either positively injurious or altogether without effect upon the
course of the disease.
Of drugs, ammonium chloride, salicylic acid, carbolic acid, quinine,
have been administered without positive effect.
It is stated that the free inunction of oil from the very beginning of
the attack was affirmed to exert a favorable influence.[12]
[Footnote 12: See Griesinger, _Virchow's Handbuch der Speciellen
Pathologie und Therapie_, ii. 2, s. 316.]
In early times the buboes were often incised, or even excised, as soon
as they began to swell. More recently they have been treated with
leeches or inunctions of mercurial ointment. The treatment by
poultices and the evacuation of pus as soon as it can be detected is
at present regarded with greater favor. Carbuncles are likewise to be
treated in accordance with accepted surgical procedures.
{785}
LEPROSY.
BY JAMES C. WHITE, M.D.
DEFINITION.--Leprosy is a constitutional disease of chronic course and
fatal termination, characterized by peculiar changes in the tissues of
skin, mucous membrane, nerves, and most organs of the body.
SYNONYMS.--Elephantiasis of Greek writers; Lepra of Arabian authors;
Anssatz (Germany); Spedalskhed (Norway). The local names in use among
the numerous races in which it prevails are too numerous to be given
here.
HISTORY.--Although great confusion has existed among the most ancient
as well as later medical writers with regard to the definition of this
disease, it having been confounded with several other affections
(elephantiasis arabum, syphilis, psoriasis, morphoea, etc.), leprosy
has prevailed in certain parts of the world from the time of the
earliest records. The biblical accounts show that it existed among the
Jews in Egypt, although it was not accurately distinguished from other
diseases resembling it in some respects. It was recognized in Greece
before the Christian era, and in the early centuries after Christ it
had extended widely over Europe. In the seventh and eighth centuries
special leper-houses were founded in Italy, France, and Germany. The
disease reached its height in Europe in the twelfth and thirteenth
centuries, when 19,000 lazarettos are said to have been in existence.
Its spread was greatly increased by the constant intercourse kept up
between Europe and the East during the Crusades. In the fifteenth
century it began to diminish, and in the course of the seventeenth it
had almost wholly disappeared from the most civilized states. It has
lingered, however, in other parts, and exists to-day in France and
Spain and Portugal, in Norway and Sweden, and in Italy, Greece, and
Southern Russia. As in ancient times, it is widely spread along the
coasts of Africa and prevails largely throughout Asia. It is found in
many of the islands of the Indian and Pacific Oceans, in Japan, New
Zealand, Madeira, the West Indies, extensively in some of the states
of Central and South America and Mexico and the Hawaiian Islands.
It may be interesting to trace its history in the United States and
adjacent districts more minutely. It is not known just when leprosy
was introduced into North America. According to the Louisiana
historian, Gayarré, the Spaniards established leper hospitals in
several of their colonies on the Gulf of Mexico during the last
century. One existed in New Orleans as late as 1785. In 1776 the
disease was reported as existing among the blacks in Florida. It seems
to have died out, and with {786} it all remembrance of its former
existence amongst us, until within the last few years, when its
occurrence in the Southern States has again attracted attention. In
Louisiana the first case was discovered in 1866 in an old woman whose
father came from the south of France; she died in 1870. In 1871 it
appeared in one of her sons, in 1872 in two others, and in 1876 in a
nephew. A sixth case developed in a young woman who was in constant
attendance upon the first case. In addition to this group, other cases
have been observed in several parishes, amounting to twenty-one in
all, as collected by Salomon of New Orleans in 1878.[1] Two other
cases, brother and sister, in Louisiana are known to the writer, one
of whom has recently died under his care. In South Carolina the
disease is reported by J. F. M. Geddings[2] to have been observed in
sixteen cases since the year 1846; four were Jews, four negroes, and
eight whites. In none was any hereditary taint to be traced. No new
cases have developed since that report.[3]
[Footnote 1: _New Orleans Med. and Surg. Journal_, March, 1878.]
[Footnote 2: _Trans. Intern. Med. Congress_, Philadelphia, 1876.]
[Footnote 3: See article on "Contagiousness of Leprosy" by writer, in
_Amer. Journ. of Med. Sciences_, Oct., 1882.]
In Minnesota and other North-western States leprosy has been known to
exist for a considerable time among the Norwegian immigrants who have
settled in them in large numbers. Holmboe in 1863 and Prof. Boeck
later made visits to these colonies while in this country, and
published reports concerning them after their return.[4] The latter
found eighteen cases among his countrymen, most of which were leprous
before emigration; in others the disease developed after arrival in
America. It had not manifested itself in any person born in this
country. The character and progress of the affection seem to have been
little influenced by residence here. Since these observations other
cases have been collected by the committee on statistics of the
American Dermatological Association,[5] showing the continuance of the
disease in these States. In 1879 there were fifteen cases in
Minnesota. Its spread in this portion of our country is slow.
[Footnote 4: _British and For. Med.-Chir. Review_, Jan., 1870, and
_Nord. Medic. Ark._, Bd. iii.]
[Footnote 5: See _Transactions_.]
Since 1871, 52 cases of the disease have been inmates of the hospital
for lepers in San Francisco, California. Of these, all, with one
exception, were Chinese, and forty-five of them had been sent back to
China. It is presumed to have shown itself after arrival in this
country, as "unproductive labor would not be imported by the Six
Companies."[6] No case of the disease known to have been acquired in
this country has yet been reported upon the Pacific Coast. One case
has developed in San Francisco after residence in the Hawaiian
Islands.
[Footnote 6: _Trans. Am. Derm. Assoc._, 1881.]
In Oregon, too, the disease has appeared among the Chinese immigrants,
steps having been recently taken to re-ship five lepers from the
poor-farm at Portland to China.
Since 1815, possibly earlier, leprosy has prevailed among the poor
French settlements along the Miramichi River, near the Bay of
Chaleurs, New Brunswick. It was first noticed in a woman whose mother
came from Normandy, and has continued mainly in her descendants since.
No measures were taken to control the disease until 1844, when a
hospital was erected on Sheldrake Island. In 1849 the present
lazaretto at {787} Tracadie was established. During the first five
years (1844-49) there were admitted 32 patients; from 1849 to 1863, 67
additional patients were received; and from the latter date to 1879,
30 more, making a total number of 129 up to the last report. The
greatest number present at any one time was 37. In 1878 there were 16
patients in the lazaretto--6 men and 10 women. The total number of
deaths in the hospital has been, up to 1878, 123. A. C. Smith, who
resides near Tracadie, states that at the latter date but three cases
were known to exist outside the lazaretto. Residence is not
compulsory, and no sufficient measures are taken to remove patients
from their homes before they may have inoculated other members of the
family. The disease is more restricted in locality than formerly.
Within the last two years two or three small groups of the disease
have been discovered in the island of Cape Breton, which are described
in the _Canadian Journal of Med. Science_, Sept., 1881.
These are all the places north of Mexico where the disease exists in
an endemic form. A considerable number of cases have been reported
within the past few years from other parts of the United States, where
it has manifested itself in persons who have formerly resided in
leprous countries or in those who have wandered from the above
infected districts. A very few instances have been recorded in which
it has appeared in those who have never visited any infected locality
or have been in apparent contact with lepers. Such cases, if
authentic, establish the possibility of a sporadic origin of the
affection. The fact of so many foci already established, and the
penetration of a race so prone to the disease as the Chinese into all
parts of the country, give the study of leprosy in America a special
importance.
ETIOLOGY.--The study of the etiology of leprosy is intimately
connected with that of its history and geographical distribution. From
the earliest times it was regarded in all parts of the world as a
contagious affection, and efforts were made by the sternest laws of
Church and State to control its spread by segregation, by interdiction
of marriage, etc. No disease has ever been regarded with an equal
degree of abhorrence by mankind; none has received greater attention
from physicians of every age. Within the present century it has come
to be regarded, almost without exception, by the profession as
non-contagious. Peculiarities of climate, soil, and modes of life have
been looked upon as predisposing, exciting, or even essential
influences in its causation; but the widespread distribution of the
disease, with the consequent diversity of diet and customs of living,
its prevalence upon the coast and in interior regions, in high
altitudes as well as at the sea-level, in Iceland as in the tropics,
show that these conditions, however they may affect the course of the
affection, have no direct relation to its causation. The theory of
heredity, as the most plausible explanation, has received its
strongest support in the investigations of Boeck and Danielssen in
Norway, where the disease can be traced for several generations in
families. The same conclusions readily present themselves where the
disease is studied in restricted localities, as in Louisiana and New
Brunswick at the present time, where, as we have seen, it manifests
itself closely in families in different generations. But this is a
narrow point of view from which to study the etiology of leprosy. It
often fails to manifest itself in the descendants of lepers in {788}
such communities, and affects persons in whose families it has never
previously existed. Moreover, in countries where it does not prevail
it not infrequently attacks individuals who have at some time visited
regions where it was endemic, and in the latter places may develop in
immigrants from parts of the world where it has never existed.
The same class of facts which seem to demonstrate its hereditary
nature may be used in support of its infectious character. The proper
field for observation in this regard would be a virgin region where
its natural course could be studied independently of theories.
Fortunately for science, such an opportunity is afforded in the
history of the disease in the Hawaiian Islands. The exact date and
mode of its introduction there are not definitely known. The islands
have for years been the resort of the whaling-fleets manned by sailors
coming from leprous regions. The natives also shipped as sailors, and
after visiting such ports returned home. The absence of any restraint
in the intercourse of crews and native women is well known. Isolated
cases may have occurred as far back as 1830, but the disease made slow
headway until about 1860, when it increased so rapidly that the
government took stringent measures to control it, all cases discovered
being sent to the leper segregation upon an island from which there is
no escape. Since 1866, 2000 cases have been received there, and at
last report the asylum contained 750 inmates. This by no means
represents the extent of its prevalence in the islands, however. As
the native population by recent census was only 44,000, it will be
seen that the proportion affected is very large. This unwonted
rapidity of spread cannot be accounted for on the ground of heredity.
Transference from individual to individual by inoculation seems to be
the only possible explanation, and all resident physicians believe
that the disease is contagious in this sense. It affects almost
exclusively those of native descent, and their habits of life are such
as would greatly facilitate its wide dissemination in this way--viz.
their great licentiousness and absence of all fear of the disease,
which affords no bar to ordinary association or cohabitation; the
crowding of large families in small huts and sharing the same mats and
blankets; the eating of poi with the fingers from the same dish;
passing a common drinking-vessel or pipe from mouth to mouth, etc.[7]
Promiscuous and compulsory vaccination with impure virus, too, has
been generally practised during recent epidemics of small-pox. It is
evident that abundant opportunity has in many ways been presented for
the inoculation of pus or blood into the circulation from infected to
healthy persons. Where immunity from contraction has followed marriage
with a leper, it may be assumed that the conditions of an abraded
surface and the contact with pus or blood have not been fulfilled. The
wide spread of syphilis among the natives, and a consequent cachexia,
have no doubt contributed to these conditions and established a
national lack of resistance to the ravages of the disease. Nor can we
overlook the proclivity of all endemic diseases to extraordinary
manifestations of virulence in insular nations not previously
protected by gradual inoculation. Many reliable cases are cited by
resident physicians where the evidence of direct communication of the
disease seems to be reliable. Facts of the same nature may be
collected in the study of the history of {789} the disease in New
Brunswick and in Louisiana, where, as above stated, much better fields
for investigating this question exist than in the Old-World regions
where the affection has been rife for centuries.
[Footnote 7: Dr. G. W. Woods, U.S.N., in _Hygienic and Med. Reports_
of Navy Department, vol. iv., 1879.]
If we admit the fact of transference by inoculation in a single
instance, there is no reason why we should not regard this as the
principal if not the only means of extension of the disease, whether
we accept or not the theory of its parasitic nature. It is not
inconsistent with our knowledge of its laws and history to believe
that leprosy is an affection communicated with difficulty, and after a
prolonged period of incubation, from one person to another by contact
with certain products of the diseased tissue; that it has in past and
present time in this way spread from nation to nation; and that its
progress as an endemic affection has been checked only by laws based
upon this theory. All the negative facts so frequently urged against
this doctrine of contagion apply as strongly to that of heredity, and
may be interpreted in support of the former. The latest investigations
into its pathology afford tangible evidence in its favor. It may at
least be claimed that the question of contagion through inoculation
must be reopened.[8]
[Footnote 8: See article on the question of contagion in leprosy in
the _American Journal of Med. Sciences_, Oct., 1882, by the writer.]
Leprosy affects both sexes in about equal degree, and may first show
itself in early childhood. It is apt to produce sterility, so that
marriages between lepers are rarely fruitful. This result seems to
limit the extension of the disease under the law of heredity if we
admit its action. There can be no doubt that cohabitation may take
place for years without communication of the disease where one party
alone is leprous; and such immunity may be explained by the failure of
favorable conditions for sexual inoculation, just as in syphilis. The
disease would naturally be most dangerous in its ulcerative tubercular
form.
SYMPTOMATOLOGY.--There are two well-marked forms of leprosy--viz. the
tubercular and the anæsthetic--which are characterized by certain
easily recognized external manifestations, and which are accompanied
by symptoms indicative of disturbances of the general economy as well
as of special organs. These forms are not always sharply defined, and
often occur simultaneously or in succession in individual cases. Both
are generally preceded by premonitory symptoms, consisting of
unaccountable languor of mind and body, tingling sensations in the
skin, rise of temperature in the evening, and various disturbances of
digestion, or by the occasional outbreak of single or several blebs.
This prodromal stage affords no indication of the type of disease to
follow, and may last for days, months, or even years, with greater or
less intervals and intensity.
TUBERCULAR LEPROSY.--This form may declare itself at once by the
characteristic tubercles, but frequently an earlier manifestation is
the appearance of macules or dull red spots, varying in size from a
pea to two or three inches in diameter. They have an indistinct
margin, a glazed and smooth surface, and become paler on pressure. The
patches, although not at all or but slightly elevated above the
general surface, are firmer, and penetrate more or less deeply into
the cutaneous tissues. They may increase in size peripherally and
undergo involution in the older central portions simultaneously.
During the latter process the color changes from a more or less dull
red to a brown, yellow, or grayish tint, and {790} finally may become
quite white. The spots also become thinner or even slightly depressed.
Their seat is principally the trunk, but also the limbs, and less
frequently the face. This condition of the skin may precede any other
changes in its tissues for months or years, the patches appearing and
disappearing or remaining as permanent stains. At last well-defined
tubercular elevations show themselves, varying in size from a small
shot to a filbert, flattened or semi-globular in form, generally
smooth and firm to the touch, and of a dull red or brown color. They
occur upon any part of the surface, but are especially abundant upon
the face, where they may cause great deformity of the features. The
forehead and eyebrows may become very greatly thickened by general
infiltration, or thrown out into very prominent folds and
protuberances by the massing of individual tubercles. The lips
thicken, the nose broadens, and the ears stand out conspicuously with
their increased bulk. All these changes in form, with the great
darkening in tint which is often present, give at times a most
repulsive expression to the face. The tubercles are sometimes to be
felt imbedded in the skin, or considerable areas are found to be
uniformly thickened and scarcely at all prominent. All forms are
capable of involution after an existence of months, and may leave
dark-colored atrophic patches to mark their seat. They are rarely
painful, and occasionally slightly sensitive. They may be transformed
into ulcers, especially upon prominent positions, as the knuckles,
elbows, knees, as the result of pressure or injury, which are
extremely indolent, although shallow, and may heal and break down
repeatedly. Occasionally they give rise to serious
complications--inflammation of the lymph-vessels, suppuration of the
joints with loss of the attendant members, as the fingers and toes.
Tubercles appear also upon the mucous membrane of the nasal cavities,
the mouth, and larynx, often in great abundance, causing a very
characteristic hoarseness or loss of voice. With these changes in the
cutaneous tissues, which may be accompanied in their periods of
greatest activity by febrile disturbances, there are developed after
months or years, with gradual failure of strength, manifestations of
changes in the internal organs, the lungs, intestines, and brain,
which may prove fatal at any time, or the patient may die of slowly
progressive marasmus. The course of the tubercular form is on the
average between eight and ten years. At any period there may supervene
manifestations of the anæsthetic type, which makes the so-called mixed
variety, in which either form may predominate.
ANÆSTHETIC LEPROSY.--This variety is characterized by the loss of
sensation in the skin over areas of varying extent, which occupy no
definite positions in relation to nerve-distribution. The anæsthetic
patches may appear upon the seat of old maculæ or former tubercles or
of a preceding bullous efflorescence, or upon parts not previously
affected in any way. They may follow a reddened and hyperæsthetic
condition of the cutaneous tissues, or they may be surrounded by a
serpiginous border of this character. The degree of anæsthesia in the
affected parts is sometimes so complete that the skin and underlying
tissues may be deeply pricked or cut or burned without the patient
being aware of the injury. Such patches may possibly regain their
sensibility. Their surface appears in later stages dry, wrinkled,
shrunken, and of a brownish color, and atrophy, not only of the skin
but of the muscles, is gradually developed, {791} in consequence of
which the expression of the face undergoes a marked change. The
eyelids and lips droop, the hair falls, the hands contract, and the
joints of the fingers and toes are laid bare, so that the phalanges,
or even the whole hands and feet, drop off. Ulceration or gangrene of
the parts may develop, and whole extremities may shrivel up. With
these manifestations of local derangements of nerve-action the
functions of the brain fail, the patient becoming stupid and incapable
of action or motion, the temperature and pulse are lowered, and death
comes slowly by marasmus or the most various complications--tetanus,
disease of the lungs, pyæmia, etc. The average duration of this form
is from eighteen to twenty years.