Leprosy

Leprosy (Lepra Arabum, Elephantiasis Graecorum, Aussatz, Spedalskhed), the greatest disease of medieval Christendom, identified, on the one hand, with a disease endemic from the earliest historical times (1500 B.C.) in the delta and valley of the Nile, and, on the other hand, with a disease now common in Asia, Africa, South America, the West Indies, and certain isolated localities of Europe. An authentic representation of the leprosy of the middle ages exists in a picture at Munich by Holbein, painted at Augsburg in 1516; St Elizabeth gives bread and wine to a prostrate group of lepers, including a bearded man whose face is covered with large round reddish knobs, an old woman whose arm is covered with brown blotches, the leg swathed in bandages through which matter oozes, the bare knee also marked with discoloured spots, and on the head a white rag or plaster, and, thirdly, a young man whose neck and face (especially round the somewhat hairless eyebrows) are spotted with brown patches of various size. It is conjectured by Virchow that the painter had made studies of lepers from the leper-houses then existing at Augsburg. These external characters of medieval leprosy agree with the descriptions of it by the ancients, and with the pictures of modern leprosy given by Danielssen and Boeck for Norway, by various authors for sporadic European cases, by Anderson for Malacca, by Carter for India, by Wolff for Madeira and by Hillis for British Guiana. There has been some confusion in the technical naming of the disease; it is called Elephantiasis (Leontiasis, Satyriasis) by the Greek writers, and Lepra by the Arabians.

Leprosy is now included among the parasitic diseases (see Parasitic Diseases). The cause is believed to be infection by the bacillus leprae, a specific microbe discovered by Armauer Hansen in 1871. It is worthy of note that tuberculosis is very common among lepers, and especially attacks the serous membranes. The essential character of leprosy is a great multiplication of cells, resembling the “granulation cells” of lupus and syphilis, in the tissues affected, which become infiltrated and thickened, with degeneration and destruction of their normal elements. The new cells vary in size from ordinary leucocytes to giant cells three or four times larger. The bacilli are found in these cells, sometimes in small numbers, sometimes in masses. The structures most affected are the skin, nerves, mucous membranes and lymphatic glands.

The symptoms arise from the anatomical changes indicated, and they vary according to the parts attacked. Three types of disease are usually described—(1) nodular, (2) smooth or anaesthetic, (3) mixed. In the first the skin is chiefly affected, in the second the nerves; the third combines the features of both. It should be understood that this classification is purely a matter of convenience, and is based on the relative prominence of symptoms, which may be combined in all degrees. The incubation period of leprosy—assuming it to be due to infection—is unknown, but cases are on record which can only be explained on the hypothesis that it may be many years. The invasion is usually slow and intermittent. There are occasional feverish attacks, with the usual constitutional disturbance and other slight premonitory signs, such as changes in the colour of the skin and in its sensibility. Sometimes, but rarely, the onset is acute and the characteristic symptoms develop rapidly. These begin with an eruption which differs markedly according to the type of disease. In the nodular form dark red or coppery patches appear on the face, backs of the hands, and feet or on the body; they are generally symmetrical, and vary from the size of a shilling upwards. They come with one of the feverish attacks and fade away when it has gone, but only to return. After a time infiltration and thickening of the skin become noticeable, and the nodules appear. They are lumpy excrescences, at first pink but changing to brown. Thickening of the skin of the face produces a highly characteristic appearance, recalling the aspect of a lion. The tissues of the eye undergo degenerative changes; the mucous membrane of the nose and throat is thickened, impairing the breathing and the voice; the eyebrows fall off; the ears and nose become thickened and enlarged. As the disease progresses the nodules tend to break down and ulcerate, leaving open sores. The patient, whose condition is extremely wretched, gradually becomes weaker, and eventually succumbs to exhaustion or is carried off by some intercurrent disease, usually inflammation of the kidneys or tuberculosis. A severe case may end fatally in two years, but, as a rule, when patients are well cared for the illness lasts several years. There is often temporary improvement, but complete recovery from this form of leprosy rarely or never occurs. The smooth type is less severe and more chronic. The eruption consists of patches of dry, slightly discoloured skin, not elevated above the surface. These patches are the result of morbid changes affecting the cutaneous nerves, and are accompanied by diminished sensibility over the areas of skin affected. At the same time certain nerve trunks in the arm and leg, and particularly the ulnar nerve, are found to be thickened. In the further stages the symptoms are those of increasing degeneration of the nerves. Bullae form on the skin, and the discoloured patches become enlarged; sensation is lost, muscular power diminished, with wasting, contraction of tendons, and all the signs of impaired nutrition. The nails become hard and clawed; perforating ulcers of the feet are common; portions of the extremities, including whole fingers and toes, die and drop off. Later, paralysis becomes more marked, affecting the muscles of the face and limbs. The disease runs a very chronic course, and may last twenty or thirty years. Recovery occasionally occurs. In the mixed form, which is probably the most common, the symptoms described are combined in varying degrees. Leprosy may be mistaken for syphilis, tuberculosis, ainhum (an obscure disease affecting negroes, in which the little toe drops off), and several affections of the skin. Diagnosis is established by the presence of the bacillus leprae in the nodules or bullae, and by the signs of nerve degeneration exhibited in the anaesthetic patches of skin and the thickened nerve trunks.

In former times leprosy was often confounded with other skin diseases, especially psoriasis and leucoderma; the white leprosy of the Old Testament was probably a form of the latter. But there is no doubt that true leprosy has existed from time immemorial. Prescriptions for treating it have been found in Egypt, to which a date of about 4600 B.C. is assigned. The disease is described by Aristotle and by later Greek writers, but not by Hippocrates, though leprosy derives its name from his “lepra” or “scaly” disease, which was no doubt psoriasis. In ancient times it was widely prevalent throughout Asia as well as in Egypt, and among the Greeks and Romans. In the middle ages it became extensively diffused in Europe, and in some countries—France, England, Germany and Spain—every considerable town had its leper-house, in which the patients were segregated. The total number of such houses has been reckoned at 19,000. The earliest one in England was established at Canterbury in 1096, and the latest at Highgate in 1472. At one time there were at least 95 religious hospitals for lepers in Great Britain and 14 in Ireland (Sir James Simpson). During the 15th century the disease underwent a remarkable diminution. It practically disappeared in the civilized parts of Europe, and the leper-houses were given up. It is a singular fact that this diminution was coincident with the great extension of syphilis (see Prostitution). The general disappearance of leprosy at this time is the more unintelligible because it did not take effect everywhere. In Scotland the disease lingered until the 19th century, and in some other parts it has never died out at all. At the present time it still exists in Norway, Iceland, along the shores of the Baltic, in South Russia, Greece, Turkey, several Mediterranean islands, the Riviera, Spain and Portugal. Isolated cases occasionally occur elsewhere, but they are usually imported. The Teutonic races seem to be especially free from the taint. Leper asylums are maintained in Norway and at two or three places in the Baltic, San Remo, Cyprus, Constantinople, Alicante and Lisbon. Except in Spain, where some increase has taken place, the disease is dying out. The number of lepers in Norway was 3000 in 1856, but has now dwindled to a few hundreds. They are no longer numerous in any part of Europe. On the other hand, leprosy prevails extensively throughout Asia, from the Mediterranean to Japan, and from Arabia to Siberia. It is also found in nearly all parts of Africa, particularly on the east and west coasts near the equator. In South Africa it has greatly increased, and attacks the Dutch as well as natives. Leper asylums have been established at Robben Island near Cape Town, and in Tembuland. In Australia, where it was introduced by Chinese, it has also spread to Europeans. In New Zealand the Maoris are affected; but the amount of leprosy is not large in either country. A much more remarkable case is that of the Hawaiian Islands, where the disease is believed to have been imported by Chinese. It was unknown before 1848, but in 1866 the number of lepers had risen to 230 and in 1882 to 4000 (Liveing). All attempts to stop it by segregating lepers in the settlement of Molokai appear to have been fruitless. In the West Indies and on the American continent, again, leprosy has a wide distribution. It is found in nearly all parts of South and Central America, and in certain parts of North America—namely, Louisiana, California (among Chinese), Minnesota, Wisconsin and North and South Dakota (Norwegians), New Brunswick (French Canadians).

It is difficult to find any explanation of the geographical distribution and behaviour of leprosy. It seems to affect islands and the sea-coast more than the interior, and to some extent this gives colour to the old belief that it is caused or fostered by a fish diet, which has been revived by Mr Jonathan Hutchinson, but is not generally accepted. Leprosy is found in interiors where fish is not an article of diet. Climate, again, has obviously little, if any, influence. The theory of heredity is equally at fault, whether it be applied to account for the spread of the disease by transmission or for its disappearance by the elimination of susceptible persons. The latter is the manner in which heredity might be expected to act, if at all, for lepers are remarkably sterile. But we see the disease persisting among the Eastern races, who have been continuously exposed to its selective influence from the earliest times, while it has disappeared among the Europeans, who were affected very much later. The opposite theory of hereditary transmission from parents to offspring is also at variance with many observed facts. Leprosy is very rarely congenital, and no cases have occurred among the descendants to the third generation of 160 Norwegian lepers settled in the United States. Again, if hereditary transmission were an effective influence, the disease could hardly have died down so rapidly as it did in Europe in the 15th century. Then we have the theory of contagion. There is no doubt that human beings are inoculable with leprosy, and that the disease may be communicated by close contact. Cases have been recorded which prove it conclusively; for instance, that of a man who had never been out of the British islands, but developed leprosy after sharing for a time the bed and clothes of his brother, who had contracted the disease in the West Indies. Some of the facts noted, such as the extensive dissemination of the disease in Europe during the middle ages, and its subsequent rapid decline, suggest the existence of some unknown epidemic factor. Poverty and insanitation are said to go with the prevalence of leprosy, but they go with every malady, and there is nothing to show that they have any special influence. Vaccination has been blamed for spreading it, and a few cases of communication by arm-to-arm inoculation are recorded. The influence of this factor, however, can only be trifling. Vaccination is a new thing, leprosy a very old one; where there is most vaccination there is no leprosy, and where there is most leprosy there is little or no vaccination. In India 78% of the lepers are unvaccinated, and in Canton since vaccination was introduced leprosy has declined (Cantlie). On the whole we must conclude that there is still much to be learnt about the conditions which govern the prevalence of leprosy.

With regard to prevention, the isolation of patients is obviously desirable, especially in the later stages, when open sores may disseminate the bacilli; but complete segregation, which has been urged, is regarded as impracticable by those who have had most experience in leprous districts. Scrupulous cleanliness should be exercised by persons attending on lepers or brought into close contact with them. In treatment the most essential thing is general care of the health, with good food and clothing. The tendency of modern therapeutics to attach increasing importance to nutrition in various morbid states, and notably in diseases of degeneration, such as tuberculosis and affections of the nervous system, is borne out by experience in leprosy, which has affinities to both; and this suggests the application to it of modern methods for improving local as well as general nutrition by physical means. A large number of internal remedies have been tried with varying results; those most recommended are chaulmoogra oil, arsenic, salicylate of soda, salol and chlorate of potash. Vergueira uses Collargol intravenously and subcutaneously, and states that in all the cases treated there was marked improvement, and hair that had been lost grew again. Calmette’s Anterenene injected subcutaneously has been followed by good results. Deycke together with R. Bey isolated from a non-ulcerated leprous nodule a streptothrix which they call S. leproides. Its relation to the bacillus is uncertain. They found that injections of this organism had marked curative effects, due to a neutral fat which they named “Nastin.” Injections of Nastin together with Benzoyl Chloride directly act on the lepra bacilli. Some cases were unaffected by this treatment, but with others the effect was marvellous. Dr W. A. Pusey of Chicago uses applications of carbon dioxide snow with good effect. In the later stages of the disease there is a wide field for surgery, which is able to give much relief to sufferers.

Literature.—For history and geographical distribution, see Hirsch, Handbuch der historisch-geographischen Pathologie (1st ed., Erlangen, 1860, with exhaustive literature). For pathology, Virchow, Die krankhaften Geschwülste (Berlin, 1863-1867), vol. ii. For clinical histories, R. Liveing, Elephantiasis Graecorum or True Leprosy (London, 1873), ch. iv. For medieval leprosy—in Germany, Virchow, in Virchow’s Archiv, five articles, vols. xviii.-xx. (1860-1861); in the Netherlands, Israëls, in Nederl. Tijdschr. voor Geneeskunde, vol. i. (1857); in Britain, J. Y. Simpson, Edin. Med. and Surg. Journ., three articles, vols. lxvi. and lxvii. (1846-1847). Treatises on modern leprosy in particular localities: Danielssen and Boeck (Norway), Traité de la Spédalskhed, with atlas of twenty-four coloured plates (Paris, 1848); A. F. Anderson, Leprosy as met with in the Straits Settlements, coloured photographs with explanatory notes (London, 1872); H. Vandyke Carter (Bombay), On Leprosy and Elephantiasis, with coloured plates (London, 1874); Hillis, Leprosy in British Guiana, an account of West Indian leprosy, with twenty-two coloured plates (London, 1882). See also the dermatological works of Hebra, Erasmus Wilson, Bazin and Jonathan Hutchinson (also the latter’s letters to The Times of the 11th of April and the 25th of May 1903); British Medical Journal (April 1, 1908); American Journal of Dermatology (Dec. 1907); The Practitioner (February 1910). An important early work is that of P. G. Hensler, Vom abendländischen Aussatze im Mittelalter (Hamburg, 1790).