Leprosy

CHAPTER I.. Introductory.

INTRODUCTORY.

The Bacillus Lepræ 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.

Leprosy appears in two forms, which are clinically pretty sharply distinguishable, and were named by Danielssen and Boeck “nodular” and “anæsthetic.” This nomenclature, introduced by these authors in their pioneer work, Om Spedalskhed, Christiania, 1847, Traité de la Spedalskhed, Paris, 1848, is so far important as it characterizes the common and most prominent symptoms of the two forms, viz., in the one, the nodular eruption on the skin, in the other, the anæsthesia which results from the widespread affection of the nerves. On the other hand, regarded from a strictly scientific standpoint, the nomenclature is scarcely the happiest: firstly, because the one form is named from the eruption on the skin, and the other from the results of the affection of another organ, the nerves; and secondly, because the nerves are affected in the nodular as well as in the other form, though the result of the affection, the anæsthesia, does not so dominate the appearance of the disease.

As Danielssen and Boeck recognised, skin eruptions are present in both forms, and since, as we shall later point out, the eruptions differ both clinically, and also somewhat in their anatomical formation, it would perhaps be more strictly correct to describe the two forms as Lepra tuberosa (tuberculosa) and Lepra maculosa or lævis. But, in the first place, it is of questionable advantage to change a universally accepted nomenclature; and secondly, it is very frequently the case that one sees the patient for the first time after the disappearance of the macular eruption with anæsthesia only, so that the name Lepra anæsthetica exactly describes the case. But in order to give to the skin eruption in the anæsthetic form its proper place in the nomenclature we will describe the two forms of the disease as Lepra tuberosa (tuberculosa), and Lepra maculo-anæsthetica. And thus we hope to have done justice both to the founders of the scientific study of Leprosy, and to the clinical appearances of the disease.

Danielssen and Boeck have also described a mixed form of the disease, in which nodular Leprosy is combined with anæsthetic. Sometimes the skin eruption disappears and the nodular form passes into the anæsthetic, and sometimes, though much more rarely, the anæsthetic 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 anæsthesia; and the natural termination of every case of nodular Leprosy is to pass into the anæsthetic form, if only, as occasionally happens, the patient live long enough; and since the skin eruptions of the maculo-anæsthetic 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-anæsthetic 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 anæsthesia 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-anæsthetic, and then proceed to demonstrate the unity of the disease in spite of the differences in form.

CHAPTER II.. Nodular Leprosy.

NODULAR LEPROSY.

Nodular Leprosy (Lepra tuberosa, L. tuberculosa) is usually easily diagnosed by its characteristic skin affection.

The leprous nodes or nodular Lepromata are of different size and colour; their consistence is at first firm and hard; they are but slightly compressible, and show little elasticity. Their form is usually semi-spherical, but they are often oblong. The smallest nodule that we have seen was not more than 1-2 mm. in diameter, and its appearance was so little characteristic, that we had to confirm the diagnosis by excision and microscopical examination. The larger the nodules, the more characteristic is their appearance. As they are almost always seated in the cutis, the epidermis over them is stretched and shiny; it is occasionally normal in colour, but usually at first reddish, later becoming yellow. The localisation of the nodules is usually characteristic. They are generally first evident on the face, on the backs of the hands, and on the dorsal surfaces of the wrists, and next on the extensor surfaces of the limbs. They are more rarely seen on the back and nates; on the flexor surfaces of the extremities, on the breast, abdomen, scrotum, and penis, they are quite exceptional; and we have never seen them on the glans penis, the palms or soles, or on the hairy scalp. Leloir has described a leprous affection of the palms of the hands, of which he himself says that it closely resembled a syphilide in that situation. Since Danielssen, with his enormous experience, never saw a leprous affection of the palm of the hand, we incline to believe that the affection which Leloir observed, not only resembled, but actually was, a syphilide.

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.

The face is usually especially characteristic, as the eyebrows are almost always the seat of nodules. The nodules are sometimes isolated though close together, sometimes there are only one or two, though usually several, and sometimes there are no distinct nodules, but the eyebrow is infiltrated both in length and breadth, and of a reddish colour. Even if the infiltration is not so great that the brow appears thickened, the reddish colour and the shadow over the eyes give to the face a characteristic expression, and one can feel the infiltration, if the brow is gripped between the thumb and forefinger. In these cases the hairs persist; in more severe infiltration, and where nodules are formed, they drop out. The forehead and cheeks usually present a diffuse or spotty redness and burnish, and with the finger one recognises the infiltration as an increased resistance. This discolouration is most evident on changes of temperature, as when a patient comes from the outside cold into a warm room. Not infrequently the suspicion of Leprosy is aroused by this change of colour, and by the shadows over the eyebrows, even years before more definite symptoms appear. But in most cases one finds distinct nodules in the eyebrows and over the countenance generally. When the nodules are numerous and large, so that the eyebrows project far over the eyes; when the cheeks and chin are beset with large rounded or flat nodules pressing on each other, so as to cause deep furrows between them; when the point of the nose, the alæ nasi, and the lips are infiltrated throughout their whole thickness; the countenance is frightfully deformed, and there is developed the so-called Facies Leonina. The lobules of the ears are almost always infiltrated, and become red, thick and elongated. Plate I is a case of tuberous leprosy of two years’ duration, the hands being swollen with leprous infiltration.

The eyes are, in the nodular form, almost always affected; nodules are frequently present in the eyelids, the upper as well as the lower, and are usually situated close to their margin. The earliest affection of the eye itself, which we have observed, is a faint clouding of the upper part of the cornea, which often appears as a very fine dotting of the corneal surface, only noticeable when one can compare the upper part of the cornea with the black pupil, and often requiring for its recognition the use of a lens. A slight infiltration of the limbus conjunctivæ is always combined with this clouding of the cornea, but it is at this early stage so slight that it cannot be noted clinically. Later on it increases, and gradually attacks the whole of the outer margin of the cornea. When this infiltration becomes greater it appears yellow, as seen through the conjunctiva running intact over it, and this gives to the eye a peculiar woe-begone aspect. It is quite exceptional for this infiltration to extend completely round the cornea, for that part of the limbus directed towards the nose is almost always free. As time goes on the infiltration increases, and a low rampart is formed around the cornea. Sooner or later the infiltration and nodule formation attack the cornea itself, in one of three different ways: first, quite superficially, immediately under the epithelium. The nodule in this case is always elevated, usually grows very rapidly till it finally covers the whole cornea, and may by its height prevent the closure of the lids. That part of the cornea lying below or behind the nodule is quite clear. Secondly, the infiltration may attack the cornea in the form of a wedge, and form a node which is not so much elevated as in the previous instance; and thirdly, the infiltration may penetrate the cornea close in front of Descemet’s membrane. The result, complete blindness, is the same in all cases when the nodule covers the pupil. A frequent accompaniment of this form of the disease is iritis, or, as anatomical investigation shows, irido-cyclitis. These forms of iritis run a chronic or sub-acute course, sometimes so stealthy and painless that it is observed by neither doctor nor patient, until adhesions have formed between the pupillary border and the capsule of the lens. Blindness may sometimes be caused by exudation into the pupil. Plate II shows a typical case of tuberous leprosy of six years’ duration. The hairs have completely disappeared from the eyebrows; on the chin a few can still be seen between the tuberosities. In the right eye is a nodule, growing from the Limbus conjunctivæ into the cornea. Nodules may also be present in the iris, and usually arise in the outer and under margin, in the angle between the cornea and the iris; they may completely fill the corresponding part of the anterior chamber, are of a yellow colour, and sometimes look exactly like an obliquely-placed hypopion, as they have an inner or upper straight, or slightly concave margin. We once did an iridectomy directly through a small early nodule, and put a stop to its further growth. On anatomical examination we also find a leprous affection of the anterior part of the retina over the ciliary body, which appears as a fine white spotting of the retina; the ciliary nerves are always for a considerable distance backwards infiltrated with leprosy, as are the membrana supra choroidea and the choroid itself.

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