LEPROSY

Definition Leprosy or Hansen's disease (HD) is a chronic disease caused by the bacteria Mycobacterium leprae , leprosy is primarily a granulomatous disease of the peripheral nerves and mucosa of the upper respiratory tract; skin lesions are the primary external sign . The disease develops slowly (from six months to 40 years!) and results in skin lesions and deformities, most often affecting the cooler places on the body (for example, eyes, nose, earlobes, hands, feet, and testicles). The skin lesions and deformities can be very disfiguring and are the reason that infected individuals historically were considered outcasts in many cultures. Although human-to-human transmission is the primary source of infection, three other species can carry and (rarely) transfer M. leprae to humans: chimpanzees, mangabey monkeys, and ninebanded armadillos. The disease is termed a chronic granulomatous disease, it produces inflammatory nodules (granulomas) in the skin and nerves over time. Causes: Mycobacterium leprae and Mycobacterium lepromatosis are the causative agents of leprosy. An intracellular, acid-fast bacterium, M. leprae is aerobic and rodshaped, and is surrounded by the waxy cell membrane coating characteristic of Mycobacterium species.

Mode of transmission: Portal of entry: Droplet infection: Leprosy may be transmitted via aerosala containing M. leprae Contact transmission;

Leprosy is transmitted from person to person by close contactbetween an infectious patient and susceptible person.this contact can be direct or indirect. Portal of entry; Through droplet infection Incubation period: Leprosy has a long Incubation period of an average of 3-5 years. Risk factors At highest risk are those living in endemic areas with poor conditions such as inadequate bedding, contaminated water, and insufficient diet, or other diseases that compromise immune function.

Classifications of leprosy:

The Ridley-Jopling system is composed of six forms or classifications, listed below according to increasing severity of symptoms:

Indeterminate leprosy: a few hypopigmented macules; can heal spontaneously, persists or advances to other forms Tuberculoid leprosy: a few hypopigmented macules, some are large and some become anesthetic (lose pain sensation); some neural involvement in which nerves become enlarged; spontaneous resolution in a few years, persists or advances to other forms Borderline tuberculoid leprosy: lesions like tuberculoid leprosy but smaller and more numerous with less nerve enlargement; this form may persist, revert to tuberculoid leprosy, or advance to other forms Mid-borderline leprosy: many reddish plaques that are asymmetrically distributed, moderately anesthetic, with regional adenopathy (swollen lymph

nodes); the form may persist, regress to another form, or progress

Borderline lepromatous leprosy: many skin lesions with macules (flat lesions) papules (raised bumps), plaques, and nodules, sometimes with or without anesthesia; the form may persist, regress or progress to lepromatous leprosy

Lepromatous leprosy: Early lesions are pale macules (flat areas) that are diffuse and symmetric; later many M. leprae organisms can be found in them. Alopecia (hair loss) occurs; often patients have no eyebrows or eyelashes. As the disease progresses, nerve involvement leads to anesthetic areas and limb weakness; progression leads to aseptic necrosis (tissue death from lack of blood to area), lepromas (skin nodules), and disfigurement of many areas including the face. The lepromatous form does not regress to the other less severe forms. Histoid leprosy is a clinical variant of lepromatous leprosy that presents with clusters of histiocytes (a type of cell involved in the inflammatory response) and a grenz zone (an area of collagen separating the lesion from normal tissue) seen in microscopic tissue sections.

The 2009 WHO classifications are simply based on the number of skin lesions as follows:

Paucibacillary leprosy: skin lesions with no bacilli (M. leprae) seen in a skin smear Multibacillary leprosy: skin lesions with bacilli (M. leprae) seen in a skin smear

Symptoms and signs Unfortunately, the early signs and symptoms of leprosy are very subtle and occur slowly (usually over years). The symptoms are similar to those that may occur with syphilis, tetanus, and leptospirosis. Numbness andloss of temperature

sensation (cannot sense very hot or cold temperatures) are some of the first symptoms that patients experience. As the disease progresses, the sensations of touch, then pain, and eventually deep pressure are decreased or lost. Signs that occur, such as relatively painless ulcers, skin lesions of hypopigmented macules (flat, pale areas of skin), and eye damage (dryness, reduced blinking) are experienced before the large ulcerations, loss of digits, and facial disfigurement develop. This long-term developing sequence of events begins and continues on the cooler areas of the body (for example, hands, feet, face, and knees). Painless skin patch accompanied by loss of sensation but not itchiness (Loss of sensation is a feature of tuberculoid leprosy, unlike lepromatous leprosy, in which sensation is preserved.

Loss of sensation or paresthesias where the affected peripheral nerves are distributed Wasting and muscle weakness Foot drop or clawed hands (may result from neuritic pain and rapid peripheral nerve damage How is leprosy diagnosed? The majority of cases of leprosy are diagnosed by clinical findings, especially since most current cases are diagnosed in areas that have limited or no laboratory equipment available. Hypopigmented patches of skin or reddish skin patches with loss of sensation, thickened peripheral nerves, or both clinical findings together often comprise the clinical diagnosis. Skin smears or biopsy material that show acid-fast bacilli with the Ziehl-Neelsen stain or the Fite stain (biopsy) can diagnose multibacillary leprosy, or if bacteria are absent, diagnose paucibacillary leprosy. Other tests such as CBC test, liver function tests, creatinine test, or a nerve biopsy may be done to help determine if other organ systems have been affected.

Treatment:
In response to the increased incidence of dapsone resistance, the WHO introduced a multidrug regimen in 1981 that includes rifampicin, dapsone, and clofazimine. Some clinical studies have also shown that certain quinolones, minocycline, and azithromycin have activity against M leprae. The WHO recently recommended

single-dose treatment with rifampin, minocycline, or ofloxacin in patients with paucibacillary leprosy who have a single skin lesion. However, the WHO still recommends the use of the long-term multidrug regimens whenever possible because they have been found to be more efficacious.

Typeof Leprosy Paucibacillary

Daily, Administered Dapsone 100 mg

Self- Monthly Supervised Rifampicin 600 mg

Months Treatment 6-12

of

Multibacillary

Dapsone100mg,

Rifampicin 600mg,

24

Clofazimine50mg Clofazimine 300mg

US regimens emphasize the use of rifampin, which is the most bactericidal drug used to treat leprosy. Although a single dose of 600 mg once monthly (the WHO standard) is considered bactericidal, treatment plans in the United States may include doses of 600 mg/day.

Paucibacillary leprosy should be treated for 6-12 months with dapsone 100 mg/day unsupervised plus rifampin 600 mg/month supervised. This regimen should be followed by treatment with dapsone as monotherapy for 3 years in

patients with tuberculoid leprosy or 5 years in patients with borderline lepromatous leprosy. Multibacillary leprosy should be treated for 24 months with dapsone 100 mg/day unsupervised, clofazimine 50 mg/day unsupervised, and rifampin 600 mg plus clofazimine 300 mg/month supervised. Corticosteroids have been used to treat nerve damage associated with leprosy, but a recent review of 3 randomized controlled trials shows no significant long-term effect. Prednisolone is believed to minimize pain and acute inflammation. The recommended initial dose is prednisolone 40 mg daily. Observations of increasing resistance in patients treated for leprosy have been reported in Southeast Asia, notably in Vietnam. The drug most commonly found to be resistant is dapsone, often in the context of prior exposure or treatment attempts with monotherapy. Although drug resistance is an ongoing concern, it is difficult to assess in this slow-growing organism. In a study of M leprae strains from South America, few of 230 strains subjected to molecular drugsusceptibility analysis were drug-resistant. Of the 230 strains, 3 were identified as clinically relapsing and were found to be resistant by genetic testing; 2 of the 3 were dapsone-resistant; and 1 was dapsone-resistant and rifampin-resistant using genetic testing for point mutation. How is leprosy prevented? Prevention of contact with droplets from nasal and other secretions from patients with untreated M. leprae infection currently is a way recommended to avoid the disease. Treatment of patients with appropriate antibiotics stops the person from spreading the disease. There is no commercially available vaccine available to prevent leprosy. However, there are reports of using BCG vaccine, the BCG vaccine along with heat-killed M. leprae organisms, and other preparations that may be protective or help to clear the infection or to shorten treatment. Except for BCG in some countries, these preparations are not readily available.