J Cutan Pathol 2010: 37 (Suppl.

1): 125136
John Wiley & Sons. Printed in Singapore

Copyright 2010 John Wiley & Sons A/S

Invited Review

Global dermatopathology: Hansens

disease current concepts and
challenges
Dr Martin Mihm is an internist, a dermatologist, a
pathologist and a humanist. It is natural then that
the complexity of Hansens disease would interest
him not only as a medical subject but also as a
deeply overwhelming human condition of global
impact. His contributions to the field of Hansens
disease are therefore not limited to academic ones
such as a seminal paper describing in detail the light
microscopy of erythema nodosum leprosum (ENL)
that he coauthored with Dr George Murphy and
others.1 Rather, and perhaps more importantly, he
chose to experience the disease in the most direct
way possible by traveling to Titigar, nearby Kolkata
in India. There he volunteered with the Missionaries
of Charity Brothers, a branch of Mother Teresas
congregation. He worked for several weeks in the
leprosarium cared by the brothers, where besides
providing basic medical attention to those afflicted by
Hansens disease, he trained the brothers how to use
the extensive medical and surgical armamentarium
donated to the mission. He catalogued the drugs
and wrote detailed directions of how to diagnose
and treat Hansens disease, lepra reactions, short
and long-term complications, as well as other basic
medical conditions afflicting these patients. He also
went so far as to offer himself as a patient when
he taught minor surgical procedures, allowing the
brothers to remove some of his many moles. His time
in India, he recounts, was one of the most significant
medical experiences of his life. He often narrates this
experience when emphasizing the magnitude of the
effects of Hansens disease.
It is noteworthy that in the year that we are
celebrating Dr Mihms 75th birthday, with the plan
to write this article already under way, he approached
one of the authors (A. Piris) with his usual enthusiasm
relating the recent finding of the oldest human
skeleton with signs of Hansens disease, as well as the
discovery of a new etiologic agent for Lepromatous

Hansens disease. This anecdote highlights his ability

to keep tabs on discoveries from disparate specialties,
as he mainly focuses on pathology and dermatology
at this time.
With this brief introduction we would like to offer
the reader a succinct account of the basic aspects
of Hansens disease, with emphasis on the current
status of the disease throughout the world and in the
United States.
History
Hansens disease is one of those conditions that,
throughout recorded human history, has remained
a constant public health issue. Despite the fact
that not all diseases described in the bible were
actually leprosy, the stigma of this entity ensured
its proclamation in the holy books as a terrible
plague that brought upon those afflicted unparalleled
consequences.
The entity has been known to the medical
literature for almost 3000 years, with the first
description appearing in the Indian medical literature
in approximately 600 BC. Furthermore, in 2009,
a 4000-year-old skeleton showing traces of leprosy
was discovered in Rajasthan, India. This represents
the oldest case of leprosy ever found.2 Throughout
human history, Hansens disease has always been
stigmatized. Isolation of those afflicted was adopted
as the most viable medical approach to Hansens
disease because of its disfiguring complications, lack
of effective treatment and potential contagiousness;
isolation ultimately led to confinement in secluded
villages or colonies.
In the late 1930s, dapsone and its derivatives were
introduced as the first effective treatment. However,
about 30 years later, resistance was acknowledged
through the clinical and histological recognition of
histoid leprosy.3 In 1982, multiple drug therapy
(MDT) was implemented, which was very successful.

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Fig. 1. Cases of leprosy around the world. Modified from WHO. Prevalence of leprosy. http://www.who.int/lep/situation/prevalence/en/
index.html

Nine years later, in 1991, the World Health Assembly

established the goal of eliminating leprosy as a public
health problem by the turn of the millennium.
Elimination was defined as reaching a prevalence
of <1 case per 10,000 people.4
The success of this initiative was tremendous,
resulting in more than 10 million patients worldwide
being released as cured. Of the 122 endemic
countries, 107 reached the target prevalence of

126

<1 per 10,000 people.4 However, despite this

massive effort, leprosy still exists as a significant
public health issue in many countries, such as
Brazil, India, Angola, the Central African Republic,
the Democratic Republic of Congo, Madagascar,
Mozambique, Nepal and the United Republic of
Tanzania (Fig. 1). Even developed countries must
still face this public health challenge to some
degree.

Invited Review
Etiology
Until recently Mycobacterium leprae was the only known
organism to cause Hansens disease. It was first
described in 1873 by Armauer Hansen in Norway,
and it represents the first bacterium to be identified
as a human pathogen. The organism is an acid-fast
bacillus with a slightly curved rod shape. It is an
intracellular parasite that can only survive within
cells, especially Schwann cells and macrophages. As
M. leprae requires a temperature of approximately
35 C, the preferred affected sites are the cooler areas
of the body such as skin (especially acral areas),
peripheral nerves, mucous membranes, eyes and
testis. Transmission of the organism is believed to
occur mainly through nasal mucous secretions after
prolonged contact with untreated persons with active
disease.
The acid-fastness of M. leprae is weaker than in
other mycobacteriae. Skin smears for bacteriological
examination are routinely stained using the
ZiehlNeelsen method. However, in tissue sections,
a modified ZiehlNeelsen stain (Fite-Faraco) is most
commonly used, as it minimizes the exposure of the
microorganisms to organic solvents, thus protecting
its acid-fastness.5 Viable microorganisms stain bright
red, whereas degenerating bacilli stain irregularly
and eventually lose their acid-fastness completely.
The Gomori methenamine silver stain can reveal
nonacid fast debris of M. leprae.6,7
In December of 2008, Han et al. reported the
discovery of a new causative organism for Hansens
disease.8,9 The new organism is named Mycobacterium
lepromatosis. Although very similar to the M. leprae,
this new organism was shown to have several genetic
differences by polymerase chain reaction. These
unique attributes allowed its classification as a new
species. The new mycobacterium was discovered
in autopsy material from two patients with diffuse
lepromatous leprosy (LL). One of these patients was
originally from Mexico. Since this discovery, another
two cases of the diffuse form of leprosy were shown
to have been caused by the same agent.
Diagnosis
Currently, there are several approaches for
the diagnosis of leprosy. These include clinical
examination, skin smears, biopsy, inoculation of
footpads of mice, serologic and skin testing, and
molecular techniques. Because of the geographic
distribution of people with the disease, with most of
the endemic areas not having access to most of these
techniques, the diagnosis of leprosy has been based
primarily on clinical exam and skin smear.
A case of leprosy is defined, according to the
Seventh Meeting of WHO Expert Committee on

Leprosy in 1997, as an individual who in the absence

of complete treatment shows one or more of the
following signs:10,11
1. Hypopigmented or reddish cutaneous lesions
with definite loss of sensation.
2. Thickened peripheral nerves (Fig. 2).
3. Skin smear or biopsy testing positive for acidfast bacilli.
This simple scheme has some flaws. For example,
not all lesions show obvious hypopigmentation or
erythematous changes, and in multibacillary disease
the changes are not always anesthetic. Also, the
thickened peripheral nerves appear later than the
skin lesions. Furthermore, although highly specific,
the microscopic detection of acid-fast bacilli has low
sensitivity.10
Despite these pitfalls, application of all three
criteria in Ethiopia rendered a diagnostic sensitivity of
97%. Besides confirming the diagnosis, the detection
of positive smears is important in that they allow
identification of the patients with multibacillary
disease that is more infectious and at higher risk
of relapses and of developing a more serious form of
the disease.
Although some authors claim that histological
examination is the gold standard for diagnosis, that
method, too, is fallible.12 In a significant number of
cases, there are false negative biopsies and clinical
correlation is used.10 Nevertheless, biopsies remain
important for histologic diagnosis, classification,
research and the inoculation of the footpads of mice.
Classification
Leprosy has various clinical presentations, depending
on the hosts immune response to the microorganisms (Fig. 3). The internationally accepted RidleyJopling classification for leprosy uses clinical
and histopathological features and the bacteriological index. This classification recognizes five different
groups throughout a spectrum: tuberculoid (TT),
borderline tuberculoid (BT), borderline borderline
(BB), borderline lepromatous (BL) and lepromatous
(LL).13 In this spectrum, patients with TT leprosy
present a robust cell-mediated immunity against the
microorganisms, only a few skin lesions and a low
bacterial load. On the other end of the spectrum,
patients with LL leprosy have little or very weak
cell-mediated immunity, many skin lesions and a
high bacterial load. The majority of patients present
with one of the borderline forms of leprosy: BT,
mid-borderline (BB) or BL.14 These forms are more
difficult to distinguish and less stable, meaning that a
case of leprosy can shift from one form to another.15

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Fig. 2. Sites of common peripheral nerve involvement. Modified from Yoder LL., Guerra IE, Hansens Disease, a guide to management in
the United States, 2nd ed. Baton Rouge, Louisiana: Hansens Disease Foundation, 2001.

Because of the unavailability or unreliability of the

skin smear in many programs and the potential for
transmitting HIV disease and hepatitis when using
unsterile techniques, the World Health Organization
(WHO) has developed a classification based on
the number of skin lesions only.10 People with
five or fewer skin lesions are classified as having
paucibacillary (PB) leprosy, whereas people with
six or more skin lesions are classified as having

128

multibacillary (MB) leprosy.16 This classification is

often used in the field to decide what type of MDT
should be given to a patient with leprosy.17,18
Clinical features
Leprosy always involves peripheral nerves, almost
always involves skin, and frequently involves mucous
membranes15 . The three main signs of the disease are

Invited Review

Fig. 3. The spectrum of leprosy. Modified from Britton WJ. Leprosy. In Cohen J, Powderly WG, eds. Infectous diseases. London: Mosby, 2003.

skin lesions, skin anesthesia and enlarged peripheral

nerves. The great majority of people exposed
effectively resist the infection, even in highly endemic
areas. For those individuals unable to resist infection
with M. leprae, the incubation period varies from 2 to
5 or more years (Table 1).
Indeterminate leprosy
This represents the initial form of the disease. The
clinical presentation is usually a single or a few
hypopigmented skin macules with minimal sensory

loss confined to the lesion. In most cases, the

disease heals spontaneously. However, some cases
remain indeterminate for a prolonged period of time,
whereas some progress to one of the established forms
of the disease.15
Polar tuberculoid
Polar TT leprosy is the localized form of the disease. It is clinically characterized by one or a
few well-circumscribed skin lesions. In dark skin,
hypopigmentation predominates over the erythema

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Negative
Negative
Usually negative, may be
doubtful

Vague, merge imperceptibly with

the surrounding healthy skin
Not affected
Many (globi)
Vague, poorly defined
external border
Slightly diminished
Many

Strongly positive (3+)

Lepromatous leprosy
Polar LL leprosy is the widespread, anergic form
of the disease, with striking proliferation of bacilli.
The disease may be present for many years before
diagnosis, and the earliest skin changes are widely and
symmetrically distributed macules.19 Flesh-colored
or erythematous papules and nodules may be present.
If left untreated, the disease progresses with diffuse
infiltration of the skin. Dermal infiltration of facial
skin gives rise to the leonine facies. Eyebrows and
eyelashes can be lost (madarosis) (Fig. 4).
Systemic involvement is seen mainly in LL because
of bacillary infiltration affecting the upper respiratory
tract, oropharynx, kidneys, liver, spleen, bones and
testes.12,20 Infiltration of the nasal structures may lead
to a saddle deformity because of septal perforation
and destruction of the anterior nasal spine.19
Borderline leprosy
Borderline leprosy includes those types of the disease
between TT and LL. BT leprosy (BT) is similar to TT,
however, the number of skin lesions is greater, the
edges of the skin lesions are less well-defined, there is a
tendency for satellite lesions to develop near the larger
lesions and individual lesions tend to be larger.15 Usually, damage to peripheral nerves is more widespread
and more severe in BT than in TT. BL leprosy is
similar to LL, however, some of the skin lesions are
selectively anesthetic and show varying degrees of

Usually negative,
may be doubtful

Not always well

defined
Impaired
Usually negative
Definition of lesions

Lepromin test

Variable
Distribution of lesions

Sensation
Bacilli in skin lesions

Single or few
Number of lesions

Single or few (up to

five)
Localized,
asymmetrical
Well-defined, clear-cut
margins
Absent
Negative

Single or few satellite

lesions present
Not diffuse,
asymmetrical
Well-defined,
clear-cut margins
Absent
Negative or weakly
positive (1+)
Positive (2+)

Evident asymmetry

Vague, poorly defined

external border
Moderately diminished
Many

Macules, papules, nodules, diffuse

infiltration
Numerous, widely distributed,
practically no normal skin areas
Symmetrical
Macules, plaques,
papules, infiltration
Many, but normal skin
areas are present
Tends to be symmetry
Plaques and dome-shaped
punched-out lesions
Several, normal skin present
Macules
Type of lesion

Infiltrated patches

Infiltrated patches

Borderline lepromatous
Boderline tuberculoid
Tuberculoid
Indeterminate
Characteristics

Table 1. Classification of leprosy29

130

or copper color more commonly observed in lighter

skin.19 The lesions are anesthetic and can also be
hairless, scaly and dry. Additionally, there may be an
enlarged peripheral nerve in the vicinity of the skin
lesions.

Borderline borderline

Lepromatous

Invited Review

Fig. 4. Lepromatous leprosy. Diffuse infiltration of facial skin with

madarosis. Courtesy of Mirian N. Sotto, MD, PhD, Hospital das
Clinicas, University of Sao Paulo, Brazil.

Invited Review
distinctness in their borders. Peripheral nerve trunk
involvement is more widespread than in LL. On the
other hand, mucous membrane involvement is less
prominent in BL than in LL. Mid-borderline leprosy
(BB) is rare because it is very unstable.15
Histoid leprosy
The term histoid leprosy refers to a rare clinicopathological variant of multibacillary leprosy.3,21 It
was initially associated with dapsone resistance and
with relapse after dapsone monotherapy.22 24 In
fact, it has been observed in patients who relapse
after many years of apparently successful dapsone
monotherapy.25 However, this form of leprosy has
also been reported in patients with relapses after
MDT and in patients with leprosy who have not
received treatment.21,25 27 It is clinically characterized by the presence of cutaneous or subcutaneous, firm, erythematous or coppery, shiny papules,
nodules or plaques located on otherwise normalappearing skin.21,28
Lepra reactions
Lepra reactions are acute inflammatory processes
that can occur spontaneously or following a series
of triggering events such as antimicrobial therapy,
infections, pregnancy, physical or mental stress,
as well as many other unknown or undetectable
conditions. As peripheral nerves are common targets,
especially in BT leprosy, these events are medical
emergencies because of the irreversibility and longterm sequelae of nerve damage.30 Also, in LL the
involvement of target organs such as testis and eyes
can lead to infertility and blindness.
There are two types of lepra reactions:
Type 1 reactions: Also known as reversal reactions, type 1 reactions are the result of increased
cellular immunity and delayed hypersensitivity to
M. leprae antigens (upgrading reactions). These antigens have been found in the peripheral nerves and in
the skin of patients with this pattern of reaction. They
present throughout the entire spectrum of Hansens
disease with exception of indeterminate leprosy, but
predominantly in borderline disease, with the following clinical features: recrudescence of old skin
lesions and appearance of new ones, acute neuritis
and recent (less than 6 months) or progressive impairment of nerve function without pain.31 Less often,
type 1 reactions may be associated with downgrading.
Type 2 reactions: Also known as ENL occurs
in the lepromatous end of the spectrum (BL and
LL), in patients with high levels of circulating
antimycobacterial antibodies. These reactions are
the result of immune complex formation leading

to cutaneous and systemic vasculitis. Besides

the hallmark painful red nodules in the skin,
these patients may show the following clinical
features: fever, myalgia, arthritis, iridocyclitis,
hepatosplenomegaly, orchitis, lymphadenitis and
glomerulonephritis.31 Secondary amyloidosis can
also present in this setting.
Lucio phenomenon is yet another type of reactional state that develops in patients with LL leprosy.
First described in Mexico, it also occurs in Central
America and rarely in other regions. It is characterized by diffuse vasculopathy, with thrombosis,
as well as ulceration of the skin; the deep dermis,
subcutaneous tissue and even fascia are affected.
Histopathology
TT leprosy shows a dermal granulomatous infiltrate
composed by epithelioid cells and Langhans giant
cells, which are surrounded by lymphocytes. The
infiltrate is characterized by a linear pattern along
the distribution of the nerves (Fig. 5). In TT, the
infiltrate involves the papillary dermis up to the
epidermis, whereas BT and more LL forms have a
preserved grenz zone in the papillary dermis. Bacilli
are very difficult to find, even with special stains.
LL leprosy is histologically characterized by a
diffuse dermal infiltrate composed of macrophages
with a foamy appearance (Virchow cells or lepra
cells). This infiltrate is separated from the epidermis
by a grenz zone (Fig. 6). The Virchow cells
contain numerous bacilli and lipid droplets in their
cytoplasm.15 Both isolated and grouped (globi) bacilli,
detected by the Fite stain, are observed (Fig. 7).
In borderline leprosy, the histological findings are
a mixture of the features observed in the polar
forms (LL and TT). Observations include increased

Fig. 5. Tuberculoid leprosy. There is a prominent lymphohistiocytic

granulomatous infiltrate along dermal nerves. Courtesy of Mirian
N. Sotto, MD, PhD, Hospital das Clinicas, University of Sao
Paulo, Brazil.

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Fig. 6. Lepromatous leprosy. There is diffuse infiltration of the

dermis by large numbers of histiocytes, with sparing of the papillary
dermis (grenz zone). Courtesy of Mirian N. Sotto, MD, PhD,
Hospital das Clinicas, University of Sao Paulo, Brazil.

Fig. 7. Lepromatous leprosy. There are numerous bacilli, which are

isolated and in globi (Fite-Faraco). Courtesy of Mirian N. Sotto,
MD, PhD, Hospital das Clinicas, University of Sao Paulo, Brazil.

numbers of lymphocytes, more relation to nerves

and increased circumscription of the granulomatous
response as it approaches the polar TT form. In
subpolar LL leprosy, plasma cells are observed in
addition to histiocytes and lymphocytes.
Indeterminate leprosy shows a superficial and deep
dermal lymphohistiocytic infiltrate around blood
vessels, dermal appendages and nerves. This infiltrate
can be scarce and nonspecific. Bacilli, which are
usually not visualized, can sometimes be identified
either singly or in small groups in relationship to
small dermal nerves.
Histoid leprosy can histologically mimic a fibrous
histiocytoma. The characteristic lesion is wellcircumscribed and composed of numerous thin,
spindle-shaped histiocytes forming interlacing bands
and whorls. In most cases, a subepidermal grenz zone

132

is present. At a higher magnification, the histiocytes

may exhibit slight vacuolation of their cytoplasm, but
always to a lesser degree than in typical lepra cells.32
There are numerous bacilli, which are longer than
in ordinary leprosy. In addition, they are arranged
along the long axis of the spindle-shaped histiocytes
(histoid habitus).3
Lepra (type 1) upgrading reactions show edema,
increased numbers of lymphocytes and giant cells,
with the formation of small groups of epithelioid
cells. In downgrading reactions the lymphocytes and
epithelioid cells are replaced by macrophages, with
an increase in the number of bacilli.33
ENL is characterized by papillary dermal edema
and an intense inflammatory cell infiltrate in the
dermis and adjacent subcutaneous fat. The infiltrate
contains lymphocytes, numerous neutrophils and
Virchow cells. In addition, some authors describe
an associated vasculitis.34
Two histopathologic patterns have been described
in Lucios phenomenon. The first pattern is
characterized by a mild mononuclear infiltrate with
endothelial cell proliferation, vascular thrombosis
and necrosis. The vascular damage seen is thought
to be a result of direct invasion by bacilli. The second
pattern shows leukocytoclastic vasculitis, which is
thought to be an immune-mediated response to
mycobacterial or skin antigens.35,36 It has been
suggested that only the leukocytoclastic vasculitis
pattern represents a severe variant of ENL and is
associated with the presence of systemic symptoms.37
Current therapy and clinical management
The WHO recommends MDT based on the
classification of the patient as PB or MB (Tables
2 and 3). If no bacilli are found on bacilloscopy, the
patient is considered paucibacillary. On the other
hand, if at least one bacillus is identified, the patient
receives MB treatment. When bacilloscopy is not
available, the classification is based on the number of
skin lesions (Tables 2 and 3). After the first dose of the
MDT, the patient is no longer infectious to others.
The United States National Hansens Disease
Program (NHDP) recommends daily rifampin, and
a longer duration of treatment than is recommended
by the WHO, largely because of WHOs cost
considerations for developing countries (Table 4).39
The treatment of type 1 reactions intends to
control the acute inflammation, ease the pain and
prevent eye and nerve damage.19 The antileprosy
treatment should be continued during a reaction.
Mild cases are treated with analgesics. Oral corticosteroids are employed in severe cases or in patients
with neuritis. If nerve tenderness is observed, the
affected limb(s) should be rested.

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Table 2. WHO multidrug therapy for adults38

Multibacillary (more than

five lesions or more than
one nerve involvement)
Paucibacillary (25 lesions
or only one nerve
involvement)
Paucibacillary (single lesion)

Rifampin

Clofazimine

Dapsone

Ofloxacin

Minocycline

Therapy duration

600 mg once
a month

300 mg once a month

and 50 mg daily

100 mg daily

Twelve blister
packs
Six blister
packs

600 mg once
a month

100 mg daily

600 mg single
dose

400 mg single
dose

100 mg single
dose

Single dose

Table 3. WHO multidrug therapy for children38

10 to 14 years of age,
multibacillary
1014 years of age,
paucibacillary
For

Rifampin

Clofazimine

Dapsone

Ofloxacin

Minocycline

450 mg once a
month
450 mg once a
month

150 mg once a month and

50 mg every other day

Therapy duration

50 mg daily

Twelve blister packs

50 mg daily

Six blister packs

children younger than 10 years of age, the dose must be adjusted according to body weight

Table 4. Treatment guidelines for immunologically competent individuals (e.g. those without immunodeficiency, immunosuppression
and prolonged corticosteroid use).
Adults
Tuberculoid (TT & BT) (WHO classification PB)
Agent
Dapsone

Dose
100 mg daily

Rifampicin

600 mg daily

Duration
12 months, and then
therapy discontinued

Adults
Lepromatous (LL, BL, BB) (WHO classification MB)
Agent
Dapsone

Dose
100 mg daily

Rifampicin
Clofazimine

600 mg daily
50 mg daily

Duration
24 months, and then
therapy discontinued

TT, tuberculoid; BT, borderline tuberculoid; BB, borderline borderline;

BL, borderline lepromatous; LL, lepromatous; PB, paucibacillary; MB,
multibacillary.
For immunologically compromised patients, these protocols may be
modified, and consultation with the NHDP is recommended

Type 2 reactions (ENL), if mild, are treated with

rest and anti-inflammatory drugs to control the
acute inflammatory skin lesions and fever.14 Aspirin
is the most commonly used anti-inflammatory
drug, but other options include indomethacin,
chloroquine, pentoxifylline and colchicine.14 Severe
ENL is treated with corticosteroids, clofazimine or
thalidomide.14,19 However, this latter drug is contraindicated in women of childbearing age because

of its teratogenic effects. Lucios phenomenon should

be treated with the MDT and with systemic
corticosteroids.40
Several immunosuppressants have been tried
and found to be useful for the treatment of leprosy reactions, including methotrexate, mycophenolate mofetil, ciclosporin and azathioprine.41 44
Treatment with infliximab, to inhibit the production of TNF-, has also been reported.45 However,
corticosteroids and thalidomide continue to be the
mainstay in the management of leprosy reactions.46
With the introduction of the MDT, leprosy is now
considered a curable disease. Early diagnosis and
treatment, with examination of close contacts, are
very important to prevent deformity and disability.
Healthcare providers should be fully aware of the
disease in its initial phases, remembering that a high
index of suspicion is necessary for the diagnosis.
Hansens disease in the United States
Despite the general notion that Hansens disease
only affects underdeveloped countries, there is a
significant presence of the disease in the United
States (Fig. 8). Several factors contribute to this,
notably including immigration. Also, it is worth
noticing that the United States of America has areas
of autochthonous infections such as the region of the
western Gulf of Mexico, and places such as Puerto
Rico, Hawaii and the Trust Islands.
Among the autochthonous cases, the main source
of the disease is believed to be the armadillo that
inhabits the endemic region of the Gulf of Mexico.
In Texas and Louisiana, more than half of the cases
are in patients born in the United States.47,48

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Fig. 8. Cases of leprosy in the United States. Modified from A summary of Hansens disease in the United States. Health resources and services
administration. National Hansenss disease program. U.S. Department of Health and Human Services. 2006. http://www.hrsa.gov/hansens/

The National Hansens Disease Registry received

a total of 137 reports of the disease in 2006. The
total number of cases registered until 2006 amounts
to 12,162.48
From all cases reported in 2006, 85% are in
persons being born outside of the United States.
But if we take into consideration the number of
cases per country, the United States ranks second
with 21 cases reported in US born persons. The
most frequent country of origin for the affected
is the Philippines with 22 cases, whereas Mexico,

134

Brazil and India are in third, fourth and fifth places,

respectively.
The NHDP, a division of the US Department of
Health and Human Services, is the main agency in
charge of providing basic medical services to patients
affected with Hansens disease in the United States.
This is carried out through a network of clinics,
the main one located in Baton Rouge, Louisiana.
The other clinics are located around the country in
Arizona, California, Florida, Illinois, Massachusetts,
New York, Puerto Rico, Texas and Washington.

Invited Review
In addition to taking care of patients and overseeing
the network of clinics, the NHDP is responsible for
conducting research and educating patients and the
healthcare community.
It is important that all physicians should be aware
that Hansens disease remains a public health issue
in this country, especially considering the fact that
early cases can only be identified if a person has some
degree of suspicion. Part of this problem is indigenous
to some regions in the country, as mentioned earlier;
a bigger part is related to immigration, with multiple
epicenters created according to immigration flux in
various regions. The problem is further compounded
by the fact that these populations may also be affected
by other infections endemic to their country of origin.
It is not uncommon for immunosuppressive therapy,
required for many of these at-risk patients, to unmask
comorbidities, such as tuberculosis, hepatitis B and
strongyloidiasis, to mention a few.
Conclusion
In the beginning of 2008, the WHO indicates that
official reports were received from 118 countries
amounting to a registered global prevalence of
212,802 cases.49 The same publication indicates that
the number of new cases detected fell from 763,202
in 2001 to 254,525 in 2007. Many countries that
were considered highly endemic have now reached
elimination rates as previously defined. This was
achieved through wide implementation of The Global
strategy for further reducing the leprosy burden and sustaining
leprosy control activities (plan period: 20062010).50 The
main strategies are early detection and prompt
treatment with MDT. Although very successful, this
approach faces many challenges, as these activities
will have to remain in place for a long period of time,
and maintaining such an endeavor for decades in
underdeveloped countries is a monumental task.
The problem is extremely complex and several
fold. First, the incubation period of the bacillus
is long. Second, the number of silent carriers is
unknown. Also, the mechanism of transmission of
the disease is not fully understood. Furthermore,
because of the stigma associated with this condition,
early diagnosis and early treatment are precluded by
considerable delays in self-reporting. There is also
the problem of the lepra reactions. Because of the
slow rate of elimination of the bacillary particles, the
reactions can occur several years after the patients
have been considered cured. Finally, the socioeconomic status of the afflicted, usually in countries
without means for early diagnosis and optimal care,
makes Hansens disease a very stubborn entity that
will more likely than not remain a considerable public
health issue for years to come.

Conflicts of interest
None.
Adriano Piris1,2 Alice Z.C. Lobo3,4 and Samuel L.
Moschella5,6
1

Dermatopathologist, Massachusetts General

Hospital, Boston, MA, USA,
e-mail: apiris@partners.org,
2 Instructor of Pathology, Harvard Medical School,
Boston, MA, USA,
3 Dermatologist, Hospital das Clinicas, University of
Sao Paulo, Brazil,
4
Research Fellow, Massachusetts General Hospital,
Harvard Medical School Boston, MA, USA,
5 Senior Dermatology Consultant, Dermatology
Department Lahey Clinic Medical Center, Burlington,
MA, USA and
6
Associate Clinical Professor, Harvard Medical
School, Boston, MA, USA

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