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Leprosy

Leprosy is a chronic bacterial infection with Mycobacterium leprae. It primarily affects the skin, mucous membranes
(e.g. nose), peripheral nervous system (nerve function), eyes and testes. The form the disease takes depends on
the person's immune response to the infection.
Leprosy is also known as Hansen disease and is one of the oldest known diseases of mankind. It is curable but if
untreated can lead to severe deformities.
Types of leprosy
There are several forms of leprosy that range from the mildest indeterminate form to the most severe lepromatous
type. More severe forms arise because of less effective immune response to the infection. Most of those infected
mount an appropriate immune response and never develop signs of leprosy.
Depending on clinical features, leprosy is classified as:
Indeterminate leprosy (IL)
Tuberculoid leprosy (TT)
Borderline tuberculoid leprosy (BT)
Borderline borderline leprosy (BB)
Borderline lepromatous leprosy (BL)
Lepromatous leprosy (LL)
Patients with indeterminate leprosy, a very early form of leprosy, may either be cured or progress to one of the
other forms of leprosy depending on their immune status. Within each type of leprosy, a patient may remain in
that stage, improve to a less debilitating form or worsen to a more debilitating form depending on their immune
state. Lepromatous leprosy is the only form that never reverts to a less severe form.
Who is at risk of leprosy?
Leprosy can affect people of all races all around the world. However, it is most common in warm, wet areas in the
tropics and subtropics. Worldwide prevalence is reported to be around 5.5 million, with 80% of these cases found
in 5 countries: India, Indonesia, Myanmar, Brazil and Nigeria. In New Zealand we most often come across cases
from Samoa, Tahiti and the Cook Islands.
Leprosy presents most often during two different periods of life, between the ages of 10 and 14 and in those aged
35-44 years old. It is rarely seen in infants.
What are the signs and symptoms of leprosy?
Once infected with the mycobacteria, the average incubation period is two to three years, but it can range from 6
months to 40 years or longer. In 90% of patients the first sign of the disease is a feeling of numbness, which may
precede skin lesions by a number of years. Temperature is the first sensation lost, followed by light touch, pain and
then deep pressure. Sensory loss usually begins in the extremities (toes and fingertips).
Borderline tuberculoid leprosy

The first skin lesion is usually the indeterminate type, which causes one or a few hypopigmented (pale) spots
before evolving into the borderline, tuberculoid or lepromatous types.
Leprosy type Features
Tuberculoid
Can be either one large red patch with well-defined raised borders or a large hypopigmented asymmetrical spot
Lesions become dry and hairless
Loss of sensation may occur at site of some lesions
Tender, thickened nerves with subsequent loss of function are common
Spontaneous resolution may occur in a few years or it may progress to borderline or rarely lepromatous types
Borderline
tuberculoid
Similar to tuberculoid type except that lesions are smaller and more numerous
Disease may stay in this stage or convert back to tuberculoid form, or progress
Borderline borderline
Numerous, red, irregularly shaped plaques
Sensory loss is moderate
Disease may stay in this stage, improve or worsen
Borderline
lepromatous
Numerous lesions of all kinds, plaques, macules, papules and nodules. Lesions looking like inverted saucers are common
Hair growth and sensation are usually not impaired over the lesions
Lepromatous
Early nerve involvement may go unnoticed
Numerous lesions of all kinds, plaques, macules, papules and nodules
Early symptoms include nasal stuffiness, discharge and bleeding, and swelling of the legs and ankles
Left untreated, the following problems may occur:
Skin thickens over forehead (leonine facies), eyebrows and eyelashes are lost, nose becomes misshapen or collapses, ear lobes thicken, upper incisor teeth
fall out
Eye involvement causing photophobia (light sensitivity), glaucoma and blindness
Skin on legs thickens and forms ulcers when nodules break down
Testicles shrivel causing sterility and enlarged breasts (males)
Internal organ infection causing enlarged liver and lymph nodes
Voice becomes hoarse due to involvement of the larynx
Slow scarring of peripheral nerves resulting in nerve thickening and sensory loss. Fingers and toes become deformed due to painless repeated trauma.
How is the diagnosis made?
Leprosy has very characteristic clinical features but the diagnosis must be confirmed because of the need for
prolonged treatment with antibiotics. A skin biopsy may show characteristic granulomas (mixed inflammatory cell
infiltrate in the deeper layers of the skin, the dermis) and involvement of the nerves. Special staining of the tissue
may show acid fast bacilli, the number visible depending on the type of leprosy.
The bacteria may also be found in lepromatous leprosy on smears taken from skin slits made in the ear lobes, but
the smears will be negative in the tuberculoid or borderline forms of the disease.
What is the treatment for leprosy?
Management of leprosy is aimed at stopping infection and minimising potential physical deformities. Antibiotics
used first-line to eliminate organisms include dapsone, rifampicin and clofazimine. Varying regimens with multidrug
therapy (MDT) are used depending on the type of leprosy and the severity of infection. This may be a combination
of two or three antibiotics given over varying lengths of time (up to years). Other antibiotics include minocycline,
ofloxacin and clarithromycin.
Oral corticosteroids and thalidomide are helpful in preventing nerve damage by reducing swelling. Long courses are
necessary to decrease severity of deformities and disabilities.
Surgery may sometimes be used to drain abscesses to restore nerve function, reconstruct collapsed nose, or to
improve function or appearance of affected areas.
Patient education is paramount. Leprosy can be cured but it is essential to take the full course of medication. It is
no longer infectious once treatment has begun. Patients should be instructed how to deal with existing nerve
damage for example protecting numb feet from injury. Physical, social and psychological rehabilitation is a
necessary for those in whom neglected disease has caused havoc.
Lepra reactions
Lepra reactions occur in 30-50% of patients with leprosy. They may occur before, or more often, after the start of
treatment and are induced by medicines, stress and surgical procedures. There are 3 main types of reaction.
Lepra type I (reversal) reaction
Lepra type I (reversal) reaction often affects those with with borderline shifting toward tuberculoid type, as the
cell-mediated immune system improves in the first few months of drug treatment or for some other reason such as
pregnancy.
Type 1 reactions result in fever, red swollen skin, and tender peripheral nerves. Treatment requires oral
corticosteroids and nonsteroidal anti-inflammatories.
Lepra type II reaction
Lepra type II reaction is also called erythema nodosum leprosum (ENL). It affects those with with borderline
lepromatous or lepromatous leprosy and is a humoral (antibody-antigen) reaction to immune complexes. Repeated
episodes tend to occur later in treatment than the type I reaction, usually after several years.
ENL presents as painful red nodules (lumps), which can blister or ulcerate, accompanied by fever, malaise, joint
pain, nerve pain, eye disease and involvement of other organs. Treatment may include clofazimine, thalidomide,
corticosteroids, colchicine, ciclosporin or tumour necrosis factor antagonists.
Lucio type II phenomenon
Lucio phenomenon is a cutaneous vasculitis in patients with lepromatous leprosy and tends to affect people who
have not taken their medication regularly. It is difficult to treat and regimens must include MDT.
Lucio phenomenon presents as odd-shaped red patches and ulcers on hands, wrists, ankles and feet. It is
associated with fever, arthritis, liver and kidney disease.

http://www.dermnetnz.org/bacterial/leprosy.html

Leprosy
Fact sheet N101
September 2012

Key facts
Leprosy is a chronic disease caused by a bacillus, Mycobacterium leprae.
Official figures show that almost 182 000 people, mainly in Asia and Africa, were affected at the beginning of 2012, with approximately 219 000
new cases reported during 2011.
M. leprae multiplies very slowly and the incubation period of the disease is about five years. Symptoms can take as long as 20 years to appear.
Leprosy is not highly infectious. It is transmitted via droplets, from the nose and mouth, during close and frequent contacts with untreated cases.
Untreated, leprosy can cause progressive and permanent damage to the skin, nerves, limbs and eyes.
Early diagnosis and treatment with multidrug therapy (MDT) remain the key elements in eliminating the disease as a public health concern.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, an acid-fast, rod-shaped bacillus. The disease mainly affects the skin,
the peripheral nerves, mucosa of the upper respiratory tract and also the eyes.
Leprosy is curable and treatment provided in the early stages averts disability.
Multidrug therapy (MDT) treatment has been made available by WHO free of charge to all patients worldwide since 1995, and provides a simple
yet highly effective cure for all types of leprosy.
Leprosy today
Leprosy control has improved significantly due to national and subnational campaigns in most endemic countries. Integration of primary leprosy
services into existing general health services has made diagnosis and treatment of the disease easy. The implementation of the global leprosy
strategy 20112015 national leprosy programmes now focus more on underserved populations and inaccessible areas to improve access and
coverage. Since control strategies are limited, national programmes actively improve case holding, contact tracing, monitoring, referrals and
record management.
According to official reports received from 105 countries and territories, the global registered prevalence of leprosy at the beginning of 2012
stood at 181 941 cases. The number of cases detected during 2011 was 219 075 compared with 228 474 in 2010.
Pockets of high endemicity still remain in some areas of Brazil, Indonesia, Philippines, Democratic Republic of Congo, India, Madagascar,
Mozambique, Nepal, and the United Republic of Tanzania. All endemic countries remain highly committed to eliminating the disease, and
continue to intensify their leprosy control activities.
Brief history - disease and treatment
Leprosy was recognized in the ancient civilizations of China, Egypt and India. The first known written mention of leprosy is dated 600 BC.
Throughout history, the afflicted have often been ostracized by their communities and families.
Although leprosy was treated differently in the past, the first breakthrough occurred in the 1940s with the development of the drug dapsone,
which arrested the disease. But the duration of the treatment was many years, even a lifetime, making it difficult for patients to follow. In the
1960s, M. leprae started to develop resistance to dapsone, the worlds only known anti-leprosy drug at that time. In the early 1960s, rifampicin
and clofazimine, the other two components of recommended multidrug therapy (MDT), were discovered.
In 1981, a WHO Study Group recommended MDT. MDT consists of 3 drugs: dapsone, rifampicin and clofazimine and this drug combination
kills the pathogen and cures the patient.
Since 1995, WHO provides free MDT for all patients in the world, initially through the drug fund provided by the Nippon Foundation and since
2000, through the MDT donation provided by Novartis and the Novartis Foundation for Sustainable Development.
Elimination of leprosy as a public health problem
In 1991 WHO's governing body, the World Health Assembly (WHA) passed a resolution to eliminate leprosy by the year 2000. Elimination of
leprosy is defined as a prevalence rate of less than 1 case per 10 000 persons. The target was achieved on time and the widespread use of MDT
reduced the disease burden dramatically.
Over the past 20 years, more than 14 million leprosy patients have been cured, about 4 million since 2000.
The prevalence rate of the disease has dropped by 90% from 21.1 per 10 000 inhabitants to less than 1 per 10 000 inhabitants in 2000.
Dramatic decrease in the global disease burden: from 5.2 million in 1985 to 805 000 in 1995 to 753 000 at the end of 1999 to 181 941 cases at the
end of 2011.
Leprosy has been eliminated from 119 countries out of 122 countries where the disease was considered as a public health problem in 1985.
So far, there has been no resistance to antileprosy treatment when used as MDT.
Efforts currently focus on eliminating leprosy at a national level in the remaining endemic countries and at a sub-national level from the others.
Actions and resources required
In order to reach all patients, leprosy treatment needs to be fully integrated into general health services. Moreover, political commitment needs to
be sustained in countries where leprosy remains a public health problem. Partners in leprosy elimination also need to continue to ensure that
human and financial resources are available.
The age-old stigma associated with the disease remains an obstacle to self-reporting and early treatment. The image of leprosy has to be changed
at the global, national and local levels. A new environment, in which patients will not hesitate to come forward for diagnosis and treatment at any
health facility, must be created.
WHO response
The WHO Strategy for leprosy elimination contains the following:
ensuring accessible and uninterrupted MDT services available to all patients through flexible and patient-friendly drug delivery systems;
ensuring the sustainability of MDT services by integrating leprosy services into the general health services and building the ability of general
health workers to treat leprosy;
encouraging self-reporting and early treatment by promoting community awareness and changing the image of leprosy;
monitoring the performance of MDT services, the quality of patients care and the progress being made towards elimination through national
disease surveillance systems.
Sustained and committed efforts by the national programmes along with the continued support from national and international partners have led
to a decline in the global burden of leprosy. Increased empowerment of people affected by the disease, together with their greater involvement in
services and community, will bring us closer to a world without leprosy.

http://www.who.int/mediacentre/factsheets/fs101/en/index.html

Leprosy Today

Child with leprosy
Today, the diagnosis and treatment of leprosy is easy and most endemic countries are striving to fully integrate leprosy services into
existing general health services. This is especially important for those under-served and marginalised communities most at risk from
leprosy, often the poorest of the poor.


Access to information, diagnosis and treatment with multidrug therapy (MDT) remain key elements in the strategy to eliminate the
disease as a public health problem, defined as reaching a prevalence of less than 1 leprosy case per 10,000 population. MDT
treatment has been made available by WHO free of charge to all patients worldwide since 1995, and provides a simple yet highly
effective cure for all types of leprosy.
According to official reports received during 2011 from 130 countries and territories, the global registered prevalence of leprosy at
the beginning of 2011 stood at 192,246 cases, while the number of new cases detected during 2010 was 228,474 (excluding the
small number of cases in Europe).
Most countries that were previously highly endemic for leprosy have achieved elimination at the national level and are intensifying
their efforts at regional and district levels. During 2007, the Democratic Republic of the Congo and Mozambique reached elimination
at the national level, and were joined by Timor-Leste by the end of 2010. However, pockets of high endemicity still remain in some
foci in Angola, Brazil, the Central African Republic, India, Madagascar, Nepal and the United Republic of Tanzania and in previously
highly endemic countries, such as the Democratic Republic of the Congo and Mozambique. These countries remain highly
committed to eliminating the disease, and continue to intensify their leprosy control activities.
Information campaigns about leprosy in high risk areas are crucial so that patients and their families, who were historically
ostracized from their communities, are encouraged to come forward and receive treatment. The most effective way of preventing
disabilities in leprosy, as well as preventing further transmission of the disease, lies in early diagnosis and treatment with MDT.
http://www.who.int/lep/en/

Causes of leprosy
Leprosy is one of the oldest recorded diseases, mentioned in Ancient Egyptian papyri from 1550 BC. It's caused by
the bacteria Mycobacterium leprae, very slow-growing bacteria similar to that which causes tuberculosis.
There are three types of the disease. The generalised form, lepromatous, attacks peripheral nerves, the skin, the
hands and feet, the mucous membranes (such as the lining of the nose) and the eyes. In contrast, the tuberculoid
form is localised, so its effects are less widespread across the body. The third type is known as borderline or
dimorphous leprosy, and is has characteristics of both other forms.
Leprosy is passed from person to person by droplets from the nose and mouth of untreated patients with severe
disease. However it's not highly infectious.
There has been a dramatic decrease in the number of cases recorded. According to the World Health
Organization (WHO) the figure has decreased from approximately 5.2 million worldwide in 1985 to 192,246 in
2011. Many countries where it was once very common are now reaching targets for elimination of the disease. It
remains most prevalent in poverty-stricken areas of in Asia, Africa and Latin America. Leprosy occurs twice as
often in men as women.
Top
Symptoms of leprosy
Leprosy is a chronic infectious disease that attacks the nervous system, particularly the nerves of the hands, feet
and face. In lepromatous leprosy, skin lesions typically develop in areas of nerve damage. These may be pale,
having lost pigment, or may develop a reddish copper colour. As the sensory nerves become damaged, sensation is
lost to pin-prick or light touch. Sensation loss starts at the fingers and toes and may only affect a small patch of
skin to begin with, but as time passes many skin lesions and nodules develop.
Another feature of lepromatous leprosy is a chronically stuffy nose, as the lining membranes become involved. The
nerves themselves may become thickened and there may also be muscle weakness in the area related to the
damaged nerve. The loss of sensation can lead to unnoticed injuries, which may in turn become infected. In
advanced cases, gangrene may set in with loss of body tissues. With time there may be significant damage to the
body.
Tuberculoid leprosy tends to be much more limited and symptoms usually consist of a few well-defined skin lesions
that are numb. Sensation loss may only affect a small patch of skin. Again, the loss of sensation can lead to
unnoticed injuries, infection and tissue damage.
Dimorphous leprosy creates skin lesions characteristic of the lepromatous and tuberculoid forms.
Another important classification in leprosy relates to the number of skin samples showing positive for bacteria. If
skin lesions are all negative for bacteria, it is known as paucibacillary leprosy, while people with positive smears
from any lesion are said to have multibacillary leprosy. Different drug treatments are used for these two types.
Top
Treatments for leprosy
Leprosy is curable, but the effectiveness of the treatment is dependent on an early diagnosis (before tissue damage
occurs). Diagnosis these days is very simple.
Since 1982, the WHO has recommended a 6-12 month course of multidrug therapy (MDT), which it provides free
throughout the world. Patients are given a course of three strong antibiotics (dapsone, rifampicin and clofazimine)
for multibacillary leprosy, and two drugs (rifampicin and dapsone) for paucibacillary leprosy. MDT can completely
cure multibacillary leprosy within 12 months and the paucibacillary form within six months. A combination of drugs
must be used because the leprosy bacteria can develop resistance to antibiotics given individually as single
treatments. MDT is highly effective, has few side effects and problems such as resistance or relapse are very rare.
If treatment is started early, deformities and disabilities can be prevented. MDT also makes the few highly
infectious patients non-infectious very quickly, which helps prevent the spread of leprosy. In a significant
percentage of cases (especially in tuberculoid leprosy), the disease will eventually burn-out or spontaneously go
into remission, even if specific treatment hasn't been given. However, much damage may have occurred by then.
Since 1985 more than 15 million people have been cured of leprosy. However, because most cases of the disease
occur in difficult to reach places, many people are still not treated early enough to prevent damage occurring.
There is currently no effective vaccine for leprosy. Efforts have been made to develop a vaccine based on the
current TB vaccine (as it is caused by a similar micro-organism) but this hasn't been very successful and although
research continues to tackle the issues, experts estimate that a leprosy vaccine is still some 10-20 years away.
http://www.bbc.co.uk/health/physical_health/conditions/leprosy.shtml
Biological Characteristics
Infectious Agent
Leprosy is cause by infection with an intercellular pathogen known asMycobacterium
leprae. M. leprae is a strongly acid-fast, rod-shaped bacterium. It has parallel sides
and rounded ends, measuring 1-8 microns in length and 0.2-0.5 micron in diameter,
and closely resembles the tubercle bacillus. M. leprae has the longest doubling time of
all known bacteria (13 days) which makes doing laboratory research (in vitro) on this
organism quite difficult. Therefore, innoculation of the foot pad of the mouse and the
armadillo are the main mechanisms for research. The organism infects the skin
because it thrives at temperatures slightly lower than that found inside the human
body. It also has an affinity for nerve cells, which is why leprosy is characterized by
loss of feeling on the skin surface. M. leprae is the only mycobacterium known to
infect nervous tissue.

A photomicrograph of M. leprae from a leprosy skin lesion (CDC)
Reservoir
Besides humans, the only known resevoir is the armadillo. It is thought that they are
a good host for Mycobacterium leprae because of their low body temperature.
http://genomenewsnetwork.org/articles/02_01/Leprosy.shtml
Armadillos have become the main source of M. leprae for genetic, biochemical, and
immunological research including development of a vaccine.
Approximately 5% of armadillos in Louisiana have naturally occurring clinical disease.
About 20% have serologic evidence of infection with organisms indistinguishable
from M. leprae. However, only occasional cases are reported among individuals
handling armadillos.
Naturally occurring infection also has been reported in non-human primates including
the African chimpanzee, sooty mangabey, and cynomolgus macaque.
Vector
It is uncertain whether or not insects can act as a vector for M. leprae. Acid-fast
bacilli, like M. leprae, have been demonstrated in biting insects.Successful
transmission of M. leprae by intracutaneous inoculation in the mouse footpad model
has been reported. However, the question whether insects actually transmit the
infection remains unanswered.
Transmission
Although there remains some uncertainty about the mode of transmission of leprosy,
most researchers agree that it is spread from person to person in respiratory droplets
or nasal discharge. M. leprae may survive outside a human host for a period of hours
or even days. Only the lepromatous form of the disease is thought to be infectious.
While human-to-human respiratory tramsmission is thought to be the likely cause of
most infections, exposure to insect vectors, infected soil, and animal reservoirs may
also be possible modes of transmission.
Most people are immune to leprosy. In endemic areas, subclinical levels of the disease
are common, but only in a select few cases will the infection progress to clinical
disease levels.
Increased Risk Groups
People that live in close contact with patients who have untreated, active,
predominately multibacillary leprosy and people living in countries with endemic
leprosy are at an increased risk of infection.

http://www.stanford.edu/group/parasites/ParaSites2005/Leprosy/bio.htm

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