CHAPTER IV.. Lepra Maculo-anæsthetica.

LEPRA MACULO-ANÆSTHETICA.

This form of leprosy was first distinctly and well described by Danielssen, who called it L. anæsthetica; but since the macules, as Danielssen recognised, play an important and constant rôle in the course of the disease, we prefer the name maculo-anæsthetica, as it includes the two most striking symptoms; the name L. nervorum used by many investigators, we do not consider satisfactory. Certainly the nerves suffer most, and the neuritis is the most prominent feature in the disease, but the skin affection is a bacillary one, which precedes or accompanies the neuritis; it is not, as is often believed, a tropho-neurosis, as we have determined by the demonstration of bacilli in both young and very old leprous patches.

Thus there disappears the sharp distinction between the two forms of the disease--the tuberous and the maculo-anæsthetic. We must regard them as the same disease, only with varied intensity in the action of the bacilli.

One can distinguish in the maculo-anæsthetic form, different stages in the course of the disease, but in general they cannot be very sharply defined from one another.

In the prodromal stage, which is of very varied duration, lasting for months or even years, the patients state that they suffer from exhaustion, general debility, rheumatoid pains of the joints or muscles, hyperæsthesia of the skin, neuralgic pain of certain nerve regions, sopor and mental depression. Ephemeral eruptions of spots are admitted; and pigmentary anomalies, sometimes atrophic, sometimes hypertrophic, were noted by Bidenkap.

Danielssen states that he has seen, at the very beginning of the disease, a slight vaso-motor disturbance, which is indicated by a bluish-red reticular appearance, which is evident most clearly on changes of temperature. These vaso-motor disturbances, which appear as slight patches which can be induced by friction, are chiefly characteristic of the maculo-anæsthetic, though they may appear during the earliest stage of the tuberous, form.

In fact, our view is that the so-called prodromal symptoms are nothing more than the earliest, indefinite, undiagnosable phenomena of infection.

One or more eruptions of pemphigoid bullæ may occur in the commencing stage, but we have more often seen them later, both accompanying the patches and in the stage of anæsthesia and mutilation.

After a longer or shorter period the typical picture of the maculo-anæsthetic form develops; the spots either appear stealthily or they may appear all at once with marked fever. Several forms of the eruption are described by investigators; in our patients usually only the erythematous and the yellowish or brownish pigmented ones have been noted. Usually both forms are seen on the same patient, for the simple erythematous spots become in time more and more pigmented--usually only at the periphery, where a bluish-red play of colours is often seen. Those eruptions which are all along pigmented and which develop very gradually, we have particularly noted in the intercostal spaces. Various forms--round, oval, gyrate--have been observed. The patches may be perfectly flat, or slightly elevated. The size varies from that of a pea up to that of the palm of the hand, and they may be even larger. At the commencement, we have usually found these patches hyperæsthetic; anæsthesia is only found in the older patches. They do not always at once attain their full size; we can often observe their growth; one may run into another, and then the initial form is lost. The number and extent of these patches are very varied; some patients present great map-like eruptions on the face, back and extremities; in others the patches are few and scattered. The seat of these patches corresponds in general to that of the nodules, but the back and the intercostal spaces are frequently the seat of patches, while on these areas nodules are only rarely, if ever, present. Plate IV gives a good picture of the patches in the maculo-anæsthetic form--duration, two years. The white centres and the slightly elevated reddish edges are very well shown. A symmetrical distribution of the patches strikes one, and has been regarded as indicative of a central localisation of the leprous poisons of which the patches are a tropho-neurotic vaso-motor symptom; but in many cases there is absolutely no symmetry, and the discovery of bacilli in the patches themselves proves them to be the direct result of the action of the lepra poison. The lymphatic glands corresponding to the position of the patches are always swollen, and the swelling may persist long after the disappearance of the patches. The duration is very varied; some are gone in a few days or even less, others may last for years. Pigmentation of the periphery and pallor of the centre indicate that the patch is already old, and the pallid centre is always anæsthetic, the anæsthesia affecting all, or only some perceptions. The signs which Hillis indicated, as diagnostic of the patches of the anæsthetic from those of the tuberous form, we cannot recognise. The patches of the tuberous form are certainly usually thicker, indicating a greater degree of infiltration, than the anæsthetic; but as both are caused by the lepra bacillus it is evident that they may be absolutely similar.

The longer the disease lasts the more does the neuritis predominate; the ulnar and peroneal trunks may be felt to be thickened, they are sensitive to pressure, and, if carefully felt for, the peripheral finer branches may be felt as delicate thickened cords. In one case we were able to feel the cutaneous nerve branches in a patch, growing daily more and more thickened, in contrast to the large not especially affected nerve trunk. The large palpable nerve trunks are not equally thickened in their whole length, but the thickening appears about the joints where the nerves run across bone.

The symptoms of this neuritis are various; at first neuralgia, and later, widespread anæsthesia, with trophic disturbances, such as the formation of pemphigoid bullæ; we have often seen hydrarthrus and pains in the joints. Motor pareses and paralyses are never absent, but they are not, as Neisser argues, due to a leprous affection of the muscles, but are a secondary neuritic symptom, as we have discovered from anatomical examination of the muscles. As the neuritis especially affects the peripheral nerve trunks, we find the secondary symptoms in the peripheral regions, usually only in the extremities and on the face. We will now more closely consider the various nervous symptoms.

Neuralgia is usually present in the extremities, in the ulnar and peroneal regions. The anæsthesia relates to the different qualities of sensation, and is not only present in the patches, but progresses gradually from the periphery toward the centre, so that at last the whole extremity, and often also parts of the trunk, become anæsthetic; the face is always more or less anæsthetic. We have often found thermal anæsthesia present alone, or accompanied by anæsthesia or analgesia. The anæsthesia may become more and more extensive, or it may very gradually disappear, indicating that the neuritis of the affected nerve has disappeared.

Trophic and vaso-motor disturbances are never absent if the neuritis is pronounced and lasts for any time; the skin may become œdematous, or it becomes thin, shiny, and slightly scaly (glossy skin). We have often seen, especially if the neuritis has lasted long, and the later symptoms such as mutilation have commenced, dark-coloured hyperkeratoses, usually symmetrical on the front of the leg, or on the dorsal surface of the hands. In one patient we observed on the toes horny, thick (1.5 cm.) symmetrically placed formations, which when thrown off left a new-formed rosy-red skin, with intact sensibility; the patient had on the front of the leg the appearances of ichthyosis.

The changes in the nails are a part of the trophic disturbance; they become thickened, brittle and cleft, and sometimes thin and diminished in size, as one finds them, as we shall see later, in the terminal stages. The secretion of sweat is affected, being diminished over the anæsthetic areas, and the hairs there are altered and fall out.

We regard the pemphigoid bullæ as a trophic symptom; they may appear at the very commencement of the disease, along with the macular eruption; but they usually appear late when the anæsthesia has become extensive; in this we agree with Danielssen. Neisser and Leloir give prominence to the early appearance of pemphigoid bullæ, Neisser believing that the irritation of the commencing interstitial neuritis causes this trophic symptom. Our reason for regarding them as trophic symptoms is that we have never been able to find bacilli in the bullæ we have examined, not even in those which appeared at the same time as the maculæ, and their marked symmetry is also in favour of their nervous origin. The bullous eruptions usually appear suddenly. The patients will discover on awaking, one or more blisters which may be already burst; some have pain and fever for hours or days before their appearance; (Leloir). They vary in size--they may be small, from the size of a pea to that of a bean, or as large as the palm of the hand.

Their contents are serous, but if the bullæ persist, they become purulent. They usually rupture early and heal, leaving behind them violet-coloured scars, which after some time become pale. If irritant factors are added, if the bullæ last long and become purulent, there develops after rupture deep ulceration, most frequent on the hands and feet.

Bullous eruptions of the mucous membranes, which Leloir has noted, we have never been fortunate enough to see.

We regard also as tropho-neurotic vaso-motor symptoms the acute rheumatoid affections of the joints, which are not infrequent in our hospital. The joints, especially the small ones of the fingers and hands, and also those of the knee and ankle, become painful and tender, and on palpation, a collection of fluid can usually be recognised. The affection is always symmetrical.

These affections of the joints, which belong to the earlier stages of the disease, usually appear simultaneously with the macular eruption, and disappear with it, but they may appear later; and after one, or it may be only after several attacks, thickening of the ends of the bones and ligaments, with stiffening of the limbs, is developed. Leprous affections of the tendon sheaths, which Wolff refers to, we have never seen. The contracture of the fingers and toes is not tendogenous, so far as we can decide from clinical and anatomical investigation; it is myogenous, the leprous paralyses, which we shall immediately describe, being the cause.

Hillis claims to have recognised a motor weakness in the prodromal stage of the disease, and considers that the nerves are already affected with the leprous virus. Such a weakness referable to a neuritis of the motor nerves we have not noted previous to the macular eruption and the onset of the anæsthesia. If there are, in the prodromal stage, muscular weaknesses, we are inclined to regard these as symptoms of the general weakness; according to our view, the skin affection is the first definite symptom of the disease. The muscular affection may always be definitely recognised as a secondary symptom by the atrophy, and the altered electrical reactions. Different degrees and varied extent of the muscular affection may be noted; trivial paresis with only very slight atrophy, which, along with the anæsthesia, may completely disappear, if the neuritis passes off without destroying the nerve fibres; and almost complete paralysis with great atrophy of the muscles.

Paralysis with atrophy is most marked on the hands and forearms, feet and calves, and on the face.

The interosseous muscles, of the hands and feet atrophy, so that the interosseous spaces appear as furrows. The thenar and hypothenar muscles also disappear, and the first interosseous space becomes a depression. The muscles of the forearms and calves also atrophy and lose their power, but complete paralysis is never developed; the patients can always move their hands and walk. In the face, all the muscles atrophy and lose their power, and thus all expression is lost. The masseter muscles occasionally, though very rarely, become so atrophic, that the lower jaw drops, and must be retained in position by a bandage. Paralysis of the Orbicularis Oris leads to dropping of the under lip, and to extreme difficulty in closing the mouth, and consequent escape of saliva. (See Plate V--a case of maculo-anæsthetic form completely cured and anæsthetic. The maculæ were developed in 1857, and the patient is still alive.) Paralysis of the Orbicularis Palpebrarum renders the closure of the eyes impossible, and paralytic ectropion of the lower lid is developed. The results of this on the eye we will refer to later. The muscular atrophy is by no means always symmetrical; one hand may present marked atrophy, while the other is tolerably useful. This indicates the peripheral development of the paralysis, as does the fact that the muscular sense is preserved, and in particular that no ataxic symptoms appear. The patients can do fine knitting and needle-work with their paretic hands. Their gait has a peculiar character, from the difficulty with which the weakened muscles raise the feet, but they can both stand and walk quite well with closed or bandaged eyes. As we shall see later, this clinical observation corresponds with the results of the anatomical examination of the cord.

Along with the paralysis and atrophy of the muscles, there appear also, as the result of the destruction of the nerve trunks, trophic affections of the bones and of the skin. The changes in the skin have been already described; when the anæsthesia is advanced, there always appear ulcerations under the heel and the ball of the foot. Most of these owe their origin simply to pressure; the weight of the body is too much for the atrophic skin. They are always callous, heal with great difficulty, and so long as the patient goes about, not at all. The ulcers are not usually deep, but it may happen that they penetrate as far as the bone, and lead to necrosis. But usually necrosis of the bones is preceded by periostitis, and when the pus makes its way out, an ulceration leading down to necrotic bone is formed. We have sometimes seen the formation of a blister precede the ulceration, but it is rare, and it is certainly not true, as stated by many authors, that ulceration of the sole is always preceded by a bulla. Not only on the feet but also on the hands, necrosis with exfoliation of bone takes place; the phalanges are especially attacked. When the atrophy of the muscles is advanced, the fingers are always claw-like, with extension of the first phalanx. The joints consequently often appear swollen, although no joint affection is present. The phalanges and the metacarpals undergo simple atrophy, becoming very thin at the middle, and since the joints retain almost their normal thickness, they appear swollen by contrast. As already noted, this clawing of the fingers is accompanied by no disease of the tendon sheaths. The bones in the feet undergo the same form of atrophy.

If, in addition to this atrophy of the bones, which was first noted and demonstrated by Prof. Hjalmar Heiberg, there ensues necrosis with exfoliation of whole phalanges or metacarpals (the carpal bones very rarely necrose), there is great mutilation of the hands and feet; all the fingers of the hand may go, and there remain on the diminished carpus only small, soft processes, each supplied with a nail--the remnants of the fingers. The toes disappear from the feet, the metatarsal bones atrophy, and several of the tarsal bones may exfoliate; so that ultimately there remains of the foot only a pyramidal cushion at the lower end of the leg. In most cases we must regard external injury as the cause of these necroses. The patients feel nothing when they injure themselves; they may burn their hands at a stove without noticing it. Under such circumstances it is not to be wondered at, that inflammation is readily excited, especially as the vitality of the parts evidently diminished. But it is remarkable how well operation wounds heal. One may do pretty extensive necrotomies, and the wounds heal well and quickly, either by granulation or by first intention. In such operations it is a frequent experience that the bone is reached before the patient feels anything, but he immediately feels pain when the periosteum is scraped or the bone attacked with forceps or saw. We believe, however, that it is only nervous individuals who complain of pain; though it is certain that when the bone is meddled with, something is felt. Probably in this connection may be explained the statement of the patients, that when walking they feel the ground. It is easy to demonstrate that a patient who is quite unconscious of any irritation on his skin, can perceive deep pressure fairly well.

These necroses and trophic disturbances, together with the muscular contraction, cause the mutilation characteristic of the last stage of the disease, which was at one time described as a special form, Lepra mutilans.

We said above that the finger joints appear enlarged on account of the atrophy of the shaft of the bone. In some cases, however, the joints, especially the ankle, show changes which must either be regarded as the remains of the rheumatoid affections of the eruptive period, or as trophic articular changes, corresponding to those seen in Tabes dorsalis. We have seen ankles and knees, but especially ankles, presenting such an appearance. In some cases post mortem examination shows widespread tuberculosis of the synovial membrane and of the ends of the bones, which we shall refer to more particularly under the pathological anatomy.

As we have already mentioned, the eyelids can no longer be closed on account of the paralysis of the orbicularis palpebrarum, and consequently the under part of the cornea remains uncovered during sleep. This leads to a punctiform drying of the epithelium of the cornea, and further, to an injection of the conjunctivæ at the under margin of the cornea; then the vessels gradually attack the cornea, which becomes opaque, at first around the xerotic spots, and later in its whole under part. It may go on to ulceration with rupture of the cornea and prolapse of the iris, and finally to complete atrophy of the globe. As a result of the paralysis, the lower lid is always ectropic, at first at its inner end, and later, completely. As the lower punctum is thus drawn away from the bulb, the tears run down over the cheeks, and the paralysed countenance looks still more woe-begone.

In the later stages, when the facial paralysis is very pronounced, the senses of smell and taste may be very much diminished, or completely lost.

We often see symptoms which are not proper to the disease itself developing during its course, such as obstinate cardialgia, acid pyrosis, and vomiting of a slimy nature indicating gastric catarrh. Diarrhœa or chronic obstruction is by no means rare, nor is albuminuria dependant on parenchymatous, interstitial or amyloid nephritis.

The course of maculo-anæsthetic leprosy is essentially chronic. Cases usually last between ten and twenty years; some may even exceed forty.

The patients often die cachectic, without one being able to find on the post mortem table any definite cause of death, or they may--though in our aseptic and antiseptic times more and more rarely--perish from septicæmia or pyæmia. Pulmonary or general tuberculosis was formerly a frequent cause of death, which, however, usually takes place from some intercurrent disease.

In the ordinary course of the disease the macular eruptions disappear, and the neuritic symptoms--anæsthesia, muscular paralysis and atrophy, and necrosis of bone--appear. Sometimes, though rarely, there are several macular eruptions after the disappearance of the earlier maculæ, or there may be an eruption of nodules. If, then, these eruptions are auto-infections, they are evidence in favour of the unity of the disease, in spite of the difference in form.

Where the bacilli come from, in these, so to speak, later eruptions, when there is no skin affection, it is difficult to say with certainty. In a case of maculo-anæsthetic leprosy we found the inguinal glands leprous, although no skin eruption was present. Possibly, then, fresh eruptions develop from the glands when the original skin eruption has disappeared. Thus the specific leprous affections gradually disappear, and only their results remain--in other words, the leprosy is healed. Most maculo-anæsthetic patients become in time purely anæsthetic; they no longer suffer from leprosy, but only from its results. The late eruptions show, however, how difficult it is to define the fact of recovery, for when all externally diagnosable signs of actual leprosy are gone, bacilli may still remain somewhere in the body, in the lymphatics, or possibly in the liver and spleen.