(Leprosy; Elephantiasis Graecorum; Satyriasis; Leontiasis)
Definition – A chronic, insidious, endemic, contagious disease due to a specific bacillus and characterized by erythema, anesthesia, pigmentation, neoplastic growths, atrophies, ulcerations and deformities varying with the parts affected.
Leprosy, the oldest of known diseases, is at the present distributed over a quarter of the habitable globe. It is common in India, China, Japan, the Philippine Islands and parts of Africa; comparatively frequent in Iceland, parts of Canada, the West Indies, Central and South America and the Hawaiian Islands. in Europe it is chiefly found in Norway, Russia, Greece, Spain and Portugal. Sporadic cases are always present in the United States especially in our large seaport cities, but it is more constantly found in California among the Asiatics, in Louisiana among West Indians and in the northwestern states like Minnesota and Wisconsin among persons of Scandinavian origin. It is difficult to say how many cases there are within the bounds of the United States proper but probably five hundred would be an approximate figure. Nearly all of these cases have contracted the disease in some other country although the actual development may have taken place years after their arrival here. For the past ten years I have had from five to ten cases under my observation yearly and their clinical histories verify the preceding assertion.
Symptoms – While the manifestations of leprosy vary widely, it is convenient to describe three principal forms (1) the nodular or tubercular, chiefly involving the skin and mucous membranes; (2) the anesthetic, chiefly attacking the peripheral nerves; (3) the mixed form including both of the other two types. The stages of the disease are not well defined but periods of incubation, of invasion and of eruptive and degenerative changes may be recognized.
The length of the stage of incubation is uncertain and difficult to exactly establish as there are no recognizable initial lesions in a vast majority of cases because it is probably that inoculation does not usually occur through the skin. A person coming from a leprous to a non-leprous country without any signs of the disease, who develops prodromal or eruptive symptoms after an interval. This indefinite interval may vary from a few weeks to years, even running as high as ten, twenty or forty years. If a patient resides some years in a leprous country, it is manifestly impossible to fix the date of the beginning of an incubation ending years later. The wide difference in the induration of the incubating period no doubt is due to personal idiosyncrasy as modified by food, habits, climate, etc., but it is interesting to note that most of the cases of prolonged incubation have been in those who removed from an endemic region to one in which the disease was not prevalent.
The period of invasion, showing prodromal symptoms, follows the stage of incubation and averages several months to a year. During this period cases of tubercular leprosy will show intermittent febrile disturbances, together with modification of the sensory or motor functions, drowsiness, depression and general weakness. Hemorrhages, general or local disorders of the sweat functions, headaches, vertigo and other less suggestive phenomena are noted. Neurotic prodromata are especially common in the anesthetic form but even here they vary greatly in character and intensity. They may be so mild as to escape notice or they may take the form of more or less severe but in constant and shifting, itching, formication, tingling, smarting, burning, stiffness, numbness and pain.
Fig. 195 – Leprosy of the nodular or tubercular type, in a West Indian Woman.
The eruptive stage of nodular or tubercular leprosy first shows as a few or many, large or small spots resembling ordinary erythema. The color is yellowish-brown or reddish but disappears on pressure. the patches may be limited to one region or distributed over the body and may spontaneously disappear and reappear. The affected skin feels slightly swollen and tense, is somewhat hyperesthetic and produces a quantity of sweat. Febrile symptoms, disappear upon the full development of the macular eruption which finally fades away, often leaving no trace behind. However, some of the spots fail to resolve, become more pigmented, sharply marked and are the seat of the first true infiltration. These nodules are split-pea in size and shape, of the same color as the macules and may remain stationary or progressively enlarge. The increased deposit is often preceded by fever and the new tubercles appear in crops.
Fig. 196 – Leprosy of the tubercular variety in a native of the West Indies.
The favorit locations of the eruption are the face especially the inner angle of the eyebrows, ears, nose, chin, cheeks and lips, although they are not uncommon upon the backs, the forearms and hands. The skin affected becomes thickened and elevated into round or flat, sharply outlined prominences which, though close together, are plainly distinct from each other. Later, coalescence of these lesions will present large soft, red, yellowish or brownish-red elevations and make the surface involved present a hideous appearance. This deformity is most marked on the face, producing the so-called “leonine” expression. While these infiltrations occasionally undergo absorption, leaving stains or scars to be succeeded by fresh crops in the same or other regions, it is common for them to progress in size and extent, becoming darker in color, for three or four years before ulceration ensues. Sometimes small hard masses may persist indefinitely or take on a keloidal form. Ulcerations of leprous growths are not uncommon, forming a shallow indolent ulcer which may or may not increase in size, be superficial or deep enough to destroy even tendons and bones. Lymph structures may be involved and even suppurate.
Mucous membranes may become affected early or late in the disease. Some authorities hold that leprosy first manifests itself in these regions while others hold with Impey that these parts are usually affected about four years after the advent of the tubercles in the skin and that they are attacked in the order nearest the surface going inward, viz., the lips, tongue, palate, fauces and larynx. Leprous ulcerations of these structures are both painful and persistent and cause a discharge that produces a characteristic sweetish odor.
Fig. 197 – Leprosy of the mixed variety with pronounced tubercular infiltrations on the face. Patient, a Greek of thirty years of age, who did not develop leprosy until resident in America.
Fig. 198 – Same as Fig. 197, showing the disappearance of the facial infiltration after receiving nastin-B for some months. After being observed for nearly a year, this case was discharged as cured.
Leprosy occurring in childhood and youth has produced a deficiency in hair formation, sexual development and general physical growth. While this form of leprosy may permit the patient to live many years, the ever-increasing intensity of the disease and the suffering therefrom reduce the patient’s endurance to such an extent that he falls an easy victim to some secondary affection such as pneumonia, bronchitis, erysipelas, kidney or gastrointestinal disorders. A few die directly from obstructing leprous growths in the throat while asthenia, consequent on the excessive drain from ulcerating sores, claims a few.
Fig. 199 – Leprosy of the maculo-anesthetic type in an adult male Pole. Much improvement in general health and total disappearance of cutaneous lesions was noted while taking Hydrocotyle 3x.
The eruptive stage of anesthetic leprosy begins with erythematous or bullous lesions. The stage of incubation is generally longer than in the tubercular form, the prodromata more distinctly neurotic and the course of the eruption less varied. The eruptive macules are similar to the primary lesions of tubercular leprosy but they are less hyperemic, more pigmented and persistent and tend to enlarge peripherally while they clear in the center. Chiefly noticed upon the trunk and extremities, the lesions are round or oval in shape; at first reddish-brown in color, they later become yellow, brown or sepia color. They may remain discrete or coalesce forming more or less extensive irregular or gyrate lesions characterized by sharply defined, slightly elevated, reddish borders with pale achromic centers. The center of the patch may become almost normal in color or even a shade darker than normal and desquamation of a variable degree may show upon a fully developed patch. On the other hand the skin may present a shiny or glossy appearance as the result of atrophy of the parts. At the onset the whole lesion may be hyperesthetic but subsequently this is limited to the advancing periphery while the central portion becomes more or less anesthetic. Loss of sensation may be found in apparently unchanged parts of the skin and occasionally hyperemia and pigmentation do not occur, and the apparent primary change is the absorption of the normal pigment. Rarely the skin becomes as white as in vitiligo.
Bullous eruptions occur chiefly on the extremities and often develop suddenly with or without sensations. They are of a variable size filled with a yellowish serum and rupture in a few hours leaving an excoriated surface. Rapid healing is the rule with pigmentation or scars as the evidence. It must here be noted that slight frictions and moderate heat may produce in leprous subjects blisters and ulcerations which in other people would not be caused by similar irritations.
Nerve changes are those of a multiple neuritis. The complex sensations of the skin may not all be lost at once. Thus the tactile sense may be present after those of temperature and pain are abolished or vice versa; or the sensation of cold lost or the opposite. However, in the more advanced cases all sensory functions disappear. Sweat and oil glands fail to produce, the hair turns white or falls out, the surface becomes dry and atrophic and hence secondary cutaneous lesions are often developed because of lowered vitality. As long as only the sensory nerves are affected, even though extensive, the disease may not affect the general health. Neuralgic pains, often paroxysmal in character and other annoying symptoms need not necessarily awaken suspicion of their grave character and the terrible results of further insidious nerve involvement may not show for many years. In advanced cases of leprosy, the nerves of the face and extremities are particularly attacked. The ulnar and peroneal nerves seem especially prone to involvement. Regular or interrupted thickenings of the ulnar nerve may sometimes be felt behind the olecranon in the earlier stages of this form of leprosy.
Complex nerve degeneration manifests itself as partial or complete paralysis, atrophy and deformity. Contractions are often noted in the hand where the first phalanx is so extended and the middle and terminal phalanges are so flexed from atrophy of the muscles and tendonous contraction as to produce the “leper claw.” Total loss of the sensory and trophic functions lead to other deformities and mutilations. Blebs appear over the phalangeal joints, ulcers destroy the deeper parts, gangrene may cause spontaneous amputation, or absorption of the bone may ensue so that one after another, the fingers are lost without ulceration or gangrene. The feet may undergo similar mutilation and deep plantar ulcers are not uncommon in those who go barefooted.
Fig. 200 – Leprosy of the anesthetic variety, showing characteristic deformities of the hands and wrists.
As the disease progresses the muscles of the extremities may become successively affected, those of the upper extremities much more so than those of the feet and legs. When the nerve supply of the face is attacked, patients may be unable to close the eyes from the paralysis of the orbicularis, the lachrymal secretion may cease causing irritation of the eyeball, the lids may become everted, the lips and cheeks may be flaccid, and partial or complete paralysis be evidenced by drooping of the muscles. When the sensory supply of the throat is affected, the functions of swallowing is so interfered with that regurgitation through the nose may be noted but, as a rule, the mucous membranes are fairly healthy in the case of pure anesthetic leprosy. The victims of this form of leprosy usually die from an intercurrent disease or from gastrointestinal disorders.
The eruptive stage of mixed leprosy usually includes lesions of both the tubercular and anesthetic forms, one or the other predominating. This variety is apt to be characterized by chronicity and severity from the combined presence of the features of both forms. This mixed type is more common in the United States than either of the pure varieties.
The duration of leprosy varies widely. Inasmuch as the tubercular form prevails most in a new country or among a virgin population, and the anesthetic in greater relative frequency with its continued prevalence in after years, it follows that the average duration may vary considerably in different countries or regions with the prevailing form of the disease. Roughly speaking it may be said that the average duration of tubercular leprosy is from five to six years, anesthetic from twelve to fifteen and mixed leprosy about ten years.
Fig. 201. Leprosy, mixed variety in a Chinaman, showing a gangrenous and ulcerative condition of the nose and right cheek, amounting to actual mutilation.
Etiology and Pathology – The bacillus leprae, discovered by Hansen in 1874, is the efficient cause but the method by which it grains access is not known. Observation would indicate that the mucous membrane of the nose and probably the mouth also was the common source of infection but it is possible that any abrasion of the skin could readily be the point of entrance. This latter fact is well shown because in tropical countries where people are prone to go barefooted, the first lesions are found on the feet in over half the cases. The anesthetic type is not as contagious as the tubercular. In fact the disease is only feeble contagious and is probably not directly contagious or inoculable from man to man. An intermediary host, such as has been observed in the causation of malaria, might explain many of the contradictory features of leprosy. Males are more subject to this disease than females in a proportion of two to one. It is rarely noted in childhood and never occurs in infancy. It is not hereditary, although a predisposition to it has been observed. Certain influences attributed to climate, race and sex may predispose to leprosy, but collective or personal habits of living, which include excesses of all kinds, bad hygiene, insufficient food and lack of cleanliness, appear to be the most important factors. Although leprosy exists in cold as well as hot climates, and moist, damp regions seem to favor its spread, the temperate climate of most of the United States and Europe is unfavorable to its development.
Fig. 202 – Leprosy, showing the results of complex nerve degeneration in the shape of spontaneous digital amputations, atrophic areas and pronounced dermatitis.
The causal bacilli resemble the tubercle bacilli closely but are found in groups, are more readily stained than the latter and are much more numerous in the surface lesions. After staining with fuchsin they appear as pink rods in length about one-half the diameter of a red blood corpuscle, in width about one- fifth their length. They may always be found in the tubercular nodules but are relatively absent in the anesthetic form. Whether it is the living bacilli or their toxins which produce the mischief is not known, but they have been found in the kidney, liver, spleen, sebaceous glands, hair-follicles and secretions of the mucous membranes when these parts have been attacked. The tubercular lesions are produced by deposits of cells in the corium and subcutaneous tissue, similar to the condition found in lupus and syphilis. They are diffuse granulomatous growths characterized by their limitation to the connective tissue elements, especially to the lymphatic system of the skin, and by their enormous number of organisms. In the anesthetic type, it is believed that the nerve changes are limited to the peripheral structures but recently central nerve involvement has been noticed.
Diagnosis – Advanced typical cases of leprosy are easily recognized in a leprous country or in those that have lived in such a country, but atypical sporadic cases may present some difficulty because their prodromata are not peculiar to leprosy.
Syphilitic macules are smaller, less colored, less permanent and do not seek the face. The nodules of syphilis are smaller, more grouped, of a deep red color, ulcerate more readily and are indiscriminate in their choice of location. It is well to remember that syphilis and leprosy may coexist.
Lupus vulgaris nodules are softer, smaller, more circumscribed, grouped in patches interspersed with the scar tissue, and do not produce the thickening of the eyebrows and ears found in leprosy.
Mycosis fungoides presents patches that are more red and eczematous than leprosy and its fungating, ulcerating growths develop upon the same patches at a later period of the disease.
Lupus erythematosus lacks the anesthesia and other neurotic symptoms of macular leprosy and is less apt to show multiple lesions.
Erythema multiforme usually manifests itself in smaller areas, does not become nodular, is acute in its evolution and presents successive gradations of color.
Tinea versicolor presents a fine scaliness of its patches without any sensory changes and the microscope will reveal its causal fungus.
Vitiligo spots are irregular in outline, whiter and more sharply defined by a pigmented border, and are normal in texture and sensibility.
Morphea may always be distinguished by its hard, lardaceous, waxy-white surface and its violaceous border.
Syringomyelia presents a loss of the heat and pain sensations without loss of the tactile sense and the facial muscles are not involved.
In general it may be said that doubtful nodular lesions should be examined microscopically for the fungus, while in anesthetic leprosy the loss of sensation and the regularity of the enlargement of the ulnar and other nerves is diagnostic.
Prognosis – There is no doubt that incipient leprosy can be cured but it is a question whether this is due to treatment or the natural course of the disease. The usual result is fatal although there may be pronounced remissions, only leading to a renewal of the disease with reinforced vigor. The typical duration of the disease has been noted before and should always be included in the prognosis. The removal to a non-leprous country, proper food, hygiene, local and internal treatment will no doubt extend the life of the patient and occasionally result in apparent cure.