this question in the affirmative without considerable qualification. It
must be confessed that we do not possess a knowledge of any definite
chain of morbid processes constituting a distinct disease that may be
designated as purpura. And yet we are able to recognize a set of
symptoms varying greatly in intensity, from the most trivial petechial
eruption to profuse and fatal hemorrhages, accompanied by a train of
manifestations which we are unable to connect with any of the causes
already spoken of, and which, indeed, depend upon no fixed exciting
cause with which we are acquainted. It may be eventually proven that
purpura, even as we understand it, is merely a set of phenomena due to
widely-differing influences acting upon the blood and blood-vessels,
and that the term will disappear from our nomenclature as indicating a
disease, but will be preserved as denoting a symptom. For the present,
{187} purpura is understood to be a group of symptoms characterized by
the effusion of blood into the tissues of the body, or upon its free
surfaces, or into its serous cavities, which seem to arise
spontaneously, and for which we are unable to assign a definite cause.
With this view of the nature of purpura it becomes necessary to exclude
from present consideration blood-extravasations from internal or
external violence, the action of the specific principles of contagious
or infectious fevers, the dyscrasia of scurvy, the influence of
poisonous substances, and, in a word, any of those affections of which
the escape of blood from the vessels constitutes an epi-phenomenon.
Purpura may be conveniently considered as presenting three varieties:
1, purpura simplex; 2, purpura hæmorrhagica; 3, purpura rheumatica.
These three forms of the disease are not distinguished by
sharply-outlined differences, but merge the one into the other, now
one, now another set of symptoms predominating. To these may be added,
likewise for convenience, three sub-varieties--purpura urticans,
purpura papulosa, and purpura nervosa. The difference between these
forms of purpura should not be considered as of more than clinical
import. Whatever variations present themselves may with probable
propriety be ascribed to complicating influences.
PURPURA SIMPLEX.--This is the mildest form of purpura, and may in many
cases readily escape observation. It may begin abruptly, in the midst
of health, without the slightest subjective symptom, or the
extravasations may be preceded for several days by some discomfort,
aching of limbs, sluggishness, anorexia, even a small amount of fever.
The eruption usually appears first upon the lower extremities,
preferably the flexor surfaces of the thighs (Duhring), but frequently
upon the legs. It extends from these points to the upper extremities
and trunk, usually sparing the face. The lesions vary in size from that
of a pin-head to that of a fingernail (petechiæ), or they may be linear
(vibices). They remain discrete, and do not increase in size throughout
their course. Each spot of hemorrhage will endure for from one to two
weeks. At first the lesions are of a livid red color, and declare their
extra-vascular nature by remaining unaltered when subjected to
pressure. The color of these spots changes, as in ordinary ecchymosis,
in consequence of the metamorphoses of the hæmatin preparatory to its
final absorption, from crimson to purple, to blue, to green, to yellow,
and finally fades away. When recent, the spots appear sharply outlined,
with sometimes a faint encircling zone of hyperæmia, but as they become
older their margins grow indistinct. While the early lesions slowly
disappear, others continue to develop, and the affection may thus be
protracted for weeks. At times the petechiæ appear in crops, recurring
every few days, the patient at one time apparently nearly well, at
another time worse than ever. Finally, the symptoms definitely
disappear, to return no more, or they pass into those of other forms of
purpura. During the course of purpura simplex the blood-vessels of the
skin alone are affected, the deeper tissues and mucous membranes
probably remaining unchanged.
Throughout the attack the general health may--usually does--remain
good. As an occasional symptom there will be observed a few vesicles or
blebs, containing blood, upon the skin. The extent of the general
eruption may vary from a few scattered petechiæ to a copious and
startling {188} number of purpuric spots. The maintenance of the
upright position tends to perpetuate the evolution of the lesions.
In elderly persons purpura simplex is sometimes observed, and has been
described by many writers as purpura senilis. Hillier, following
Bateman, describes it as occurring in old women "upon the outside of
the forearms in successive dark, purple blotches of an irregular form
and various magnitude."[1] Aged men as well as women are liable to the
affection, which may quite as well appear upon the lower extremities of
either sex. It is altogether likely, however, that in such cases
degenerations of the vascular walls alone may cause the extravasations.
[Footnote 1: Reynolds's _System of Medicine_, vol. i. p. 792.]
PURPURA HÆMORRHAGICA (MORBUS MACULOSUS WERLHOFII).--In this form of
purpura there are added to the symptoms of purpura simplex hemorrhages
into and from the various mucous tracts, the nasal, faucial,
pharyngeal, gastric, intestinal, renal, uterine, rarely the pulmonary
mucous membranes, and exceptionally into the various serous membranes
and cavities. It may begin abruptly, in the midst of apparently
vigorous health, or after premonitory symptoms extending over several
days, vague sensations of discomfort--headache, pains, anorexia,
indisposition to exertion, and the like--or it may occur as a
transition from other forms of purpura. Usually there is no fever.
The hemorrhagic spots upon the skin appear much as in purpura simplex,
though the lesions are larger, acquiring the size of coins or even of
the palm of the hand. Spots soon appear upon the visible mucous
membranes, and free hemorrhages occur; indeed, the latter may be the
first symptom observed. Epistaxis is of most common occurrence, but
bleeding from the mouth, stomach, and intestines almost as frequently
results. The gums are almost constantly affected, and upon inspection
these may be found covered with blackish scabs, upon removal of which
the mucous membrane will be found pale and not swollen--an important
point in diagnosticating this affection from scurvy. Vesicles and blebs
filled with blood form both on the skin and mucous membranes. They
quickly rupture and discharge their contents. Bleeding from the stomach
and intestines is revealed--in the former case by the vomiting of a
brownish material resembling coffee-grounds; in the latter case by the
passage of black, tar-like evacuations. Pulmonary hemorrhage is to be
distinguished from hæmatemesis by the frothy and arterial character of
the blood. Hæmaturia may proceed from any part of the urinary tract.
Bleeding from several parts may occur at the same time, and may be very
copious.
In the mucous membranes extravasations of greater or less extent may
occur, as in the derma. Into the serous membranes they may take place
with or without effusion into serous cavities. It is only, however, in
cases that will almost certainly end fatally that the effusions into
these cavities are encountered. Hemorrhages into the substance of the
lungs, into the brain and other viscera, as well as into the tissues
generally, are occasionally observed.
At the outset of these bleedings the general health of the patient may
appear unimpaired, and if they be few in number and moderate in extent
but slight evidences of debility may be shown throughout the attack;
but it is often the case that the loss of blood is excessive and long
continued, and symptoms of profound anæmia supervene. The {189} patient
becomes greatly exhausted; intense pallor is developed, shortly
followed by general oedema. Attacks of syncope appear, and in fatal
cases--which are not common--death results from asthenia. This result
may occur after a few days from the profuseness of the hemorrhage;
usually, however, only after several weeks. Throughout the attack the
cutaneous lesions continue to develop, either irregularly or in
successive outbreaks, scattered over the general surface, involving the
face less frequently than other parts. These spots undergo the
color-changes peculiar to extravasated blood, and may be seen in all
the stages of involution in the same patient. Fever, usually absent
throughout the attack, may appear at the height of the affection, but
does not run high. Local inflammations are exceedingly rare. In
favorable cases recovery follows the gradual mitigation and
disappearance of the symptoms, but relapses frequently occur, and
convalescence may be retarded for months.
PURPURA RHEUMATICA (PELIOSIS RHEUMATICA).--Schoenlein in 1829 described
as peliosis rheumatica an affection in which the symptoms of purpura
simplex were associated with pain and often with effusion into the
joints, especially those of the knee and ankle. He considered it as an
independent malady. This opinion has been shared by Fuchs, Hebra,
Kaposi, Neumann, and many others. Kaposi[2] regards it as related to
erythema nodosum, with which affection, indeed, it possesses some
features in common. It probably, however, constitutes a complication of
ordinary purpura. That it is not primarily rheumatic is shown by the
almost invariable absence of many of the symptoms characteristic of
rheumatism; that it cannot be an independent affection appears from its
intimate relations with other forms of purpura.
[Footnote 2: _Hautkrankheiten_, 1880, p. 277.]
Purpura rheumatica commonly begins with malaise, anorexia, debility,
sometimes with mild fever. The patient is soon attacked with pains, of
a more or less acute character, in the joints, especially the knees and
ankles. There may be some effusion into the joint and cutaneous oedema.
After a few days the nature of the complaint will be revealed by an
eruption of petechiæ, first near the painful joints, but soon
extending, involving in many cases even the head and trunk. The
eruption may be at first slightly elevated and surrounded by a fine
halo of hyperæmic injection.
The pains usually subside upon the appearance of the eruption, and the
malady may be completed after a single outbreak. More commonly new
joint-pains are experienced, fresh crops of petechiæ appear, and the
trouble may be prolonged for weeks, even months, the patient meanwhile
suffering not very greatly in general health. The lesions may be
cutaneous only; rarely bleeding from mucous surfaces will occur
(Scheby-Buch). Albuminuria may be present (Kaposi). An annual type is
said by Kaposi, Neumann, and others to be sometimes observed, the
spring and autumn being the usual seasons for the outbreaks. This is
supposed to indicate a relationship with erythema nodosum and
multiforme. Cardiac murmurs have been detected in the course of purpura
rheumatica,[3] but these were probably anæmic or antedated the purpuric
symptoms. Purpura rheumatica never seems to result in endo- or
pericarditis.
[Footnote 3: Kinnicutt, _Archives of Dermatology_, i. p. 193; Mollière,
_Ann. de Dermatol._, v. p. 44.]
SUB-VARIETIES.--Henoch[4] and Couty[5] have described a form of {190}
purpura mostly observed in children, in whom rheumatoid pains occur
along with colic and vomiting of greenish or bilious matter, tenesmus,
and sometimes with loss of blood from the bowels. The disease may be
protracted throughout months by relapses. Cutaneous oedema frequently
occurs. Couty regards it as a form whose peculiarities justify its
assignment to a position of its own. The cause of the associated train
of symptoms is supposed (Couty) to reside in the sympathetic system,
and the name purpura nervosa is proposed for it. So many features of
ordinary purpura are manifested in these cases that it seems better to
consider them as examples of ordinary purpura complicated with
gastro-intestinal derangement. It has been suggested that the nausea,
vomiting, and abdominal pains may result from extravasation of blood
into the peritoneal tissue.[6]
[Footnote 4: _Berl. Klin. Wochenschr._, 51, 1874.]
[Footnote 5: _Gaz. Hebd._, 36 _et seq._, 1876.]
[Footnote 6: Immermann, _Ziemssen's Cyclopæd._, vol. xvii. p. 265.]
In the course of purpura there is frequently observed, more especially
in purpura simplex, a wheal-like arrangement of the eruption--such,
indeed, as occurs in urticaria. The term purpura urticans has been
given to this sub-variety, which may or may not be accompanied by
itching. Scheby-Buch has suggested that the urticaria may, with more
propriety, be attributed to the gastric disturbances that so often
accompany the forms of purpura presenting it.[7] The wheals are usually
seen upon the lower extremities, but may appear elsewhere. A
considerable degree of oedema may be present, particularly in lax
tissue, such as that of the scrotum, eyelids, etc.
[Footnote 7: _Deutsche Arch. f. Klin. Med._, B. xiv. p. 490.]
Purpura papulosa (lichen lividus, Willan) is a form of purpura where,
in the midst of ecchymoses, livid papules appear. These probably depend
upon a large amount of hemorrhage occurring within a limited space,
most often surrounding the orifices of hair-follicles, because these
are supplied with a capillary network that comes directly from the
deeper layer.[8] They are formed most abundantly on the legs of
scrofulous, cachectic persons who have purpura. Care must be taken to
distinguish this form of purpura from erythema multiforme and erythema
nodosum, where blood is usually extravasated secondarily into the
tissues. Those cases only where the purpura is primary should be
recognized as purpura papulosa.
[Footnote 8: Hebra, _Skin Diseases_, New Syd. Soc. Transact., ii. p.
425.]
The purpuric effusion appears to act as an irritant upon the tissues,
and to excite inflammation. Gangrene of the mucous coat of the
intestines has resulted from extensive hemorrhagic extravasations, and
from a similar cause cutaneous gangrene has been known. These
complications, however, are rare.
ETIOLOGY.--The immediate causes of purpura are quite unknown. Both
sexes and persons of every age are affected by it. While it is most
often seen in debilitated subjects, those in vigorous health possess no
immunity. It has often been observed during convalescence from other
maladies. It cannot be said that those who are miserably clothed, fed,
and lodged are especially predisposed to attacks of purpura. Between
purpura and hæmophilia, etiologically, there are many points of
difference. Purpura is not hereditary, nor is there a purpuric
diathesis in the strict sense of the term. Some persons, indeed, seem
to possess a {191} predisposition to the disease, and some authors
claim for purpura rheumatica a distinct annual type. This, however, is
not at all certain.
Recently it has been claimed that purpura hæmorrhagica depends upon the
presence of a minute organism in the blood. Petrone[9] injected blood
drawn from patients with this disease under the skin of rabbits,
producing widely-distributed hemorrhages. In the blood of these
individuals and of the injected rabbits micrococci and bacilli were
detected. Watson Cheyne[10] also describes a plugging of the
capillaries with bacilli. These were 1/7700 of an inch in length and
1/20000 of an inch in diameter, and were arranged in colonies. In
another case there were found micrococci arranged in chains. These
swarmed in the capillaries and some larger vessels, and sometimes
completely blocked them. Although an origin in infection has thus been
claimed for purpura hæmorrhagica, the fact that more than one variety
of micro-organism was observed cannot fail to excite suspicion of,
possibly, erroneous observation.
[Footnote 9: _Lo Sperimentale_, 51, 1883.]
[Footnote 10: _Lancet_, i., 1884, 344.]
PATHOLOGY.--In the foregoing description those extravasations of blood
due to simple mechanical violence, as from flea-bite, and sudden
increase of blood-pressure, as in the effort of coughing in whooping
cough, also from the deleterious influence exerted upon the
blood-vessels and blood by certain drugs, the specific fevers, Bright's
disease, and the like, have been excluded. Only those have been
considered where the effusion of blood seemed to occur spontaneously,
and the symptoms to result from some peculiar but not understood morbid
process. The hemorrhage is but a symptom; the process by which it is
brought about depends upon some change in the blood or blood-vessels.
We do not know what these subtle changes are. The blood of purpuric
patients has been carefully examined, but, with the exception above
mentioned, no definite changes have been discovered. Immermann[11]
found during the first stage of the disease the blood-corpuscles
perfectly normal in appearance, the white corpuscles subsequently
slightly exceeding the red in number--a simple result of copious
hemorrhage. No stated chemical changes in the blood are known in
purpura, nor is it known how the blood escapes from the vessels. It
undoubtedly escapes through alterations in the vascular wall, but it is
also true that red blood-corpuscles, as well as the pale ones, may find
their way in considerable numbers through the unruptured wall of the
vessels, per diapedesin, as was first suggested by Velpeau, but
definitely determined by Stricker. The causes of this migration are
obscure. Immermann[12] asserts that a fatty degeneration of the
vascular tissues and of the muscles takes place. This, however, is
manifestly a result of the loss of blood, and not its cause. Dr. Wilson
Fox[13] found extensive albuminoid disease of the muscles and
capillaries of the skin; but the albuminoid degeneration involved
several organs of a patient with syphilis, and the purpura was
certainly secondary to the morbid conditions. Rigal and Cornil[14]
think that the hemorrhages are a result either of sympathetic
irritation or of diminished action of the vaso-motor centre. It is
indeed altogether likely that the cause will ultimately be found to
reside in the vaso-motor system.
[Footnote 11: _Ziemssen's Cyclop._, xvii. p. 258.]
[Footnote 12: _Loc. cit._]
[Footnote 13: _Brit. and Foreign Med.-Chir. Review_, Oct., 1865.]
[Footnote 14: _L'Union Méd._, 5, 6, 7, 1880.]
{192} DIAGNOSIS.--The affection bearing the closest resemblance to
spontaneous purpura is scurvy; indeed, its supposed relationship to
this disease has given purpura one of its synonyms, land scurvy. The
two affections, however, are probably without the slightest
relationship. They possess in common the hemorrhagic symptoms, both in
the tissues and from free surfaces, but the resemblance does not extend
much beyond this. Scurvy depends upon deprivation of fresh vegetable
food and the use of unsuitable and insufficient food generally, and
upon bad hygienic surroundings. Purpura may--frequently does--appear in
broken-down constitutions, but it equally attacks the strong and
vigorous, while the character of food exerts no special influence on
its production. Scurvy only follows long-continued privations and as a
culmination of a train of distressing symptoms. Purpura appears in the
midst of health, or after brief premonition, or during convalescence
from totally unrelated diseases. In scurvy there is a decided tendency
toward ulceration, which is absent in purpura. In scurvy the mouth and
gums inflame and ulcerate, the latter becoming swollen, spongy, and of
a bluish-red color. In purpura, ulceration of the buccal mucous
membrane does not occur, and the gums are pale and intact. The curative
influence of fresh vegetables, lime-juice, etc. in the treatment of
scurvy is not observed in purpura. It has been claimed that purpura is
but a mild degree of scurvy: this cannot be so, for we may have a mild
scurvy or a severe, even fatal, purpura.
The hemorrhagic diathesis, or hæmophilia, presents points of analogy
with purpura. Here, however, is found the almost constant history of
heredity and the implication only of persons of the male sex. The
disposition to bleed at all times upon the receipt of the smallest
injury is quite unlike the suddenly-developed and transitory
hemorrhages of purpura, which are also more generally distributed.
With the secondary hemorrhagic effusions and ecchymoses that occur in
conditions of profound alterations of the blood and blood-vessels in
cases of malignant small-pox, scarlatina, typhus fever, etc., and in
some cases of poisoning, as from phosphorus, spontaneous purpura
presents identities, but the history of the complaint and the condition
of the patient will prevent error. A knowledge of the circumstances
will serve to distinguish purpura simplex from the petechiæ and small
ecchymoses produced by fleas, by diminished atmospheric pressure, by
coughing, in the course of Bright's disease, etc.
Purpura rheumatica presents, as has been shown, many points of
resemblance to erythema multiforme and erythema nodosum. The mild
fever, the joint-pains, the extravasations of the latter affections,
are much like the symptoms of this form of purpura. The nodular,
inflamed, tender condition of the lesions, their location--frequently
upon the extensor surfaces of the extremities--their course and
duration, usually serve to identify erythema nodosum, while with
erythema multiforme it is usually not difficult to observe its
essentially inflammatory character. Scheby-Buch has shown the
difficulties often opposed to the differentiation of purpuric lesions
and ecchymoses due to violence.[15] Where the petechial eruption of
purpura simplex is well marked, where the internal hemorrhages of
purpura hæmorrhagica are copious, the inquiries of the observer will
usually lead him to correct conclusions. Where the {193} ecchymoses are
larger and upon exposed parts of the body, the diagnosis from the
lesions alone becomes impossible, and due consideration of all
concomitant circumstances is essential. It should be remembered that in
purpura very slight violence may call forth extensive ecchymosis. This
circumstance has important medico-legal bearings.
[Footnote 15: _Viertelj. f. Dermatol. und Syph._, 1879, p. 99.]
PROGNOSIS.--Purpura usually terminates favorably. Its course runs from
two to six weeks, rarely longer. Relapses and remissions are frequent.
Purpura simplex is of very little gravity, and need excite little
apprehension. Purpura rheumatica almost always ends in recovery; fatal
terminations, however, have been known. Purpura hæmorrhagica is of much
more serious import. Even here, however, though the patient may fall
into profound debility from loss of blood, recovery is the rule, the
symptoms gradually diminishing in severity until health becomes
re-established. In fatal cases death ensues after prolonged and profuse
losses of blood. Purpura may subside after a single outbreak or many
relapses, and recrudescences may occur extending through months. Anæmia
may persist long after the disappearance of purpuric symptoms. A
tendency to purpura may be shown at irregular intervals for years, and
even throughout life.
TREATMENT.--Very mild cases of purpura simplex require no treatment,
not even confinement within doors. The patient is often first made
aware of his disease by accident; doubtless it frequently escapes
detection altogether. It has been observed that purpura often appears
upon the lower limbs of convalescents from other diseases when they
first essay the upright position. Relapses of purpura also frequently
appear as the patient leaves his bed. We have here an important
indication for treatment--viz. the maintenance of the recumbent posture
in cases of any degree of severity. Fresh vegetables and vegetable
acids do not have the same happy influence as in scurvy. It is
manifestly important that appropriate food should be administered in
sufficient quantity, both to improve the general health and to repair
the exhausting losses of blood. Milk is an exceedingly valuable article
of diet in these cases, being but little apt to irritate the mucous
membrane of the alimentary canal.