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Leprosy
Leprosy
What is leprosy?
Leprosy is a disease caused by the bacteria Mycobacterium leprae that causes damage
to the skin and the peripheral nervous system. 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 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 nine-
banded armadillos. The disease is termed a chronic granulomatous disease because it
produces inflammatory nodules (granulomas) in the skin and nerves over time.
Picture of a person with leprosy (Hansen's disease)
Unfortunately, the history of leprosy and its interaction with man is one of suffering and
misunderstanding. The newest research suggests that at least as early as 4000 B.C.
individuals had been infected with M. leprae (studies are ongoing to prove this by
genetic analysis), while the first known written reference to the disease was found on
Egyptian papyrus in about 1550 B.C. The disease was well recognized in ancient China,
Egypt, and India. Because the disease was poorly understood, very disfiguring, slow to
show symptoms, and had no known treatment, many cultures thought the disease was
a curse or punishment from the gods. Consequently, leprosy was left to be "treated" by
priests or holy men, not physicians.
Since the disease often appeared in family members, some people thought it was
hereditary; other people noted that if there was little or no contact with infected
individuals, the disease did not infect others. Consequently, some cultures considered
infected people (and occasionally their close relatives) as "unclean" or as " lepers" and
ruled they could not associate with uninfected people. Often infected people had to
wear special clothing and ring bells so uninfected people could avoid them.
The Romans and the Crusaders brought the disease to Europe, and the Europeans
Unfortunately, the early signs and symptoms of leprosy are very subtle and occur slowly
(usually over years). Numbness and loss of temperature sensation (cannot sense very
hot or cold temperatures) are some of the first symptoms that patients experience. As
the disease progresses, the sensation 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-time developing sequence of events begins and
continues on the cooler areas of the body (for example, hands, feet, face, and knees).
There are multiple forms of leprosy described in the literature. The forms of leprosy are
based on the person's immune response to M. leprae. A good immune response can
produce the so-called tuberculoid form of the disease, with limited skin lesions and
some asymmetric nerve involvement. A poor immune response can result in the
lepromatous form, characterized by extensive skin and symmetric nerve involvement.
Some patients may have aspects of both forms. Currently, two classification systems
exist in the medical literature: the WHO system and the Ridley-Jopling system. The
Ridley-Jopling system is composed of six forms or classifications, listed below
according to increasing severity of symptoms:
• 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
• 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.
• 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
However, the WHO further modifies these two classifications with clinical criteria
because "�of the non-availability or non-dependability of the skin-smear services. The
clinical system of classification for the purpose of treatment includes the use of number
of skin lesions and nerves involved as the basis for grouping leprosy patients into
multibacillary (MB) and paucibacillary (PB) leprosy." Investigators state that up to about
four to five skin lesions constitutes paucibacillary leprosy, while about five or more
constitutes multibacillary leprosy.
Mulitdrug therapy (MDT) with three antibiotics (dapsone, rifampicin, and clofazimine) is
used for multibacillary leprosy, while a modified MDT with two antibiotics (dapsone and
rifampicin) is recommended for paucibacillary leprosy. Paucibacillary leprosy usually
includes indeterminate, tuberculoid, and borderline tuberculoid leprosy from the Ridley-
Jopling classification, while multibacillary leprosy usually includes the double (mid-)
borderline, borderline lepromatous, and lepromatous leprosy.
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
Ziel-Nelson stain or the Fite stain (biopsy) can diagnose multibacillary leprosy, or if
bacteria are absent, diagnose paucibacillary leprosy. Other tests can be done, but most
of these are done by specialized labs and may help a clinician to place the patient in the
more detailed Ridley-Jopling classification and are not routinely done (lepromin test,
phenolic glycolipid-1 test, PCR, lymphocyte migration inhibition test or LMIT). 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.
The majority of cases (mainly clinically diagnosed) are treated with antibiotics. The
recommended antibiotics, their dosages and length of time of administration are based
on the form or classification of the disease and whether or not the patient is supervised
by a medical professional. In general, paucibacillary leprosy is treated with two
antibiotics, dapsone and rifampicin, while multibacillary leprosy is treated with the same
two plus a third antibiotic, clofazimine. Usually, the antibiotics are given for at least six to
12 months or more. Each patient, depending on the above criteria, has a schedule for
their individual treatment, so treatment schedules should be planned by a clinician
knowledgeable about that patient's initial diagnostic classification.
Antibiotics can treat paucibacillary leprosy with little or no residual effects on the patient.
Multibacillary leprosy can be kept from advancing, and living M. leprae can be
essentially eliminated from the person by antibiotics, but the damage done before
antibiotics are administered is usually not reversible. Recently, the WHO suggested that
single-dose treatment of patients with only one skin lesion with
rifampicin, minocycline (Minocin), orofloxacin (Floxin) is effective. Studies of other
antibiotics are ongoing.
The role for surgery in the treatment of leprosy occurs after medical treatment
(antibiotics) has been completed with negative skin smears (no detectable acid-fast
bacilli) and is often only needed in advanced cases. Surgery is individualized for each
patient with the goal to attempt cosmetic improvements and, if possible, to restore limb
function and some neural functions that were lost to the disease.
Prevention of contact with droplets from nasal and other secretions from patients with
untreated M. leprae infection currently is a way to avoid the disease. Treatment of
patients with appropriate antibiotics stops the person from spreading the disease.
People that live with individuals that have untreated leprosy are about eight times as
likely to develop the disease, because investigators speculate that family members
have close proximity to infectious droplets. Leprosy is not hereditary.
Many people get exposed to leprosy throughout the world, but the disease in not highly
contagious; researchers suggest that over 95% of exposures result in no disease. In the
U.S., there are about 200-300 new cases diagnosed per year, with most coming from
exposures during foreign travel. The majority of worldwide cases are found in the tropics
or subtropics (for example, Brazil, India, and Indonesia). The WHO reports about
500,000 to 700,000 new cases per year worldwide, with curing of about 14 million cases
since 1985.
http://www.cdc.gov/nczved/dfbmd/disease_listing/leprosy_ti.html
http://www.who.int/lep/classification/en/index.html
http://www.who.int/lep/mdt/en/
http://emedicine.medscape.com/article/1104977-overview
http://www3.niaid.nih.gov/topics/leprosy/Understanding/today.htm
• Leprosy is a slowly developing, progressive disease that damages the skin and
nervous system.
• Early symptoms begin in cooler areas of the body and include loss of sensation.
• Signs of leprosy are painless ulcers, skin lesions of hypopigmented macules (flat,
pale areas of skin) and eye damage (dryness, reduced blinking). Later, large
ulcerations, loss of digits, skin nodules, and facial disfigurement may develop.
• Hansen's Disease
Leprosy
Leprosy is an infectious disease that has been known since biblical times. It is
characterized by disfiguring skin sores, nerve damage, and progressive debilitation.
Causes
Leprosy is caused by the organism Mycobacterium leprae. It is not very contagious
(difficult to transmit) and has a long incubation period (time before symptoms appear),
which makes it difficult to determine where or when the disease was contracted.
Children are more susceptible than adults to contracting the disease.
Leprosy has two common forms, tuberculoid and lepromatous, and these have been
further subdivided. Both forms produce sores on the skin, but the lepromatous form is
most severe, producing large, disfiguring lumps and bumps ( nodules).
All forms of the disease eventually cause nerve damage in the arms and legs, which
causes sensory loss in the skin and muscle weakness. People with long-term leprosy
may lose the use of their hands or feet due to repeated injury resulting from lack of
sensation.
Symptoms
Symptoms include:
• Skin lesions that are lighter than your normal skin color
○ Lesions have decreased sensation to touch, heat, or pain
○ Lesions do not heal after several weeks to months
• Numbness or absent sensation in the hands, arms, feet, and legs
• Muscle weakness
Treatment
A number of different antibiotics are used to kill the bacteria that cause the disease.
Outlook (Prognosis)
Early recognition is important. Early treatment limits damage by the disease, renders
the person noninfectious (you can't catch the disease from them), and allows for a
normal lifestyle.
Possible Complications
• Cosmetic disfigurement
• Permanent nerve damage
Prevention
Prevention consists of avoiding close physical contact with untreated people. People on
long-term medication become noninfectious (they do not transmit the organism that
causes the disease).
Alternative Names
Hansen's disease
Lepros
y
• Leprosy ranges from mild (with one or a few skin areas affected) to
severe (with many skin areas affected and damage to many
organs).
• Rashes and bumps appear, the affected areas become numb, and
muscles may become weak.
Because without treatment, people with leprosy are visibly disfigured and
often have significant disability, they have long been feared and shunned
by others. Although leprosy is not highly contagious, rarely causes death,
and can be effectively treated with antibiotics, it still causes anxiety. As a
result, people with leprosy and their family members often suffer
psychologic and social problems.
During 2007, over 250,000 new cases were reported. About 90% of these
cases occurred in the following eight countries (from the most cases to
the least): India, Brazil, Indonesia, Congo, Bangladesh, Nigeria, Nepal,
and Ethiopia. In 2006, 137 new cases were reported in the United States.
Cases occurred in 30 states, but over half occurred in six states:
California, Florida, Louisiana, Massachusetts, New York, and Texas.
Almost all cases of leprosy in the United States involve people who
emigrated from developing countries.
Leprosy can develop at any age but appears to develop most often in
people aged 5 to 15 years or over 30.
About 95% of people who are infected with Mycobacterium leprae do not
develop leprosy because their immune system fights off the infection.
People who develop leprosy may have genes that make them susceptible
to the infection once they are exposed.
Tuberculoid
Leprosy
Symptoms
Because the bacteria that cause leprosy multiply very slowly, symptoms
usually do not begin until at least 1 year after people have been infected.
On average, symptoms appear 5 to 7 years after infection. Once
symptoms begin, they progress slowly.
Leprosy affects mainly the skin and peripheral nerves Characteristic
rashes and bumps develop. Infection of the nerves makes the skin numb
or the muscles weak in areas controlled by the infected nerves.
The most severe symptoms result from infection of the peripheral nerves,
which causes deterioration of the sense of touch and a corresponding
inability to feel pain and temperature. People with peripheral nerve
damage may unknowingly burn, cut, or otherwise harm themselves.
Repeated damage may eventually lead to loss of fingers and toes. Also,
damage to peripheral nerves may cause muscle weakness that can result
in deformities. For example, the fingers may be weakened, causing them
to curve inward (like a claw). Muscles may become too weak to flex the
foot—a condition called footdrop. Infected nerves may enlarge so that
during a physical examination, doctors can feel them.
Skin infection can lead to areas of swelling and lumps, which can be
particularly disfiguring on the face.
• Feet: Sores may also develop on the soles of the feet, making
walking painful.
Diagnosis
Symptoms (such as distinctive rashes that do not disappear, enlarged
nerves, loss of the sense of touch, and deformities that result from
muscle weakness) provide strong clues to the diagnosis of leprosy.
• If leprosy is severe,
people may have to
take antibiotics for the
rest of their life.
Prevention
Because leprosy is not very contagious, risk of spread is low. Only the
untreated lepromatous form is contagious, although even then the
infection is not easily spread. Once treatment has begun, leprosy cannot
be spread. Avoiding contact with bodily fluids from and the rash on
infected people is the best prevention. The BCG (bacille Calmette-
Guérin) vaccine, used to prevent tuberculosis, provides some protection
against leprosy, but it is not often used to prevent leprosy.
Treatment
Antibiotics can stop the progression of leprosy but do not reverse any
nerve damage or deformity. Thus, early detection and treatment are
vitally important. Because some leprosy bacteria are resistant to certain
antibiotics, doctors prescribe more than one drug. The drugs chosen
depend on the type of leprosy:
, rifampin
, andclofazimine
and clofazimine
plus clofazimine
, ofloxacin
, and minocycline
.
Dapsone
. Its most serious side effects are damage to the liver and flu-like
symptoms. Clofazimine
Mortality/Morbidity
If severe and left untreated, leprosy can cause clinically significant and debilitating
deformity. Since 1943, when sulfone was introduced as the first effective treatment for
leprosy, antibiotic treatment has dramatically improved patients' outcomes. Early
diagnosis and effective antimicrobial treatment can arrest and even cure leprosy.
Race
Leprosy occurs in persons of all races. African blacks have a high incidence of the
tuberculoid form of leprosy. People with light skin and Chinese individuals tend to
contract the lepromatous type of leprosy. Leprosy is endemic in Asia, Africa, the Pacific
basin, and Latin America (excluding Chile). Leprosy is more a rural than urban disease.
Sex
In adults, the lepromatous type of leprosy is more common in men than in women after
puberty, with a male-to-female ratio of 2:1. In children, the tuberculoid form of leprosy
predominates and no sex preference is reported. Women tend to have a delayed
presentation, which increases rates of deformity.
Age
Leprosy has a bimodal age distribution, with peaks at ages 10-14 years and 35-44
years. Leprosy is rare in infants. Children appear to be most susceptible to leprosy and
tend to have the tuberculoid form.
Leprosy is a chronic bacterial disease of the skin and nerves in the hands
and feet and, in some cases, the lining of the nose. Leprosy is a rare disease
in the United States.
Anyone can get leprosy, but children seem to be more susceptible than
adults.
It is not clear how the leprosy germ is spread, but household and prolonged
close contact is important. The germs probably enter the body through the
nose and possibly through broken skin. The germs get in the air through
nasal discharge of untreated lepromatous patients.
Tuberculoid leprosy symptoms are a few well-defined skin lesions that are
numb. Lepromatous leprosy symptoms are a chronically stuffy nose and
many skin lesions and nodules on both sides of the body.
It usually takes about four years for tuberculoid leprosy symptoms to appear
and about eight years for lepromatous leprosy symptoms to appear.
In most cases, a person will not infect others after about three months of
starting treatment.
Patients with leprosy should be treated by a doctor who has experience with
the disease. Treatment is with multiple drugs for six months to two years.
The best way to prevent the spread of leprosy is the early diagnosis and
treatment of people who are infected. For household contacts, immediate
and annual examinations are recommended for at least five years after last
contact with a person who is infectious.