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INTRODUCTION AND NOMENCLATURE 1

NODULAR LEPROSY 5

Seat, 5; Localisation, 5; Affection of the Eyes, 8; Affection of the Extremities, 10; Affection of the Mucous Membrane, 11; Affection of the Lymphatic Glands, 13; Subcutaneous Nodules, 13; Affection of Nerves in this Form, 14; Fate of the Nodules, 15; Commencement of the Disease, 16; Duration of the Eruptions, 19; Fate of the Patient, 19; Combination with Tuberculosis, 22; Comparison with that Disease, 23; Affection of the Lungs, 26; Cultivation of the Bacilli, 27, Miliary Leprosy, 28; Mental Symptoms, 29.

STRUCTURE OF THE LEPROMA 31

Movement of the Bacilli, 31; Nature of the Cells, 32; Nature of the Blood Vessels, 32; Softening of the Nodule, 34; "Brown Elements," 34; "Globi," 35; Position of the Bacilli in the Cells, 37; Position of the Bacilli in the Blood Vessels, 38; Effect of Measles, 40; Digestion of the Bacilli by the Cells, 41; Structure of the Bacilli, 41; Bacilli in the Sweat Glands, 43; Affection of the Testicle, 45; Affection of the Liver, 47; Affection of the Spleen, 47; Affection of the Glands, 48; Affection of the Nerves, 50.

LEPRA MACULO-ANAESTHETICA 52

Prodromal Stage, 53; Development of Bullae, 53; The Macular Eruption, 54; Symmetry, 55; Neuritis, 56; Trophic Disturbances, 57; Affections of the Joints, 59; Motor Weakness, 60; Atrophy of the Interossei, 61; Paralysis of the Orbicularis oris et palpebrarum, 61; Trophic Affections of the Bones and Skin, 62; Sensation, 64; Loss of Smell and Taste, 66; Duration, 66; Cause of Death, 66; Recovery, 67.

PATHOLOGICAL ANATOMY OF THE MACULO-ANAESTHETIC FORM 68

Comparison of Recent and Old Macuoles, 68; Alterations in the Nerves, 70; Bacilli in the Nerves, 70; The Spinal Cord, 71; Lymphatic Glands, 72; Muscular Affections, 72; Association with Tuberculosis, 73; Necrosis of Bones, 75; Joint Affection, 76; Difference between the two Forms of the Disease, 77; Influence of Climate, 79.

DIAGNOSIS AND PROGNOSIS 82

Diagnosis from Psoriasis, 82; Diagnosis from Syphilis, 83; Diagnosis from Syringo-myelia, 83; Leprosy in France, 83; Morven's Disease, 83; Prognosis, 84.

ETIOLOGY 86

Humoral Pathology, 86; Heredity, 87; Miasma, 87; Contagion, 88; Baumgarten's Dormant Heredity, 89; Nature of Heredity, 90; Father Damien's Case, 93; Leprosy among Norwegians in North America, 94; Inoculation, 95; Hutchinson's Fish Theory, 96; Direct Proofs of Contagion, 97; Hospital Experience in Norway, 99; Communication of the Disease, 102; Greater Danger of Nodular Form, 102; Leprosy and Vaccination, 103.

TREATMENT 105

Ancient Treatment, 105; Treatment in the Eighteenth Century, 105; Specific Remedies--Madar 107, Cashew Oil 107, Gurjun Oil 109, Chaul Moogra 111, Hoang-nan 113, Ussacou 113; Carbolic Acid, 114; Creasote, 114; Phosphorus, 115; Arsenic, 115; Ichthyol, 115; Kreuznach, 115; Mercury, 116; Iodine, 116; Tuberculin, 117; Chlorate of Potassium, 118; Hydroxylamin, 118; Europhen, 119; Aristol, 120; Naphthol and Salol, 120; Methyl Blue, 120; Nerve Stretching, 120; Electricity, 121; Salicylate of Soda, 122; Importance of Isolation, 124; Results in Norway, 125.

TABLES.

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The Frequent Complication of Nodular Leprosy with Tuberculosis.

The Proportion of Tuberculosis in Maculo-Anaesthetic Leprosy.

The Proportions of the Two Forms of the Disease in Different Districts.

The Results of Isolation in Norway.

LIST OF PLATES.

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LEPROSY:

INTRODUCTORY.

The Bacillus Leprae has now been recognised in all leprous products, and although the fact has not yet been experimentally demonstrated, we may practically say with confidence that Leprosy is a chronic disease caused by the Lepra bacillus.

Danielssen and Boeck have also described a mixed form of the disease, in which nodular Leprosy is combined with anaesthetic. Sometimes the skin eruption disappears and the nodular form passes into the anaesthetic, and sometimes, though much more rarely, the anaesthetic into the nodular; and since, further, the two forms are so different in their clinical appearances that they look almost like different diseases, the recognition of a mixed form might appear to be justified. But since every case of nodular Leprosy is accompanied by affection of the nerves and anaesthesia; and the natural termination of every case of nodular Leprosy is to pass into the anaesthetic form, if only, as occasionally happens, the patient live long enough; and since the skin eruptions of the maculo-anaesthetic form are characterized, just as those of the nodular form, by the presence of the leprosy bacillus, we regard the transformation of a case of maculo-anaesthetic into nodular Leprosy only as a sign of the unity of the two forms, and we delete altogether the name of mixed Leprosy. Otherwise every case of nodular Leprosy must, at all events after some years of existence, properly be called "mixed," for in such cases anaesthesia is never absent.

It has been attempted to indicate as a special form of the disease a Nervous Leprosy, in which no characteristic skin affection is present. In view of what we have noted above, that nerve Leprosy is present in both forms of the disease, and that an eruption may be noted at some period in all carefully observed cases of the disease, this attempt to indicate a special nerve Leprosy is evidently wrong.

See Neisser: Lepra in Ziemssen's Handbook.

We will first of all discuss separately the two forms of the disease, the nodular and the maculo-anaesthetic, and then proceed to demonstrate the unity of the disease in spite of the differences in form.

NODULAR LEPROSY.

Nodular Leprosy is usually easily diagnosed by its characteristic skin affection.

Here in Norway where the people often go barefoot, wading in streams, marshes and rivers, the backs of the feet and the under part of the calves are frequently the seat of the first leprous eruption, not so often in the form of nodules, as of a dense, regular infiltration. Now since, as we have noted above, the face and back of the hands are the usual seats of predilection for the earliest appearance of the eruption, it appears not improbable that this has its explanation in the climatic influences on these parts, possibly influenced by the structure of the skin, especially the cutaneous vessels. That there are peculiarities in the structure of the tissues, which determine the localisation of the poison, one may conclude; for certain organs are never affected with leprosy, in spite of the fact that the poison has evidently at some time circulated in the blood.

On the extremities the nodules always appear singly, but when closely set may run together to form large plaques. On the backs of the hands and fingers nodules are very frequently, and on the extensor surfaces of the thighs and the front of the legs almost always, found. The calves are often also infiltrated as a whole, especially on the fibular side close above the ankle, and this infiltration reaches as high as the middle of the leg; the skin is tense and shiny, reddish blue in colour, and in this infiltrated part ulcers resembling varicose ulcers readily appear, which are as difficult, if not more so, to heal. They are surrounded by thick elevated walls, may last for years, and occasionally completely surround the leg.

Of the mucous membranes, those of the nose, mouth, larynx and pharynx are affected. The nasal mucous membrane is affected only in its anterior part along with the alae nasi and the anterior part of the septum. If a general infiltration takes place in this situation, the softening and ulceration which may ensue lead eventually to the disappearance of all the soft parts of the nose; the bones are never affected. In the mouth, the mucous membrane of the lips, of course, shares in the process when these are completely infiltrated, and even on the mucous membrane of the cheeks one occasionally sees and feels thickening and infiltration. The tongue is often the seat of nodules, which in all respects correspond to those of the skin. The gums, the velum, and the uvula may be either infiltrated or dotted with nodules. The rest of the mucous membrane of the pharynx is more frequently infiltrated than beset with nodules, and the same is true of the epiglottis, which sometimes becomes quite stiff and almost immoveable. In the larynx, the true and false cords are more frequently the seat of infiltration than of nodules; the voice is rough and hoarse, the rima glottidis is often so narrowed that respiration is rendered difficult; excessive narrowing of the rima is only present in the late stages, and is proportionately rare. When the mucous membrane ulcerates the cords grow together, both anteriorly and posteriorly, and when the infiltration disappears there remains a scar tissue, which, by its contraction, reduces the rima to a small slit, a few millim?tres wide. In such cases a very little mucus is sufficient almost or completely to close the opening, and the patient may perish from suffocation. Usually an emetic suffices to open up the hole at once; but tracheotomy is often necessary to supply air to the patient, the attacks of suffocation are so frequent, and since he is already voiceless, he loses nothing by the operation.

The lymphatic glands in relation to the affected skin and mucous membrane are always swollen: this leprous swelling is always indolent, and never goes on to suppuration. Sometimes the glandular swelling may aid in the diagnosis, if the skin affection is not absolutely characteristic, though this is most rarely the case.

The nodules are almost always seated in the cutis, but they may, though rarely, be placed deeper in the subcutaneous connective tissue; they form then no projections, but the skin over them is almost always somewhat hyperaemic and bluish-red, and, if the finger is passed over the place, the thickening or the nodule may be felt in the deeper parts. It is in our experience that a patient who had only this form of nodules was regarded by a colleague, well acquainted with the disease, as free from Leprosy, probably because he did not use his fingers.

From the symptoms described above, the diagnosis is almost always very easy, and we ourselves know of no disease of the skin which can be confounded with nodular leprosy. If necessary a piece of skin may be removed and examined for the presence of bacilli, which, at least in the nodular form of the disease, are never absent. This, we have once had occasion to do.

In addition to the skin the nerves are also affected, not always at the commencement, but always in the later stages. Whether all peripheral nerves are affected we cannot say--certainly the facial, radial, ulnar, median and peroneal are always diseased. According to our investigation the nerves of the extremities are affected throughout their whole length, but the affection is severe only at certain places, viz., where the nerves run superficially over bones or joints, as the median at the wrist, the ulnar at the elbow, and the peroneal where it crosses the fibula. As a result of this nerve affection we have pain followed by anaesthesia. The pains in the arms, hands, feet and calves are sometimes very severe and persistent. The affection at first causes pain, by pressure on the nerves, and later--when the pressure has led to atrophy--anaesthesia. Now since, as we shall later clearly demonstrate, leprous affections tend to heal, it is not infrequently the case that nerve affections, when slight, pass off without having specially injured the nerves, and these nerves may be the seat of fresh infections, and thus the patient suffers from repeated painful attacks through a course of years. This is particularly the case where there is general infiltration of the legs, and is either the result of repeated attacks of the same, or of the implication of different nerve branches. The nodules are often painful when first developed, but later on sensation is deadened.

Of internal organs, the testicle, liver, and spleen, are always affected in this form, but we shall consider them later in the description of the pathological anatomy.

Before we more closely describe the course of the disease, we shall first briefly discuss the fate of the nodules. These usually remain for years unchanged, growing very little or not at all. The skin over and around them, or rather its vascular supply, is very sensitive to changes of temperature, so that the skin, as we have already indicated, changes its colour with change of temperature from dilatation of the blood vessels. The vessels evidently suffer from the invasion of the leprous poison. New outbreaks have often the appearance of an "erythema nodosum," with great hyperaemia. We had once the opportunity of examining a piece cut out of such an erythema-like eruption, and found dilated vessels and round cells, and only after long search a few bacilli. One must conjecture that there is deposited with the bacilli a chemical poison which affects the vessels, or that the bacilli produce the poison, and that this poison has its action only in its immediate neighbourhood.

But occasionally the nodules grow so luxuriantly that the epidermis develops furrows and clefts which may reach down to the nodules, and then a bloody fluid comes out of them which dries up on the surface to a reddish brown scab. Or it may happen that the upper horny layers of epithelium disappear, and that only a few rows of cells of the rete Malpighii remain. In this case the exuded fluid less often dries, the surface is usually blood-red and moist, and appears like an ulceration, though it is not really one. When this takes place on the face, particularly on the lips, or on the backs of the fingers, the sufferings of the patient are very much increased. After several years the nodules usually soften about the middle of their base, and the nodule may sink in over the softened part; or they burst, the softened part is thrown off, and now is developed the true ulceration by which the nodule may be completely eliminated and sunken stellate scars alone remain.

The determination of the commencement of the disease is exceptionally difficult, indeed impossible, for it must always be founded on the statements of the patient, and the patients either observe themselves insufficiently, as may frequently be noted, or they conceal many facts. As a matter of fact we do not know the earliest symptoms of the disease. According to Danielssen and Boeck, the patients often suffer long and repeatedly, before the outbreak of the disease, from weakness, with rheumatoid pains and fever. This the patients frequently corroborate. But we are inclined to regard these attacks of fever as indications of the already existing disease. It appears to us more probable that the disease begins with some form of local affection which is so indistinct that the patient himself does not notice it, or at least lays no weight upon it, and that these local affections are analogous to others with which we are familiar, namely, the nodules which may last for years before new and such definite eruptions appear, that the disease cannot any longer be ignored or kept secret. We believe therefore, that the patients do not really know when they commence to be ill, and that they date the beginning of the disease from a later eruption. If at the commencement only the extremities are affected the patients may conceal their condition for years, and through this concealment become so accustomed to lie, that later it is impossible to receive from them correct information.

The cases are very frequent in which the patients have for several years only scattered nodules, and then suddenly a fresh outbreak of numerous nodules. The disease always advances by outbreaks of eruptions which repeat themselves at longer or shorter intervals. It is very often the case that the older nodules soften during a fresh outbreak, and completely or partly disappear; and these outbreaks are always accompanied by fever, the temperature rising to 39? or 40? Cent. Now we know that the nodules, if the patient is affected by another febrile disease, may disappear. It is, therefore, difficult to decide whether the disappearance of the nodules is the cause or the result of the eruptive fever. But we possess certain observations in which the disappearance of the nodules has begun before the onset of the fever, and in which, therefore, the fever and the later eruption appear to be caused by the softening of the nodules. Supported by these observations we regard the eruptions as auto-infections, in which bacilli from the older lepromata pass into the blood, and thus new areas of the skin or other organs are affected. We have often observed that an irido-cyclitis, or an affection of the throat, arises during an eruption, and also that the nerves beneath the nodules become swollen and painful, and once we have seen the testicle become swollen and painful during an eruption. As to the affections of the liver and spleen we have no clinical observations; they appear to cause no clinical symptoms, or at all events, such indefinite ones that, although our attention has been directed to them, they have escaped observation.

If there is added to this, amyloid degeneration of the kidneys, liver, spleen and intestine, with diarrhoea, it can only be desired that death shall put an end to such a condition, and that usually takes place soon, although occasionally the patient may linger for months. Whether leprosy alone is responsible for the end is, we think, doubtful; as remarked above, the affections of the liver and spleen appear to be without much significance.

The biliary secretion is never influenced, and there is according to our observations no special leprous anaemia. The patients are indeed often anaemic, or become so during the disease, but we have not been able to convince ourselves that this is a direct result of the leprosy. In many examinations of the blood, which were indeed undertaken for other purposes, we have never noticed anything remarkable in relation to the number or form of the blood corpuscles.

As in almost all cases of nodular leprosy nephritis is present, we are inclined to regard this nephritis as a frequent cause of the marasmus which ensues. Tuberculosis was formerly a frequent occurrence in our hospitals, where our observations have chiefly been made. The relationship between this and leprosy we will discuss later.

The prognosis in the case of patients in whom the eruptions appear less frequently is more favourable, and they may live many years. Either they die from an intercurrent disease or as a result of their nephritis, or they become in time anaesthetic, that is, according to our view, they recover. When the nodules become stationary they ultimately soften, as described above, and may be absorbed without opening, though this is rare and usually occurs only with single nodules; or they burst and ulcerate; in either case they leave scars. If this takes place in all the nodules and the patient is attacked by no fresh eruptions, then anaesthesia gradually develops as the result of the affection of the nerves; in the nerves, too, the specific leprous affection disappears, and there remains only scar tissue, which by compression destroys the nerve fibres and thus causes anaesthesia. Then gradually all specific leprous affections disappear, and the patient is healed from his leprosy, and may live many years in perfect health, having lost practically nothing of his power of work. Such cases are unfortunately not very frequent; but we have had the opportunity of examining some after death and have not been able to discover in them any specific leprous affection.

Thus one is struck with the fact, how little leprosy of itself influences the health of the patient, and if nodular leprosy usually shortens life, that takes place probably because in this form the frequent ulceration leads to amyloid degeneration of the internal organs, or that the nephritis is a sequel of the leprosy. The nephritis appears either as the so-called parenchymatous or as the interstitial; according to our examinations it is never bacillary. Further, as nephritis is very much rarer in the maculo-anaesthetic form of the disease, it must be assumed that nodular leprosy in some way causes nephritis. The same is true of amyloid degeneration. The duration of life of a patient with the nodular form of leprosy is in general eight to nine years after the definite outbreak of the disease.

The most frequent complication which we have seen in our institutions is tuberculosis, particularly some years ago, for then the institutions were over-crowded, and consequently the sanitary conditions were in many respects unsatisfactory.

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