Chapter XLVIII: Part II: , Showing Predominance of Malarial Element . . . . 617 (47)

It is certain, however, that it is incapable of being freely transmitted to great distances in the air. Whether or not it is conveyed or retained by the discharges from the bowel is not known. The history of recently observed outbreaks, from which alone definite and trustworthy facts are to be obtained, goes to show that the exciting cause of the plague clings closely to the patients and their immediate belongings. The closer the relation between those sick and the healthy, the greater the risk that the latter will contract the disease. Those in the house with the patients are more liable to fall sick than those in the adjoining houses--those who are constantly in their presence than those who occasionally see them. Thus, nurses much more frequently contract the plague than doctors, though the latter have in all epidemics been largely numbered among the victims. Among 357 deaths in the outbreak in Vetlanka, already referred to, were a priest, his wife and mother, three doctors, six assistant medical officers, and two Sisters of Mercy. Dr. Cabiadis remarks that the information obtained "shows that the malady propagated itself, in the first instance, from the sick to their relatives and to those who lived with them or who assisted them during their illness. If, on the one hand, these facts showed its contagious character, on the other hand evidence is {777} still wanting to prove whether this transmission of the malady was caused by contact with the sick and their clothing, or by breathing an atmosphere impregnated with the deleterious particles emanating from their morbid bodies."

The period of incubation is from two to seven days. In the report of the commission of the French Academy of Medicine, drawn up by Prus in 1844, the statement appears that the plague has never shown itself among compromised persons after an isolation of eight days. The recent outbreaks tend to confirm this conclusion. L. Arnaud concluded from observations made at Benghazi in 1874 that the mean duration of this period was five or six days, and that the maximum did not exceed eight days. Cabiadis sets this stage down as three days as the rule, but as occasionally not exceeding twenty-four hours. He found no data, however, to show the longest period to which it could extend. Hirsch, from information collected in his investigation of the same epidemic (that of Astrakhan), concluded that the minimum period of incubation observed was from two to three days, the maximum more than eight, and that the average was five days. He states that very short or very long periods were seldom observed.

SYMPTOMATOLOGY.--Individual cases of the plague, as of other epidemic diseases, differ in their onset and progress under different circumstances and at different periods of particular outbreaks. Besides the ordinary form, to which as a type the greater number of the cases more or less closely conform, there are, on the one hand, others so severe that death takes place before the characteristic manifestations have time to appear, and, on the other hand, cases so light that such manifestations are but partly developed, and the nature of the malady is only to be recognized in the light of the prevalent epidemic influence.

Hence among the cases three forms are recognized: (_a_) The grave or ordinary form; (_b_) the fulminant form; and (_c_) the larval or abortive form.

(_a_) Grave or Ordinary Form.--The plague in typical cases is a febrile malady of the most acute kind, with localizations in the form of buboes or carbuncles.

The course of the attack may, for convenience of description, be divided into four stages: 1, the stage of invasion; 2, the stage of intense fever; 3, the stage of fully-developed localizations; and 4, the stage of convalescence.[7]

[Footnote 7: This formal division of the description is suggested in some of the older accounts. (See "_Loimologia; or, An Historical Account of the Plague in London in 1665_, by Nathan Hodges, M.D., and Fellow of the College of Physicians, who resided in the City all that Time, Lond., 1721.")

The appearance of the plague in France in 1720 was the occasion of a great number of curious and interesting publications on this subject.]

1. The stage of invasion is marked by a feeling of lassitude, by pains in the loins and extremities. There is extreme bodily and mental weakness, headache, fulness and throbbing of the head, dizziness. The patient's expression is dull, stupid; he replies to questions slowly or awkwardly, his face is pale, his eyes languid, his gait feeble and staggering. The appearance in this stage has been compared by several observers to that of a drunken man. Shivering occurs, but if fever be present it is slight. Nausea, vomiting, and diarrhoea are symptoms sometimes {778} observed. This stage begins suddenly. It is often imperfectly developed, and it may last only a few hours or a day or two.

2. The second stage is characterized by fever of the most intense kind. It is ushered in by a chill, sometimes slight, commonly severe. The lassitude continues, the headache increases, the dulness deepens to stupor or gives way to delirium. The temperature rises to 102°-104° F., or even to 107.6° F. The pulse quickly mounts to 120 or 130. The skin is hot and dry; the patient complains of burning inward heat and of great, sometimes unbearable, thirst. The eyes are sunken and injected; the tongue moist, pale, and thickly covered with a chalk-white or grayish pasty coating; the vomiting often continues. The delirium is commonly active or noisy, and accompanied by great restlessness; it may, however, be mild, tending to sopor or coma. The progress of the disease now rapidly advances. The patient falls into the so-called typhoid state. His tongue becomes dry, hard, and fissured; sordes collect upon the teeth and lips, bloody crusts about the nostrils. At this time the evidences of failure of the forces of the circulation become conspicuous. The pulse grows feeble, small, often irregular--sometimes it can scarcely be felt; the lips become bluish, the extremities cold. There is tendency to collapse. During the course of this stage buboes begin to make their appearance. Sometimes the enlargement of the superficial lymphatics is preceded by tenderness or pain of more or less intensity; often the glands are found to be enlarged only upon search.

The termination of this stage is marked by a sudden fall of the temperature to subnormal ranges (93.2° F. has been observed); at the same time copious strong-smelling sweat not infrequently occurs. The pulse grows feebler, and falls to 100 or below it, and the mind becomes clearer.

3. These changes lead up to the stage of fully-developed local manifestations. The enlarged lymphatics are most commonly situated in the groins or on the upper part of the thighs at a point below that commonly the seat of venereal buboes; less often they are to be found in the armpits or the region of the angle of the jaw; as a rule, they occupy only one or two of these positions in the same patient. They vary in size from a little mass or kernel, only to be discovered after careful search, to the bulk of a hen's egg or a mandarin orange. The swelling of the gland takes place at times with great rapidity. Suppuration is followed by the discharge of an ichorous pus, and not rarely by ulcerative destruction of the surrounding tissues. Suppuration occurs more frequently than resolution, but is comparatively rare in fatal cases. Hence it has come to be popularly regarded as a favorable prognostic sign, whilst the early subsidence of the swelling has been looked upon as an omen of grave import.

The time of the appearance of the buboes varies greatly. In the greater number of cases they have shown themselves on the second, third, or fourth day of the attack, occasionally within six or eight hours of the beginning of the attack, and occasionally they have been observed to precede the general manifestation of the disease; rarely they have appeared as late as the fifth day. In many cases they are absent altogether.

Carbuncles demand attention as being among the characteristic local manifestations of this stage. They are less common than buboes. Their usual position is upon the lower extremities, the buttocks, or the back of {779} the neck. In favorable cases the gangrene after a few days becomes limited and the slough separates. Boils also occasionally appear.

Petechiæ occur in the worst cases, and often at an early period in the course of the disease. Their appearance usually indicates a fatal issue. They occupy at times extensive areas of the body or the greater part of its surface; at times they appear only in the neighborhood of the buboes. They vary in size from a mere speck to spots several lines in diameter. When very numerous they give a livid hue to the skin, and that appearance to the cadaver to which, together with the high mortality, was doubtless due the term black death by which severe epidemics were known in the Middle Ages.

Vibices and extensive ecchymoses sometimes appear shortly before death.

4. The stage of convalescence sets in between the sixth and tenth days. It is often protracted by prolonged suppuration of the bubonic enlargements. Both relapses and distinct second attacks have been noted by recent as well as the older observers.

In addition to the foregoing sketch of the course of the disease in its ordinary form it is necessary to describe certain other symptoms.

The attack has sometimes begun with a convulsive tremor, at other times with a prolonged shaking, which has lasted from six hours to three days, the patient remaining free from fever and not complaining of cold. This condition has terminated in coma, followed speedily by death.

Sometimes the attack has come upon the patient with great confusion of mind, so that he appears dazed, or else a curious distraction has befallen him in the midst of his ordinary avocations. If absent from home, such patients commonly at once set out to return, either trembling and staggering as though tipsy, or else rushing wildly through the streets with frantic gestures and outcries.

The vomited matters are usually at first gastric mucus with bile, afterward dark coffee-colored fluid; in certain cases blood is vomited. Bleeding from the nose, lungs, bowels, vagina, and urethra have also been observed. Cases attended by hemorrhages have in almost all instances terminated fatally.

Constipation has been, as a rule, present during the acute stages; later in the attack diarrhoea has occasionally occurred. It has been looked upon as a favorable symptom.

The urine has been diminished and suppressed in grave cases. Trustworthy observations, both as to its quantity and its chemical composition, are wanting. It has been observed to contain blood.

As has been already pointed out, the Máhámari of North-western India has been especially characterized by lung symptoms. Other regions also have been visited by epidemics in which acute pulmonary lesions formed a prominent part of the morbid complexus.

(_b_) The Fulminant Form.--Chiefly in the early days or weeks of epidemics, but to some extent also later, cases occur in which the intensity of the sickness is so great that the patient dies before its usual manifestations have time to develop. The duration of the whole attack, which ends fatally, is often not more than a few hours; its symptoms, which differ but little if at all from those of similar cases of other epidemic diseases--such, for example, as epidemic cerebro-spinal fever in its fulminant {780} form--are of the most aggravated character, and the patient perishes overwhelmed by the infection as though struck by a thunderbolt. Profound disturbance of the nervous centres, convulsions, coma, the rapid formation of vibices and petechiæ, collapse, are the speedy forerunners of the fatal issue.

(_c_) The Larval or Abortive Form.--Toward the close of an epidemic the character of the disease usually undergoes a change. It becomes less malignant. The cases present the essential symptoms, but in diminished intensity. Some cases terminate in an early defervescence with rapid subsidence of beginning local manifestations; others present merely the evidences of a slight disturbance of the general health, without any characteristic symptoms of the prevalent disorder; others, again, are characterized by the appearance of buboes without pain or fever. These swellings undergo resolution in fourteen days or thereabout. Exceptionally they suppurate.

The duration of the plague is from six to ten days in typical cases running a favorable course; those of fatal cases from one to twenty days. Clot Bey[8] found the duration of the worst cases two or three days, of those next in point of severity five or six days, whilst in milder cases death did not occur until the second or third week. Of 534 fatal cases noted by W. H. Colvill, 126 occurred one day after the attack, 80 two days after it, 105 three days, 76 four days, 60 five days, 26 six days after the attack. After six days the number of deaths rapidly declined; on the nineteenth day 1 death, and on the twentieth day after the attack 11 deaths, occurred. It is said that death after the seventh day is commonly not in consequence of the disease itself, but of sequels. Of 16 fatal cases in the village Prischib in Astrakhan, noted in the report of Dr. Cabiadis, and of whom the names, as well as the day of their exposure, their falling sick, and their death are given, 1 died in one day, 4 in two days, 6 in three days, 3 in four days, and 2 in six days.

[Footnote 8: _De la Peste observée en Égypte_, Paris, 1840.]

The mortality of the plague is greater than that of any other epidemic disease. In all epidemics a large majority of those who contract the disease die. This is especially true of epidemics at their beginning, when it has often happened that for a time all the cases have perished. Of this, as of other epidemic diseases, it is true that the death-rate has varied in different outbreaks and at different periods of the same outbreak. Colvill states that in the epidemic of 1874 in Mesopotamia the mortality of stricken villages during the first half of the time was 93 to 95 per cent. of those attacked, but that afterward the majority of those attacked recovered. The same authority states that in Bagdad in 1876 the mortality was 55.7 per cent. of persons attacked. Arnauld gives the mortality at Benghazi in 1874 as 39 per cent. of attacks. The death-rate at Vetlanka was 82 per cent. of those attacked. In Toulon in 1721, of a population of about 26,000 human beings, about 20,000 were attacked, and of these 16,000 died. It has been by no means of rare occurrence that nearly half the population of towns have perished in an epidemic, or that small villages have been completely depopulated by this scourge.

COMPLICATIONS AND SEQUELS.--The appalling mortality of the plague on its approach, the rapidity of its spread, the popular commotion upon its appearance, its brief course, and the fact that its recent outbreaks have {781} taken place in regions where trained European physicians have been, with a few exceptions, beyond reach, all unite in maintaining the gloom that has since the Middle Ages enveloped the clinical facts of this disease.

Of its clinical course, beyond the brief outline already given, little is accurately known, of its complications still less. In some of the recent epidemics, and particularly in the outbreaks of plague in India, the evidences of pulmonary lesions have been so conspicuous that they deserve to be classed among the essential manifestations of the disease rather than as complications; in others pulmonary congestion, hæmoptysis, the evidences of croupous or catarrhal pneumonia, have occurred in a small proportion of the cases. Aside from this, there is nothing to be said as to the complications.

Among the known sequels are protracted ulceration of the enlarged lymphatics, boils, superficial or deep abscesses, catarrhal pneumonia, pertussis, mental troubles, and the like. Extensive and deep cicatrices are not infrequently found in the site of the ulcerating local manifestations.

MORBID ANATOMY.--The existing knowledge of the morbid anatomy of the plague is but scanty. The observers of the early outbreaks contributed nothing; the recent outbreaks have taken place under circumstances in which anatomical investigations were impracticable. The knowledge which we possess is almost wholly due to the investigations conducted by the French in Egypt at the close of the last and the beginning of the present century, and again during the years 1833 to 1838.

The descriptions of Bulant,[9] Clot Bey, and others point to gross lesions, such as are found after death in the acute stages of the infectious diseases in general. The viscera were engorged with dark fluid blood; ecchymoses were often found in the mucous and the serous membranes, in the substance of the different organs, and into the connective tissue. The spleen was in almost all cases enlarged, softened, and of a dark color. Not rarely the kidneys were deeply engorged, and extravasations of blood into their substance, their pelves, and into the surrounding connective tissues were often encountered.

[Footnote 9: _De la peste oriental d'apres les matérnaux recuillés à Alexandrie, à Smyrne, etc., pendant les Années 1833 à 1838_, Paris, 1839.]

The only constant and characteristic changes relate to the lymphatic system. The lymphatic glands were, as a rule, enlarged and deeply injected with blood. Where no buboes existed the glands of the various cavities of the body showed evidences of acute inflammatory processes. In some instances the affection of the glands appeared to be general; less frequently it was most conspicuous in, or apparently limited to, one or more great groups. Thus, the bronchial, the mediastinal, the mesenteric, the lumbar, etc. were severally the seat of marked changes with or without enlargement of superficial groups, or several of these groups were at the same time implicated.

In no instance were symmetrical enlargements of the inguinal regions, the axillæ, or the throat met with.

According to Runnel,[10] in 2700 cases there were inguinal buboes in 1841, axillary in 569, maxillary in 231; inguinal buboes occurred 175 times on both sides, 729 times on the right only, 589 times on the left only; the axillary buboes were double 9 times, right only 185, left only {782} 163. Buboes of the neck only occurred 130 times, and of them 67 cases were children.

[Footnote 10: _A Treatise on the Plague_, London, 1791.]

The connective tissue surrounding the affected glands was the seat of an infiltration sometimes serous, sometimes cellular; it also very commonly contained more or less extensive extravasations of blood. Even where no buboes appeared on the surface of the body the glands were enlarged to twice their usual size or more. The substance of the glands in the larger swellings was at times uniformly red or violet, again whitish or marbled or pulpy or denser, or of the consistence of fat. It was also sometimes soft like jelly, and rarely it contained minute collections of pus. Some observers speak of dilatation of the lymph-vessels in the neighborhood of the enlarged glands.

DIAGNOSIS.--The difficulties attending the recognition of the plague at the beginning of an outbreak speedily subside. The rapid spread of the disease, its frightful mortality, the overwhelming intensity of the symptoms, the prompt occurrence of cases characterized by buboes, carbuncles, or petechiæ, are collectively considered diagnostic of this, and of no other disease whatever. In regions subject to the repeated visitations of this pest there exists a universal unwillingness to mention even the name of a disease whose suspected presence alone is followed by consequences of the most serious nature to the freedom of personal and commercial intercourse. To this unwillingness, rather than to any real likeness between the plague and other diseases with which it has been compared, are to be traced most of the difficulties as to the differential diagnosis that have been raised, especially in the regions bordering on the Mediterranean Sea.

It is not, therefore, necessary in this place to discuss the diagnosis between the plague and malarial and other pernicious fevers, malignant typhus, epidemic dysentery, lymphadenitis, syphilitic buboes, parotitis, and so forth.

TREATMENT.--Preventive.--The efficient treatment consists in prophylaxis. The history of this disease indicates with singular clearness the measures which, properly carried out, are capable of controlling the spread of the epidemic diseases. These measures arrange themselves into two groups, of which the first has to do with the removal of the conditions familiar to the development of the disease, the predisposing influences; and the second with the restriction of the disease to the locality in which it shows itself--isolation, quarantine.

The conditions favorable to the development of the plague have already been set forth under the heading Etiology. They relate to poverty and ignorance, and their attendant evils, in communities. They are those conditions which tend to disappear under the influences of civilization, and in truth it may be said that at the present time the plague occurs only in half-civilized countries.

Preventive medicine has achieved no other work comparing in magnitude and importance with the extinction of the plague in Europe. This was, to use the words of Hirsch, "a gradual process, and kept pace in great measure with the development and perfection of the quarantine system with reference to the Orient and the different countries of Europe." This author continues: "I cannot, in fact, understand how any one criticising the facts without prejudice, and having regard to the {783} state of the plague in the East, can for a moment hesitate to attribute the chief cause of the disappearance of the plague from European soil to a well-regulated quarantine system." The European has by no means lost his susceptibility to the disease. He is liable to attack in the East. His protection at home lies in the restriction of the exciting cause of the disease to its present haunts.

Any extended notice of quarantine and quarantine laws is beyond the scope of this article. It may be said, however, that with reference to the plague measures quite unnecessary under ordinary circumstances assume the greatest importance when this disease makes its appearance in countries bordering upon Europe, and that no amount of hardship to individuals necessary to avert so great a calamity as a plague epidemic could be looked upon as excessive. Indeed, we can with difficulty realize the severity with which measures of isolation have been carried into effect at times when the devastation produced by the plague was still vividly remembered. Violation of the orders issued during an epidemic has been punished with no less a penalty than death. It is related that upon the appearance of the plague in the little town of Noja in Lower Italy in 1815, troops were despatched immediately to surround the place with a cordon. The town was encircled by two deep ditches, and opposite the gates three ditches were spanned by drawbridges, which served as a means for the introduction of provisions, but no other communication was allowed. Only letters were allowed to leave the city, and these were first dipped in vinegar. Cannons were posted at the city gates. The ditches were occupied by sentinels, who were ordered to shoot down any one who approached and failed to stand still the moment he was hailed. A plague patient who escaped while delirious and attempted to pass the lines was, in fact, shot dead. Outside this cordon two others were established. Those who disobeyed the orders were treated with the greatest severity. An inhabitant of Noja, who had thrown a pack of cards to the soldiers, together with the soldier who picked it up, was tried by court-martial and shot.[11]

[Footnote 11: _Ueber die Pest zu Noja_, Nürnberg, 1818, quoted by Liebermeister in _Ziemssen's Encyclopedia_, article "Plague."]

Lower Italy, possibly Europe also, owed its escape to the rigorous measures carried out in this instance; nor can it be doubted that the measures of isolation practised during the outbreak on the Volga 1878-79 restricted the disease to the district in which it appeared and brought it to a speedy end. On this occasion three efficient cordons were established to isolate the infected places. The first cordon was put around every place where plague prevailed, to prevent persons from entering or quitting that locality until forty-two days had elapsed after the last attack of the malady there. The second cordon was formed around the infected area, encircling all the infected localities. Its circumference extended 800 kilometres, and was guarded by pickets of soldiers stationed at intervals of five kilometres. This cordon had four quarantine stations. The third and outermost cordon was established round the whole province of Astrakhan. It served to control the functions of the inner cordons, inasmuch as all persons coming from within its area, who could not prove that they had undergone quarantine at the stations of the middle cordon, were stopped.

{784} The complete disinfection of all clothing and other articles used in the service of the sick is to be included among measures of prophylaxis. It is no uncommon thing to destroy by fire the houses in which cases have occurred, along with their contents.

No efficient means of protection are known for those who during an outbreak cannot escape from the infected neighborhood. It would be without purpose other than to amuse the reader to reproduce the quaint fancies of the older physicians in this matter, or to dwell upon the amulets and incantations, the absurd costumes, the protective power of tobacco, according to Diemerhoeck, or the disbelief in its virtues on the part of Hodges, who preferred "canary, of the best sort, of which he frequently drank while he attended the sick."

Clinical.--"The treatment of individual cases must in the present state of knowledge be expectant and symptomatic. Notwithstanding our acquaintance with the symptoms that characterize plague, we are utterly ignorant of the treatment best suited to its cases" (Cabiadis).

Physicians who have written from personal observation unite in advising a treatment of the simplest kind. Ventilation, cleanliness, a liquid diet, abundant cool drinks, are to be ordered. The initial collapse and the evidences of failure of the circulation call for the use of stimulants, and especially of alcohol. Cold or tepid sponging, in accordance with the sensations of the patient, may be resorted to. If there be high fever an energetic antipyretic treatment might be carried out. Cold effusion is said to have been of use in many instances.

Purging, bloodletting, mercurials, blistering, emetics, have proved 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.

PATHOLOGICAL ANATOMY.--The structural changes which take place in the tissues of parts which are the seat of the appearances above described have received the special study of many excellent observers[9] in recent times, and are now well understood. A section through the thickened skin or a tubercle shows the corium and underlying connective tissue infiltrated with round cells, as in lupus and syphilis; in other words, converted into "granulation tissue." This change first takes place along the course of the cutaneous vessels and glands, penetrating more deeply and forming a firmer cell new-growth in proportion to duration, the cells being enclosed in a coarse meshwork of fibrous tissue, and encroaching upon the various structures of the skin, so as to produce atrophy and finally destruction of all its characteristic tissues. This cell-infiltration may of itself undergo later changes, as fatty degeneration and softening (ulceration). The lymph-glands and corpuscles assume a special fatty metamorphosis. An examination of the tubercles upon the mucous membrane reveals the same small-celled new-growth. In the nerve-tissues also marked structural changes are found, both in the central and peripheral systems, in the anæsthetic form of the disease. In many cases the posterior segments of the gray cornua and the fibres of the commissure, as well as the nerves of the extremities, have been found altered by inflammation, which will account for the disordered sensibility and the subsequent disturbances of nutrition, muscular atrophy, etc. The nerve-trunks are often to be felt beneath the skin, thickened and sensitive on pressure. The chronic cell-infiltration affects the fibrous structure of the outer sheath, the neurilemma, and the septa between the nerve-bundles, producing fatty metamorphosis and atrophy of the nerve-bundles. Similar cell-infiltrations are found also in the connective tissue of all the internal organs of the body, which lead to destructive processes in their respective structures.

[Footnote 9: Boeck and Danielssen, _Traité de la Spedalskhed_, Paris, 1848; Virchow, _Die Krankhaften Geschwülste_; Kaposi in _Hebra's Lehrbuch der Hautkrankheiten_; Monasterski, _Vierteljahressch. für Derm. u. Syph._, 1879, p. 203; Hansen, _Virchow's Archiv_, Band 79, 1880; Neisser, _Virchow's Archiv_, Band 84, 1881; Cornil et Souchard, _Annales de Derm. et de Syph._, 1881, No. 4.]

Within the last two years repeated observations have been made which confirm the statement published by Hansen in 1873, that a peculiar bacterium occurs in leprous tissues, which, it is claimed, establishes the parasitic nature of the affection. These examinations have been carried on with leprous material derived from many parts of the world, and the results have been uniform. Within the round cells which characterize the cutaneous neoplasms, both in the distinct tubercles and the diffused {792} infiltrations, small agglomerations of minute rod- or staff-like bodies (bacilli) are found, arranged in parallel rows or placed end to end. Their length is one-half or three-fourths the diameter of a red blood-globule, and their breadth is one-fourth their length. With them minute granular particles are seen in the cells. They occur in greatest numbers in the cells of the upper layers of the true skin, which are considerably swollen by their presence. They never penetrate the epithelial layer, nor are they found in epithelial cells in any position. When the protoplasm of the cell is interfered with by the later tissue-changes of the disease, the bacillus perishes. They are found not only in the leprous cells, but also in those of the connective tissue running between the agglomerated masses of the former. Between the leprous cells and the filaments of connective tissue but few free bacilli are seen. The neoplasms of the mucous membrane and of many organs of the body have been found to contain them also. In the blood they have been detected by some observers. Their presence in the nerve-tissues is of importance as throwing light upon the question of the specific or inflammatory nature of the morbid processes above described as affecting them. If we regard the bacteria as pathognomonic of leprous tissue-changes, their occurrence, recognized in the cells penetrating between the fibres of the peripheral nerves, would seem to make all primary structural changes identical, and the anæsthetic as much as the tubercular form the direct result of their presence. Neisser draws the following conclusions from his investigations: "Leprosy is a real bacterial disease, caused by a special kind of bacterium. The bacilli appear in the tissues as such, or more probably as spores, and remain for a longer or shorter time in a state of incubation, according to circumstances, in dépôts, perhaps in the lymph-glands. This period, much longer than in other infective diseases, is in proportion to the physiological resistance of the human organism compared with the feeble developing power of the bacilli. It, as well as the course of the disease, is more rapid in tropical countries than in Europe. From these dépôts the disease extends throughout the body in those portions of the skin most exposed, the face, hands, elbows, knees, and into the peripheral nerves. The other organs are less freely invaded. The bacilli excite inflammation, and by a specific action transform the migrating cell into the leprous cell. Leprosy is probably an infectious disease, and its specific products are contagious--viz. the leprous cells of the tubercles, the tissue-fluids, and the pus containing bacilli or viable spores. On the other hand, the pus may not always be infectious, as the fluid contained in the bullæ is not."