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Background

Leprosy is a chronic infection caused by the acid-fast, rod-shaped bacillusMycobacterium leprae. Leprosy
can be considered 2 connected diseases that primarily affect superficial tissues, especially the skin and
peripheral nerves. Initially, a mycobacterial infection causes a wide array of cellular immune responses.
These immunologic events then elicit the second part of the disease, a peripheral neuropathy with
potentially long-term consequences.
The social and psychological effects of leprosy, as well as its highly visible debilities and sequelae (as
seen in the image below), have resulted in a historical stigma associated with leprosy. To minimize the
prejudice against those with leprosy, the condition is also known as Hansen disease, named after G.A.
Hansen, who is credited with the 1873 discovery of M leprae. This mycobacterium grows extremely
slowly and has not been successfully cultured in vitro.

Hands with Z-thumbs, clawing, contractures, and shortening of fingers due to repetitive injury and healing. Ho Chi Minh City, Vietnam.
(Courtesy of D. Scott Smith, MD)

In the 1990s, the World Health Organization (WHO) launched a campaign to eliminate leprosy as a public
health problem by 2000. Elimination, as defined by the WHO, was defined as a reduction of patients with
leprosy requiring multidrug therapy to fewer than 1 per 10,000 population. This goal was achieved in
terms of global prevalence by 2002, but 15 of the 122 countries where leprosy was endemic in 1985 still
have prevalence rates of greater than 1 per 10,000 population. [1] Although multidrug regimens have been
used globally to cure nearly 14 million patients with leprosy since 1985, the number of new leprosy cases
remained relatively unchanged from 1980 to 2000, ranging from 500,000-700,000 worldwide per year.
[2]
Access and delivery of antibiotics continues to be a problem in the most endemic nations. With the
precise transmission mechanism of leprosy still unknown and a lack of an effective vaccine, leprosy will
probably continue to pose an ongoing public health problem in the coming decades. The goal of the WHO
by the end of 2015 is to reduce the rate of new cases with grade-2 disabilities worldwide by at least 35%.
This will be carried out by enforcing activities to decrease the delay in diagnosing the disease and actuate
treatment with multidrug therapy. This will also have the impact of reducing transmission of the disease
in the community.[2]
Pathophysiology
Leprosy can manifest in different forms, depending on the host response to the organism. Individuals who
have a vigorous cellular immune response to M leprae have the tuberculoid form of the disease that
usually involves the skin and peripheral nerves. The number of skin lesions is limited, and they tend to be
dry and hypoesthetic. Nerve involvement is usually asymmetric. This form of the disease is also referred
to as paucibacillary leprosy because of the low number of bacteria in the skin lesions (ie, < 5 skin lesions,
with absence of organisms on smear). Results of skin tests with antigen from killed organisms are positive
in these individuals. Individuals with minimal cellular immune response have the lepromatous form of the
disease, which is characterized by extensive skin involvement. Skin lesions are often described as

infiltrated nodules and plaques, and nerve involvement tends to be symmetric in distribution. The
organism grows best at 27-30C; therefore, skin lesions tend to develop in the cooler areas of the body,
with sparing of the groin, axilla, and scalp. This form of the disease is also referred to as multibacillary
leprosy because of the large number of bacteria found in the lesions (ie, >6 lesions, with possible
visualization of bacilli on smear). Results of skin tests with antigen from killed organisms are
nonreactive.
Patients may also present with features of both categories; however, over time, they usually evolve to one
or the other (indeterminate or borderline leprosy). Interestingly, most individuals who are exposed to
leprosy never develop the disease. Classification of leprosy: Leprosy has 2 classification schemas: the 5category Ridley-Jopling system and the simpler and more commonly used WHO standard. RidleyJopling: Depending on the host response to the organism, leprosy can manifest clinically along a spectrum
bounded by the tuberculoid and lepromatous forms of the disease. Most patients fall into the intermediate
classifications, which include borderline tuberculoid leprosy, midborderline leprosy, and borderline
lepromatous leprosy. The classification of the disease typically changes as it evolves during its
progression or management. The Ridley-Jopling system is used globally and forms the basis of clinical
studies of leprosy. It may also be more useful in guiding treatment regimens and assessing risk of acute
complications. Physical findings in each subtype are presented in the Clinical section. WHO system: The
WHO recommends classifying leprosy according to the number of lesions and the presence of bacilli on a
skin smear. This method is useful in countries where biopsy analysis in unavailable. Paucibacillary
leprosy is characterized by 5 or fewer lesions with absence of organisms on smear. Paucibacillary leprosy
generally includes the tuberculoid and borderline lepromatous categories from the Ridley-Jopling system.
Multibacillary leprosy is marked by 6 or more lesions with possible visualization of bacilli on smear.
Lepromatous leprosy, borderline lepromatous leprosy, and midborderline leprosy on the Ridley-Jopling
scale are included in the multibacillary leprosy category.
Frequency
United States
An average of 150-250 cases are diagnosed each year in the United states. [1] In 2010, according to the
Registry of National Hansens Disease Programs (NHDP), 205 new cases of leprosy were detected in the
United States.[3] In 2010 WHO reports, 169 new cases of leprosy were detected. The number of new cases
of MB leprosy reported was 105. The number of females among the new cases was 53, and 6 cases in
children were reported. No cases of relapse were reported in 2010. [2] Most cases of leprosy in the United
States are found in immigrants, although endemic foci exist in parts of Louisiana, Florida, and Texas
along the Gulf of Mexico; in Mexican and Asian California populations; and in Spanish Americans in
New York City. Around 75% of these detected leprosy cases involve patients who have lived in foreign
countries, primarily Asia, Africa, and Latin America. [3] Some cases among native US citizens can be
accounted for by exposure to leprosy overseas. Some cases can be attributed to a contact with a known
case of leprosy or exposure to infected armadillos.Based on genetic analysis studies, wild armadillos and
many patients with leprosy in the southern United States are infected with the same strain of M leprae.
[4]
Leprosy may be a zoonosis in the southern United States because armadillos are a large reservoir for
this disease. Nonetheless, history of exposure cannot be verified in many patients. [3]
International

According to WHO figures and as reported by 130 countries, the global annual detection rates have
declined from 2004-2010, when 407,791 and 228,474 new cases were reported, respectively (see the
images below). The prevalence registered worldwide at the beginning of 2010 was 192,246 cases. Of the
new cases, 95% were detected worldwide during 2010 in the following countries: Angola, Bangladesh,
Brazil, China, Democratic Republic of the Congo, India, Ethiopia, Indonesia, Madagascar, Mozambique,
Myanmar, Nepal, Nigeria, Philippines, Sri Lanka, Sudan, and United Republic of Tanzania. [2] These
countries still exhibit pockets of high endemicity.

Leprosy prevalence rates, data reported to WHO as of beginning January 2011. Courtesy of WHO, Leprosy: Global situation,
http://www.who.int/lep/situation/en/, accessed March 14, 2013.

Leprosy new case detection rates, data reported to WHO as of beginning January 2011. Courtesy of WHO, Leprosy: Global situation,
http://www.who.int/lep/situation/en/, accessed March 14, 2013.

Mortality/Morbidity
Leprosy is rarely fatal, and the primary consequence of infection is nerve impairment and debilitating
sequelae. According to one study, 33-56% of newly diagnosed patients already displayed signs of
impaired nerve function.[5]According to estimates, 3 million people who have completed multidrug
therapy for leprosy have sustained disability due to nerve damage. Although both lepromatous leprosy
and tuberculoid leprosy involve the skin and peripheral nerves, tuberculoid leprosy has more severe
manifestations. Nerve involvement results in loss of sensory and motor function, which may lead to
frequent trauma and amputation. The ulnar nerve is most commonly involved.

Damage in the following nerves is associated with characteristic impairments in leprosy:


Ulnar and median - Clawed hand
Posterior tibial - Plantar insensitivity and clawed toes
Common peroneal -Foot drop
Radial cutaneous, facial, and greater auricular nerves (may also be involved; as seen in
the image below)

Infiltration by bacteria may lead to destruction of nasal cartilage (lepromatous leprosy), ocular
involvement, and diffuse thickening of the skin. Advanced cases of leprosy involve the loss of eyebrows
and lashes, but these deformities are less common today.

Worldwide, leprosy is considered the most common cause of crippling of the hand, which is
caused by ulnar nerve involvement. [6] Peroneal nerve involvement can lead to foot drop, posterior tibial
nerve involvement, and clawed toes.
Race
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Leprosy was once endemic worldwide, and no racial predilection is known. In the late 1800s, the
incidence of leprosy in northern Europe and North America dropped dramatically, and the disease is now
reported primarily in tropical areas.
Sex
Leprosy is generally more common in males than in females, with a male-to-female ratio of 1.5:1. In
some areas in Africa, the prevalence of leprosy among females is equal to or greater than that in males. [2]
Age
Leprosy can occur at any age, but, in developing countries, the age-specific incidence of leprosy peaks in
children younger than 10 years, who account for 20% of leprosy cases. Leprosy is very rare in infants;
however, they are at a relatively high risk of acquiring leprosy from the mother, especially in cases of
lepromatous leprosy or midborderline leprosy.

Sumber:
Smith D.S. Leprosy. Available at : http://emedicine.medscape.com/article/220455-overview. Accessed on:
January, 11,2016.