The pocks in such cases are
merely filled with blood instead of with pus or sero-pus. In some
forms of hemorrhagic variola, as indeed would be suggested by their
clinical observation, there is hemorrhage directly into the tissues of
the integument, or, more probably in severe cases, a mere passive
leaking of the sanguineous fluid with its coloring matter through the
relaxed and weakened vascular walls.
The morbid changes occurring in the viscera are described by
Curschmann as follows: The mucous surfaces may be the seat of
pustules, diffuse purulent infiltration, and catarrhal, croupous, or
diphtheritic inflammation. As regards the extent of diffusion of the
pustular lesions, they occur, according to Wagner, in bronchi of the
second and even of the third order, rarely in the stomach and
intestines, and in the rectum only in its lowest portion. The bladder,
urethra, and serous surfaces are always exempt. The lungs, breast,
liver, spleen, brain, and spinal medulla are variously involved. Often
the tissues of these organs are quite unchanged as regards their
macroscopical appearance. At other times the tissues appear swollen,
granular, and undergo a fatty degeneration. In purpura variolosa the
spleen and walls of the heart, however, are seen to be firm, dark-red,
and more or less indurated.
DIAGNOSIS.--The establishment of a correct diagnosis where there is
question of variola is one of the most critical and important of the
duties of a physician. Upon such decisions have turned, again and
again, {448} professional success or disaster. To pronounce that case
to be variolous which is not of such a nature is to subject one to the
indignation of the few and the ridicule of the many. On the other
hand, to be guilty of treating a patient with small-pox, and of
remaining ignorant of the nature of the malady, is to subject many
ignorant people to the danger of exposure to the disease and to render
one's self liable for the redress sought by recourse to the civil
authorities and the law. It is difficult to decide which predicament
is the graver.
Typical variola vera is readily recognized by its characteristic
features. As usual, it is the atypical and modified forms where the
difficulty most often arises and where the danger to the physician is
proportionately increased.
In the invasion stage of the disease it is often impossible to
recognize any symptoms characteristic of variola. High fever with
severe lumbar pain, considerable gastric distress, and the appearance
of one of the invasion rashes (roseola variolosa) would, however, put
the observant practitioner on his guard. I have often noticed in these
cases a symptom which, apparently insignificant, has on more than one
occasion preceded the eruptive period. It is the occurrence upon the
centre of the two cheeks of a vivid damask-red blush, occasionally
having a purplish-red hue, and with a very remarkable circumscribed
area. This may be recognized in children and adults of both sexes when
it occurs in typical aspect, and is undoubtedly a hyperæmia of the
character of that producing the rashes in Simon's triangles.
When the variolous exanthem first appears the practitioner should
secure as soon as practicable a history of the invasion stage if this
has not been subject to his personal observation. He should then make
careful inquiry as to the possibility of a neighboring source of
contagion, and ascertain by inspection whether the person of the
patient exhibits the evidences of successful vaccination. In this
connection it is always well to estimate the value of the elements
represented by (_a_) the period ascertained as having elapsed since
the last successful vaccination; (_b_) the typical or atypical
character of the existing cicatrices of vaccinia; (_c_) the unicity or
multiplicity of the cicatrices simultaneously resulting from
vaccinations performed at one and the same date.
Without question, the first papular lesions of variola resemble those
of rubeola or measles to an extent which has often deceived the most
expert diagnosticians. The distinguishing points are--(1) In measles,
catarrhal symptoms (conjunctival, nasal, laryngeal, bronchial), which
are usually absent in the early stages of variola, and later are
obviously associated with the irritation set up of the pustules of the
maturing period. (2) The difference in the temperature record, that
noted in the invasion stage of variola varying from 104° to 105° F.,
while in rubeola it is rarely registered above 103° F. Moreover, in
typical variola the defervescence is marked and characteristic on the
appearance of the exanthem, while in rubeola, when the rash appears,
the temperature is usually sustained at a maximum, and may even rise.
(3) The differences in the rashes of the two disorders. The papules of
variola, even in its confluent forms, are, when first observed,
remarkably discrete and exhibit not the slightest tendency to
grouping, while the maculo-papules of rubeola are (_a_) developed
simultaneously on the face and trunk, while those of variola {449}
commonly appear first on the face and afterward on the trunk, the
older, and larger therefore, in the site of earliest appearance; (_b_)
are set in clusters or groups having a distinct tendency to crescentic
arrangement, a symptom decidedly best appreciated by the eye when the
eruption is viewed in totality or in large areas with the eye of the
observer somewhat removed from the surface; (_c_) are often made to
disappear or pale beneath the pressure of the finger, while there is
greater persistence of color in the variolous papules; (_d_) are
surrounded by little or no halo, each elementary lesion of the
eruption being abruptly defined upon the sound skin, while the
variolous papule is apt to rest upon a circlet of hyperæmic
integument.
Even with careful observation of all the specific differences between
the two diseases, they may, for a brief time, so resemble each other
as to defy the skill of the expert. In all doubtful cases the
physician should invariably admit the doubt and defer an exact
diagnosis for twenty-four hours. During the delay the variolous
exanthem should betray its individuality by the formation of a minute
vesicular apex at the summit of several papules.
In scarlatina the uniform diffusion of the exanthematous blush, the
absence of papules and vesico-papules, the continuance of the fever
after the rash has appeared, the characteristic scarlet or
boiled-lobster color of the skin, and the anginose condition of the
throat, are all significant symptoms. In hemorrhagic small-pox the
color of the integument is a much more purplish and lurid-reddish hue,
rapidly reaching that stage where it refuses to pale under the
pressure of the finger, and never leaving in the track of the
finger-nail quickly drawn over its surface the peculiar transitory
yellowish-white line which can be usually obtained in the skin of the
patient with scarlatina.
The pustular stage of variola might be confounded with the pustular
syphiloderm. But in the latter there should be a history of a chronic
rather than of an acute affection, and, as a result, the simultaneous
appearance of lesions in very different stages of their career, some
distended with pus, others ruptured and crusted, yet others which have
recently formed in the immediate vicinity of the oldest lesions, while
the latter have been in full involution or have been replaced by
superficial losses of tissue.
The resemblance of pustular variola to certain suppurative and other
disorders of the sebaceous glands is well attested by the name given
by certain French authors to molluscum epitheliale (M. contagiosum, M.
sebaceum)--viz. acne varioliformis. But in the case of acneiform
disorders the concurrence of comedones, the chronic course of the
disease, the absence of fever and systemic disturbance, and the
particularly irregular distribution of the lesions upon the face, with
failure to appear elsewhere,--all these facts forbid the confusion of
the affection with variola. In medicamentous acne, accompanied by the
sudden appearance of numerous pustular lesions symmetrically displayed
upon the surface, there will indeed be a source of error. In such
cases, of course, a history of the ingestion of a medicament capable
of producing a rash will afford valuable aid in the diagnosis. In
pustular forms of dermatitis medicamentosa there will usually be found
a more abundant development of the pus-containing lesions upon the
head and both arms and forearms, with {450} no tendency to extension
over very large areas of the trunk and lower extremities--a
circumstance which a delay of but a few hours will often substantiate.
The absence of marked defervescence is the most characteristic
difference between variola in its eruptive stage and typhus, typhoid,
and relapsing fevers. Pneumonia, cerebro-spinal meningitis, acute
miliary tuberculosis, and gastric fever are all to be differentiated
from variola by the occurrence of symptoms characteristic of the
involvement of the several organs which in these diseases respectively
are more particularly impaired.
PROGNOSIS.--The prognosis of variola is wellnigh inseparably
associated with the question of protection by vaccination. Variola
vera in the unprotected is an exceedingly fatal malady, the death-rate
varying in different epidemics according to the severity of each and
the ages and hygienic surroundings of the victims of the disease.
Certainly, from 15 to 50 per cent. of unprotected individuals affected
with the disease occurring in epidemic form in any given community
will perish. This number may, however, be enormously increased, as,
for example, among a large number of unprotected negroes crowded
together in a filthy prison, or when the malady makes a periodical
visitation to an insular community where long isolation has begotten a
carelessness with respect to vaccination.
With respect to individual cases it may be asserted, first, that an
intense series of prodromic symptoms, followed by the appearance of an
unusually large number of cutaneous lesions, is often unfavorable.
Confluence of the latter adds to the gravity; hemorrhagic and purpuric
symptoms are in the highest degree portentous, and commonly indicate a
fatal result. Women pregnant or in the puerperal state, infants at the
breast, and persons of both sexes at advanced ages, are little able to
resist the ravages of the disease. According to Kaposi, women recently
delivered prematurely or who have lately suffered from an abortion
succumb more often than others of their sex. Chronic alcoholism among
male subjects and the cachexia induced by all chronic visceral and
systemic disorders are sources of weakness which largely increase the
death-list by adding to the heavy strain upon the vital energies. The
prognosis is rendered uncertain or unpromising by extensive
involvement of the mucous as well as of the cutaneous surfaces, by
marked visceral complications, by evidences of shock or exhaustion
before the apogee of the exanthem is reached, by grave sequelæ, and
even by simple complications of the malady when, instead of entering
promptly upon convalescence, the patient lingers for weeks in a
typhoid condition. An unfavorable symptom in any case is the sudden
cessation of the processes actively pursued upon the surface of the
body. The swelling of the integument then suddenly diminishes and the
crusts by which it was covered shrivel. The eruption, in brief, seems
to undergo what may be described as a collapse. The pulse at such
moments usually flutters feebly, and there are other portents of
dissolution which the eye of the physician will hardly fail to
interpret correctly. The fluids in such instances mechanically drain
away from the surface of the body to seek the deeper parts. This is
not peculiar to small-pox. Similar phenomena occur even in the case of
other than exudative affections of the skin. In pityriasis rubra the
{451} patient dies leaving an integument apparently unaffected, and I
have seen a patient dead of even multiple sarcoma of the skin when the
tumors were reduced fully one-half in bulk as the result of a similar
cause.
On the other hand, the practitioner should never forget that even
apparently desperate cases of variola rally and are won back to life.
That the exudative process should be in full evolution at the surface
of the body is, cæteris paribus, certainly so far a good omen. The
most hideous, extensive, and stench-emitting crusts have hidden for a
time the forms that have for many subsequent years not only known the
enjoyment of life, but have made that life of inestimable value to
others. The physician in the presence of this most loathsome and
formidable disease should never despair.