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LEPROSY

HANSEN DISEASE Aetiology Mycobacterium leprae Mode of transmissionClose contact Direct transmission nasal secretions ? Indirect - ? Insects such as mosquitoes or other insect

Introduction
Despite first discovering Mycobacterium leprae (the bacteria that causes leprosy) in 1873, leprosy research scientists still do not completely understand how leprosy is spread.

Most scientists believe that leprosy can be spread from person to person in infected respiratory droplets. While this may be one way in which leprosy is spread, more than 50 percent of the people who develop leprosy have no confirmed contact with an infected person.
Factors that may influence how leprosy is spread include:

Environmental conditions The degree of susceptibility of the person

The extent of exposure.

Incubation period
Although the incubation period is typically between three and five years, it can range from six months to several decades.

Risks
People who live within the same household as a person with untreated leprosy have an eightfold increased risk of developing the disease. genetic factors relating to susceptibility and/or prolonged intimate contact.

The spouse is the least-at risk family member. The people who are at the greatest risk of leprosy transmission are: Parents of someone with leprosy Children of someone with leprosy Brothers or sisters of someone with leprosy.

Clinical Disease Basically, leprosy is a disease of the nervous system but not all nerves are affected. The central nervous system is spared but the peripheral and cutaneous nervous systems can be infiltrated by M.leprae bacilli and then, secondarily, the skin is affected.

History
In order for the doctor to make a diagnosis of leprosy, he or she will likely ask questions about:

Symptoms +

1. 2. 3. 4. 5.

Current medical conditions Medications Travel history Family history of any medical conditions Possible exposure to someone with leprosy.

A proper diagnosis, therefore, will require the presence of the following three CARDINAL SIGNS:-

1. one or more hypopigmented, anaesthetic skin patches, typical of leprosy; 2. one or more thickened peripheral nerves; or 3. a positive skin smear.

SKIN:
1. Erythematous or Hypo-pigmented patch of skin with loss of

sensation to either/or/and touch, pain, temperature. 2. Smooth, oily, shiny and oedematous appearance of the skin 3. Diffuse erythema of the skin

4. On a shiny, erythematous and oedematous skin, there may be nodules or papules


5. There may be a sudden onset of painful erythematous nodules as above. 6. There may be a thickening of the earlobes 7. The eyebrows may become thin or even disappear, beginninng from the outerside- alopecia.

Anaethesia 90% of patient present with numbness first, sometimes years before the skin lesions appear. Temperature is the first sensation that is lost. Patients cannot sense extremes of hot or cold. The next sensation lost is light touch, then pain, and finally deep pressure. These losses are especially apparent in the hands and feet.

NERVES:
1. There may be an area of anaesthesia or numbness with/or pins and needles, ants crawling or tingling in any area. 2. In the hands or feet, there may be a weakening of the small muscles and/or the presence of disability in those areas.

3. Thickening and/or tenderness of the peripheral and cutaneous nerves


4. Weakening or loss of function of the sweating mechanism in those areas

1. 2. 3. 4. 5. 6. 7.

Nerves to feel Supra trocheal nerve Auricular nerve Ulnar Lateral popliteal Redial Median Superficial radial

three other tests:-

1. Skin Examination by HistoPathology. If the tissue is stained for acid-alcohol-fast M.leprae, the bacilli may be seen in the nerve. The need for such examination is rare

2. Histamine Test Histamine Phosphate in one drop (1:1000) is

placed on an area of normal skin of the patient and pricked with a needle. If the nerve is intact, there will be a weal and an erythematous flare/ There will be a weal as in the first test but NO erythematous flare IF the nerve is damaged, indicating a case of H.D. This test is rarely needed and is performed only as a last resort.

3. Sweat Test:
Inject Pilocarpine Nitrate (1:1000) intradermally both in normal skin and skin with a suspected lesion of H.D. If the lesion is affected by M.leprae, sweating is absent. This can be determined by applying Tr. Iodine on the skin, allowing to dry and then covering the area with starch before injecting . A positive sweat response will turn the starch blue, indicating that the sweating mechanism is intact.

In most cases, this is done by feeling with the back of the fingers for coolness of the normal, moist skin, in comparison with the warmness of dry skin affected by M.leprae.

There are two types of lepra reactions Lepra type 1 reactions:Borderline leprosy patients may develop a flare-up of inflammation in existing leprosy skin lesions or newly infected skin and/or nerves(neuritis). This is associated with a fever and pain of the affected region. This reaction commonly happens during therapy and is then also called "reversal reaction". It is due to T helper cells that produce large quantities of cytokines such as interferon-gamma. Corticosteroids have to be taken for several months to mitigate this reaction.

Lepra type 2 reactions: Approximately 50% of patients with lepromatous leprosy will develop a painful condition called erythema nodosum leprosum (ENL) in the first years of successful leprosy therapy. ENL is due to circulating immune complexes and vasculitis involving inflammatory cells, T helper cells and tumor necrosis factor released by these cells. This inflammatory process eventually leads to skin ulceration and also to inflammation of lymph glands (lymphadenitis), arthritis, glomerulonephritis (an inflammatory kidney disease) and orchitis (=inflammation of testicles). Other target tissues are the bone marrow and the liver, which leads to hemolytic anemia and abnormal liver function tests.

Milder cases are treated with aspirin, more severe cases with a brief course of corticosteroid medication.
If there are recurrent lepra type 2 reactions the treatment of choice is thalidomide (although this cannot be given to pregnant women or if pregnancy is a possibility because of fetal malformations from the drug).

There are two principle forms of leprosy. These are: Lepromatous leprosy (multibacillary leprosy). Occurs in patients with decreased function of the immune system. This form is characterized by many skin lesions and the symmetrical (occurs on both sides of the body equally) involvement of the peripheral nerves (nerves outside the brain and spinal cord). Nerve involvement includes lack of feeling, muscle weakness and paralysis. Many of the bacteria that cause the disease can be found in the skin and nasal mucus. This is the most contagious form of leprosy. Unlike other forms of leprosy, lepromatous leprosy never reverts to a less debilitating form.

Tuberculoid leprosy (paucibacillary leprosy). Occurs in patients with normal immune function. This form is characterized by greater nervous involvement that is typically asymmetrical (occurs more on one side of the body than the other). Lack of feeling is common and skin lesions are few or absent. Few or no bacteria can usually be found in the lesions.

Borderline leprosy (dimorphous leprosy) falls between these two variations. The signs, symptoms and findings of this form may fall anywhere in between lepromatous and tuberculoid leprosy, but it often leans toward one or the other. It can also become more serious, turning into either form if the body does not fight off the infection.

Indeterminate leprosy is an often very mild form with few or no skin lesions. It often resembles tuberculosis and frequently resolves on its own. However, the condition may progress to a more severe form in patients with weakened immune systems. Bacteria are found in very small numbers, if at all, in lesions.

Treatment of Leprosy
When lots of bacilli are present in biopsies, a combination of dapsone, clofacimine and rifampin is given for between 2 and 5 years. Multiple skin biopsies have to be negative before treatment is stopped. For cases with a paucity of bacilli on biopsies (tuberculoid leprosy) dapsone and rifampin are given for 6 months to 12 months.

PREVENTION
Personal hygeine Adequate nutrition BCG 30% of Tuberculoid type Adequate water supply