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LEPROMATOUS (Multibacilliary) LEPROSY
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LEPROMATOUS (LL) or MULTIBACILLIARY (MB) LEPROSY
Lepromatous Leprosy (LL) is found in those patients with no or very little immune resistance to the Micobacterium leprae organism. They are not able to mount a response because of a lack of Cell-Mediated Immunity (CMI) . In such cases, the very defence cell - the macrophage - which is meant to destroy the bacillus, through phagocytosis (engulfing/digesting) actually is a good environment for the bacillus and plays the role of host, enabling the bacillus to multiply within the cell. It has been known for up to 300 M.leprae to fit into one macrophage which swells up like a balloon instead of retaining its oemeba-like shape. The macrophage, which is meant to contain the spread of the disease by ingesting and digesting such foreign organisms, becomes a convenient vehicle for the M.leprae to be transported, in the blood stream, to all parts of the body. Whereas we used the key word “localised” to describe the contained or curbed Tuberculoid (TT) leprosy - a “Paucibacilliary” type , in relation to Lepromatous (LL) leprosy - a “Multibacilliary” type , we may use words like “PROFUSE” or “PROLIFEROUS” or wide-spread. Wherever the M.leprae are deposited by the macrophage, perhaps after it has ruptured, the bacilli, colonise the locality, grow and produce lesions. They remain free to be taken up by other macrophages to be transported further afield, and so on. Besides the peripheral nerves and skin, the nasal mucosa and that of the upper respiratory tract, along with the anterior (cooler front) part of the eye, also are infiltrated by bacilliferous macrophages.
When a biopsy of the affected LL skin is examined pathologically, it shows large collections of macrophages with foamy cytoplasm, called “Foam Cells” (they also are called “Lepra Cells” or “Virchow Cells). Along with these, there are “Plasma Cells” and a few lymphocytes. Such collections of cells, initially are seen around the hair follicles, sweat and sebaceous glands, blood-vessels and nerves. As the disease proliferates, they cover the whole sub-epithelial tissue, forming a band-like infiltrate. Acid-fast Zeihl Neelsen stain reveals M.leprae inside the macrophages.
As the disease progresses, the sweat and sebaceous glands, along with the hair follicles become subject to pressure atrophy. In large areas of the skin, sweating is impaired, hair ceases to grow from the body, particularly the cooler, eye-brow region and eye-lashes. The skin may also take on a “puckered” appearance, like that of an orange-peel, being also dry and wrinkled. Whereas, in Tuberculoid leprosy pressure atrophy is due to a large collection of lymphocytes and epithelioid cells, even in the relatively early stages of the disease, in Lepromatous leprosy, pressure atrophy occures in the advanced stages of the disease due to the proliferation of different cells, viz:- macrophages, “Foam Cells” and “Plasma Cells”
Nerves are affected in lepromatous leprosy but LATER in time than in Tuberculoid leprosy. In lepromatous leprosy, anaesthesia in the skin and paralysis of the peripheral nerve trunks may not develop until late in the course of the disease, perhaps after many years. Whereas in Tuberculoid leprosy, the affected nerves may contain very few M.leprae, yet be paralysed due to a strong CMI immune response, in lepromatous leprosy, acid-fast M.leprae may be seen in large numbers inside the macrophages, even in the perineural and Schwann Cells , without the nerve suffering much impairment until late in the course of the disease. This is clear evidence that it is not so much the leprosy bacilli that are causing the nerve damage and consequent paralysis, but the body’s immune system violently reacting to the antigens liberated from the cell wall of the dying and dead M.leprae.
There are several varieties of Lepromatous leprosy viz:- Macular, Diffuse, Infiltrated, Nodular, Polyneuritic
CLINICAL MANIFESTATIONS OF MACULAR LEPROMATOUS LEPROSY (LL):-
(1) Generally present on Face, Buttocks, Trunk and the extensor aspect of the extremities
(2) Patches are multiple, or vaguely erythematous / hypopigmented. Distribution is symetrical
(3) Texture of skin - waxy appearance, smooth and erythematous
(4) Margin of patch NOT well defined (as in TT) but merges into surrounding area
(5) Initially, patches do NOT have sensory deficit
(6) Sweating may be lost in lesions but later than in TT.
(7) To compensate, the body’s cooling system makes the sweatable areas sweat excessively
(8) Nerves are not thickened.
(9) Skin smears and nasal scrapings Bacteriologically POSITIVE.
NODULAR LEPROMATOUS LEPROSY:-
(1) “Nodular” is the most advanced form of lepromatous leprosy
(2) The Nodules are rounded and circumscribed
(3) Nodules are of varying size and shape
(4) LL Nodules are not tender
(5) LL Nodules progress very slowly, taking several months to develop
(6) The Nodules are found in mucous membrane and in the skin
(7) They are most common on the face and ear-lobes
(8) When LL nodules ulcerate, they discharge large numbers of M.leprae
(9) These are the TRUE “leprous” or “lepromatous” ulcers, unlike those on hands and feet which are secondary and due to injuries such as burns etc. “Histoid” nodules, described by Wade, in 1963, are somewhat rare and painless. They are well circumscribed, soft and highly positive for acid-fast M.leprae bacilli.
CLINICAL MANIFESTATIONS OF ADVANCED LEPROMATOUS LEPROSY:-
While in the early stages of LL leprosy there may be little nerve involvement and, consequently, little if any sensory deficit and paralysis, a fine infiltration may be seen when the patient’s skin is examined in the bright sunlight. In advanced LL cases, the following is manifest:-
(1) Nerve involvement including “Glove and Stocking “ type of anaesthesia in late stage. The main nerves involved are Ulnar, Median, Radial, Lateral Popliteal, Posterior Tibial and Facial . Especially in times of reaction (Erythema Nodosum Leprosum or ENL) the nerves may be tender and very painful.
(2) Nasal involvement including :- a) Crust formation; b) Blocking of the nose; c) Ulceration of the nasal mucosa; d) Epistaxis or nose-bleeding; e) Maggots in the nose if patient has not inserted nose plugs to keep out flies; f) Destruction of the nasal septum; g) Depression of the bridge of the nose.
(3) The Larynx may be involved as follows:- a) Epiglotis infiltrated by M.leprae also affecting vocal cords and producing a hoarseness of voice and sometimes a squeeky , female-type voice.; b) especially during ENL reaction, there may be severe oedema of the larynx. In some rare, chronically neglected cases, when laryngeal oedema causes obstruction of the airways, tracheotomy may be necessary to enable the patient to breathe - a most traumatic form of suffering .
(4) The eye may be seriously affected, involving :- a) the Conjunctiva and Slera where lepromatous nodules may be seen; b) Epislera; c) Cornea with keratitis leading to visual impairment and sometimes total blindness; d) Iris - especially during ENL reaction, acute iridocylitis may be experienced, leading to red and painful eyes and sometimes severe glaucoma. In some cases chronic iridocyclitis may result in intraoccular pressure and glaucoma.
(5) In the male, the testes may actually be destroyed, leading to Gynocomastia (female type breasts) and Gynocotelia (female type nipples).
(6) E.N.L. Reaction in Lepromatous Leprosy is most traumatic. Contrary to the belief that “leprosy patients don’t experience pain”, in the case of ENL reaction, nodules of the intradermal and subcutaneous types are most painful and tender. Often these nodules appear in “crops” and often they are associated with acute Iridocyclitis and acute neuritis. In very severe cases, only the controversial drug Thalidomide, (not given to women able to bear children) provides the sufferer with any measure of relief.
(7) Bones are often involved, especially during and after ENL reaction. Fingers may become misshapen because they softened during prolonged inflammation at time of reaction, when fingers must be encased in a plaster cast to keep them in proper shape.
(8) In some rare cases of chronic inflammation and oedema, the patient may suffer from a “Frozen Hand” due to fibrosis.
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