CLINICAL

REVIEW

An update on the diagnosis and treatment of leprosy

Samuel L. Moschella, MD
Burlington, Massachussetts

lthough the World Health Organization

(WHO) has assumed the authoritative role
for the guidelines for the diagnosis and treatment of leprosy, certain endemic countries such as
India and Brazil and countries with traditional interest in the disease such as England and the United
States have utilized their own programs which have
been modified by the WHO. The prevalence of
leprosy in endemic areas has been significantly
reduced, but unfortunately the incidence continues
to be the same. However, the WHO is optimistic
about the elimination of leprosy as a public health
problem in the near future; such optimism is not
shared by many leprologists. The practicing physician especially in the non-endemic areas, such as the
USA, should be aware of the current diagnostic and
therapeutic approach to leprosy as recommended
not only by the National Leprosy Center of the USA
and its satellite clinics, but also by the WHO. If the
practicing dermatologist wants to treat leprosy and
become a dermatoleprologist, it is mandatory that
he is knowledgeable about its diagnosis, treatment,
reactions, the associated neuritis, and the prevention
and management of its disabilities.
With the potential decline of leprosy, leprosy
control will become part of primary health care,
which must promote early detection, adequate treatment, prevention, and management of disabilities.

GLOBAL SITUATION1,2
Over the years through expert committees and
study groups, the WHO has assumed responsibility
for recommending guidelines especially to underdeveloped countries for the management of leprosy.
These guidelines take into consideration not only
From the Department of Dermatology, Lahey Clinic Medical
Center.
Funding sources: None.
Conflicts of interest: None identified.
Reprint requests: Samuel L. Moschella, MD, Dermatology Department, Lahey Clinic Medical Center, 41 Mall Road Burlington, MA
01805. E-mail: Samuel_L_Moschella@lahey.org.
J Am Acad Dermatol 2004;51:417-26.
0190-9622/$30.00
2004 by the American Academy of Dermatology, Inc.
doi:10.1016/j.jaad.2003.11.072

their efficacy, but also the cost and availability of the

necessary personnel. Although it has become literally the authoritative resource, one must recognize
that in some endemic areas such as Ethiopia, Brazil,
and India, and in the non-endemic areas such as the
United States and England, there are research and
study centers assessing, developing, and recommending modifications of the current diagnostic and
therapeutic approaches.
Leprosy is regarded as a special public health
problem because of its capacity to cause permanent
disabilities with their social consequences of discrimination and stigma. The three main objectives in
controlling leprosy are to interrupt transmission, cure
patients, and prevent development of deformities.
In 1981, the number of leprosy patients in the
world was more than 12 million. With the implementation of multiple drug therapy (MDT) in
1982 and the World Health Assembly resolution in
1991, the global effort for the Elimination of Leprosy
as a Public Health Problem was prioritized and has
resulted in over 10 million patients being cured. In
countries with more than 100 cases registered, the
WHO reported in January 2002 that, in the year 2000,
a total of 597,232 cases were registered and 719,330
new cases were diagnosed.1 This report emphasized
the reduction of the global prevalence from 12 per
10,000 in 1985 to slightly below 1 per 10,000 in 2002.
Of the 122 countries in which leprosy was considered
endemic, 107 have achieved the elimination target
(1 per 10,000). Approximately 83% of the leprosy
cases live in six countries: Nepal, Madagascar,
Myanmar, Indonesia, and especially India and
Brazil. As of 2001, there are 6,518 active cases of
leprosy registered in the USA.3
The number of new cases remained about the
same until the last 10 years during which there has
been a significant increase. This increase may be
a result of increased case detection campaigns and
expansion of geographical coverage by leprosy
services in endemic countries.
Encouraged by the success of the multiple drug
therapy, the WHO assembly adopted, in 1991, a declaration to eliminate leprosy as a public health
problem by the year 2000. Since it was apparent that
the goal was not to be achieved, a global alliance for
417

418 Moschella

the elimination consisting of a core group of the

governments of leprosy endemic countries, the
Nippon Foundation, the International Federation of
Antileprosy Association, Novartis, and the WHO was
formed. It will cooperate with other institutions to
eliminate leprosy by the end of 2005. The past
pessimism by various authors, which I share, still
persists.4 This lack of enthusiasm is fueled by the
following:
1. There is no sound evidence that the leprosy
elimination strategy has had an impact on
transmission so far.
2. The incidence has increased in spite of the
significant reduction of the prevalence.
3. Leprosy is not only a bacterial, but also an
immunologic disease. Failure of early diagnosis and treatment will increase neural disease.
After patients are cured by MDT, a quarter of
them experience immunologic nerve damage
reactions. Emphasis on elimination can eclipse
the quality of care necessary to prevent the
disability, which will prevail long after 2005.
4. Other areas that need to be addressed which
could have a significant impact on elimination
are the recognition of the hidden prevalence,
the subclinical infected, the possible role of the
non-human reservoir or of sources of infection
in the environment responsible for transmission, the need for a skin or serologic tests to
diagnose subclinical, early, or questionable
cases, the availability of an effective, cheap,
proven prophylactic and possible immunotherapeutic vaccine, and the need of personnel
capable of making an early diagnosis and to
clinically differentiate paucibacillary (PB) and
multibacillary (MB) disease.

DIAGNOSIS OF LEPROSY5,6
Leprosy is a slowly progressive infectious disease
caused by Mycobacterial leprae and complicated by
potential intermittent hypersensitivity reactions (the
so-called lepra reactions). It is highly infective with
low pathogenicity and virulence and has a long incubation period. The skin, superficial peripheral
nerves, anterior chamber of the eyes, and testes, all
cooler parts of the body are the most frequently
affected organs. Its geographic distribution varied in
the past, but presently it is endemic mainly in subtropical areas. Early accurate diagnosis and therapy
are most important for the control of the disease, the
management of the patient, and prevention of disabilities. Underdiagnosis promotes transmission and
results in unnecessary suffering and deformities; overdiagnosis is responsible for unnecessary treatment,
stress, stigma, and misleading epidemiologic statistics.

J AM ACAD DERMATOL
SEPTEMBER 2004

At the Seventh Meeting of the WHO Expert

Committee on Leprosy in 1997, a case of leprosy
was defined as an individual who has not completed
a course of treatment and has one or more of the
three cardinal signs:7
 Hypopigmented or reddish skin lesions with loss
of sensation
 Involvement of the peripheral nerves as demonstrated by their thickening and associated loss of
sensation
 Skin-smear positive for acid-fast bacilli
The specificity of the diagnosis based on the
presence of anesthetic hypopigmented or erythematous macular lesions is reduced in multibacillary
cases because the lesions can be less distinct and less
anesthetic. Consequently macular anesthetic lesions
as a single diagnostic criterion for multibacillary (MB)
disease has resulted in up to 30% of patients to be
missed; this contrasts with patients with distinct
maculoanesthetic lesions of paucibacillary (PB) disease, which are reportedly diagnostic in 90% of
cases.
In Ethiopia where all 3 cardinal signs were used,
the diagnostic sensitivity was 97%.1
Peripheral nerve enlargement usually appears
later than skin lesions. The most commonly involved
nerves are the ulnar and common peroneal. The
presence of one or more enlarged nerves is seen
more commonly in multibacillary disease. False
positive reporting of nerve involvement may result
from poor examination technique or the nonspecific
enlargement of nerves such as in manual workers.
To improve the certainty of the diagnosis of
leprosy, a balanced approach to the diagnosis is
strongly recommended and would be the appreciation of the presence of a thickened nerve and one
other diagnostic sign such as:
 typical hypopigmented or erythematous skin
lesions with or without sensory loss or
 typical nerve-function impairment such as loss of
sensation of palms or soles
Leprosy can present as a purely neural disease
without skin lesions; the so-called neuritic leprosy.
Nerve biopsy is confirmatory. The incidence is 0.5%
in Ethiopia, 4.6% in India and 8.7% in Nepal.
The diagnostic specificity of skin smears is almost
100%; however, its sensitivity is rarely more than 50%
because smear positive patients represent only 1050% of cases. The inherent problems of skin smears
are the logistics and the reliability of the technique of
taking, staining, and interpretting the slide. Skin
smears identify those with multibacillary disease
who are the most infectious and also those patients
who are experiencing clinical relapses.

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Tissue biopsies are used to make a histologic diagnosis, to classify the disease, to provide material for
inoculation of footpad of mice, and for research
purposes. The biopsy cannot be regarded as the
diagnostic gold standard since a number of them can
be nondiagnostic or doubtful. In practice, a clinical
and histopathologic correlation may be necessary for
diagnosis. Although the histopathologic diagnostic
specificity of non-reactive disease is high, the histopathologic differentiation of relapse from reversal can
be difficult or impossible. In a study in China, immunohistopathologic studies staining biopsies of PB
patients with phenolic glycolipid-1 antigen proved to
be more specific diagnostically than routine hematoxylin and eosin histopathologic examination.8
In the field, the diagnosis and classification of
leprosy have been based on clinical examination and
skin smears when facilities are available. Diagnostic
tests such as histologic examination of involved
tissues, inoculation of footpads of mice, serologic
and skin testing, and polymerase chain reaction
studies have been confined to countries which have
such studies available to their practicing physicians
and in academic and research centers.
Two laboratory tests, which so far have not played
a significant role in the diagnosis of leprosy, are the
serological tests for anti-PGL-1 antibodies and the
PCR test. There are two methods to determine the
presence of anti-PGL-1 antibodies, the M. leprae
particle agglutination assay (MLPA) and the enzymelinked immunosorbent assay which was further refined into a dipstick assay which has greater
specificity.9 The PGL-1 antibody testing is specific
and sensitive in patients with MB disease, but unfortunately it is not very helpful in the diagnosis of PB
disease and is unable to predict, among contacts of
known cases in the general population, who will
develop the disease.
PCR testing is a highly sensitive and specific diagnostic tool. Unfortunately, in countries where it is
available, such as at the National Hansen Disease
Center, Baton Rouge, Louisiana, USA, it has not proved so far to be as effective diagnostically as anticipated in indeterminate or paucibacillary disease.10

CLASSIFICATION OF LEPROSY11
In 1966, Ridley and Jopling created a classification
of leprosy, based on the immunologic response of
the host to M leprae, into a five-group system: TT
(polar tuberculoid), BT (borderline tuberculoid), BB
(borderline), BL (borderline lepromatous), and LL
(polar lepromatous) (Table III).12 In 1982, the WHO
study group for chemotherapy for control programs
recommended the classification of all patients be
based on Ridley-Joplin classification and the esti-

mated bacterial load in skin-slit smears. The TT and

BT patients who had a bacillary index (BI) # 2+
were classified as paucibacillary disease, and BB, BL,
and LL patients who had a BI > 2+ were classified as
multibacillary disease. The BI reflects the number of
acid-fast bacilli per average oil immersion field and
is expressed on a 0-to-6+ semilogarithmic scale; BI
of 2+ is the presence of 1 to 10 bacilli per 10 oil
immersion fields. In 1988, the WHO Expert
Committee on Leprosy, to avoid further treatment
failure of PB patients with positive skin-slit smears,
recommended such cases be classified as multibacillary disease and consequently any patient with
a positive skin smear is classified as multibacillary
disease.
In the USA, the Public Health Service recommends
that the classification of leprosy should be made on
clinical evaluation, and skin smears from several sites
and skin biopsies which can be read if necessary by
the National Hansens Disease Center, Baton Rouge,
Louisiana. The Ridley-Jopling classification is used.
In general, PB disease is equivalent to indeterminate
I, TT (tuberculoid), and BT (borderline tuberculoid)
disease, and MB is equivalent to BB (borderline), BL
(borderline lepromatous) and LL (lepromatous) disease with the Ridley-Jopling classification. In the US,
the terms paucibacillary and multibacillary are used
to direct drug therapy.
In 1998, the WHO Expert Committee on Leprosy
declared skin-slit smears were not essential for
multiple drug therapy (MDT) and the number of
clinical lesions present was to be the basis for
classification.13 This was motivated by the unavailability or unreliability of the skin smear in many
programs and the potential for transmitting HIV
disease and hepatitis by unsterile techniques. The
recommended clinical diagnostic guidelines are as
follows: patients who are not experiencing reactions
and have less than five skin lesions are to be classified
as paucibacillary disease (PB), and those with greater
than five skin lesions are to be classified as
multibacillary (MB). Unfortunately, there will be
a sufficient number of MB cases classified as above
PB to cause some concern because of the potential
for their inadequate treatment.

REACTIONS13,15
There are two main categories of reactions in
leprosy: Type I14 reaction, which is called a reversal
lepra reaction and is an example of Type IV cell
mediated allergic hypersensitivity reaction (Coombs
and Gell); and Type II lepra reaction,15 which is
reported as erythema nodosum leprosum and is an
example of Type III humoral hypersensitivity reaction (Coombs and Gell) (Table II). Among the

420 Moschella

precipitating factors are physical and mental stress,

multiple drug therapy, vaccines, pregnancy, surgical
procedures, injuries, intercurrent infections, and
other antibacterial treatments. Inflammatory edema
of hands, feet, and face is seen in both types and is
a sign of severe reaction. Nerve tenderness and pain
are often associated with both types. The most
common and frequent nerves involved are the ulnar,
the facial, median, common peroneal, and posterior
tibial nerves. The related physical impairment may
be acute and dramatic such as facial paralysis or foot
drop.
The reversal reaction (Type I)14 occurs in borderline tuberculoid (BT), borderline (BB), and especially borderline lepromatous (BT) and possibly in
tuberculoid (TT) leprosy. The present skin lesions
become more erythematous, edematous, raised, and
rarely ulcerated. Patients with BL, BB, and occasionally BT disease exhibit not only changes in the
existing skin lesions but may develop similar new
lesions.
Erythema nodosum leprosum (ENL) (Type II)
reaction15 is immunologically characterized by immune complex deposition in tissue space, blood, and
lymphatic vessels, which give rise to acute inflammatory foci. It usually occurs most frequently in
patients with LL and occasionally in those with BL.
Increased cell-mediated immunity may play a role in
the precipitation of an attack. The tumor necrosis
factor alpha is elevated in ENL. During therapy or
occasionally during pregnancy, patients with BB to
LL disease can develop ENL characterized by the
sudden appearance of crops of erythematous tender
nodules or plaques, and rarely vesicular, pustular, or
necrotic lesions. The eruption favors the extensor
surfaces of the extremities and face. Other signs and
symptoms are fever and malaise, iritis, epistaxis,
muscle and bone pain, nerve pain, joint pain, lymphadenitis, epididymo-orchitis, and proteinuria.
A third rare type of reaction, the Lucio phenomenon,15,16 is usually restricted to Central and South
America and immigrants from those areas. It occurs
in a subtype of lepromatous leprosy called the
primary diffuse lepromatous leprosy (la lepra bonita)
characterized by a diffuse infiltration of the skin by
a granulomatous process heavily loaded with mycobacterium leprae. Significant nasal mucosa involvement usually occurs with subsequent nasal
destruction. Ocular and peripheral nerve involvement are significantly less frequent than in the other
subtypes of lepromatous leprosy. Intermittent fever,
generalized lymphadenopathy, and splenomegaly
may be seen. The eruption (the Lucio phenomenon)
is characterized by the presence of symmetrical
erythematous, black, stellate, necrotic lesions of the

J AM ACAD DERMATOL
SEPTEMBER 2004

extremities and occasionally the face. The patients

are usually on no treatment when it occurs.

HANSENS DISEASE AND PREGNANCY20,21

It is not uncommon for leprosy to present itself
during pregnancy or early puerperium. Because of
alterations in immune responses during pregnancy,
leprosy patients who become pregnant are more
prone to develop type I and II reactions, a downgrading of their disease, and relapses. Type II reaction usually occurs during pregnancy, especially
during the third trimester and lactation. Type I reaction occurs, especially during puerperium. Infants
run a relatively high risk of contracting leprosy from
untreated mothers, especially if she has BB or LL
disease. It has been recommended that rifampin be
given only as a single monthly dose during pregnancy, but dapsone and clofazimine can be used
or continued. For reactions, prednisone and clofazimine can be given, but thalidomide should be
avoided. It is advisable to postpone pregnancy temporarily during post-therapy period if there is evidence of reaction, relapse, or neuritis.

HANSENS DISEASE AND HIV DISEASE22,23

Unlike in tuberculosis, HIV disease has not had
a significant impact on the clinical course of treated
and untreated leprosy. However, it has been reported that the neuritis in co-infected people can be
more severe and the reversal reaction may be more
frequent after therapy. In endemic areas of HIV
disease and leprosy, there does not appear to be
a greater incidence of leprosy among HIV patients. It
may be because of the very slow proliferation of the
bacilli or the prolonged incubation period, or perhaps a particular cellular mechanism involved in its
pathogenesis.

THERAPY OF NONREACTIVE DISEASE24-26

In 1981, the WHO Study Group recommended
multiple drug therapy (MDT) for the following
reasons:24
1. To address dapsone resistance and to discourage resistance to other drugs to be used.
2. To promote compliance and to get away from
long-term monotherapy such as dapsone.
3. To keep rifampin in all therapeutic regimens
because of its powerful bactericidal action and
its effectiveness even when taken once
a month.
4. To promote compliance and cost effectiveness.
In 1997, the WHO Expert Committee suggested
that it might be possible to reduce duration of MDT
for multibacillary disease from 2 years to one year
and also recommended the treatment of a single PB

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lesion with one dose of ROM (rifampin, ofloxacin,

and minocycline).26
The current WHO recommended MDT for adults
is as follows:
1. For paucibacillary disease (PB), 600 mg rifampin monthly and 100 mg dapsone daily with
six cycles in 9 months.
2. For multibacillary disease (MB), 600 mg rifampin and 300 mg clofazimine monthly and 100
mg dapsone and 50 mg clofazimine daily with
24 cycles in 36 months or 12 cycles in 18
months
3. For single PB lesion, rifampin 600 mg, ofloxacin 400 mg, minocycline 100 mg. Because of
a lack of long-term follow-up, this recommendation should be considered experimental.
Single-lesion leprosy, which is often indeterminate leprosy, heals spontaneously in 80% of
patients. It is too early to determine if those of
the 20% who develop classifiable leprosy will
benefit from single ROM treatment. It may cure
some patients but will only delay the onset of
multibacillary disease in others. In the United
States, a biopsy of a suspicious lesion is
recommended to establish the diagnosis and
deliver the appropriate treatment.
Other drugs with anti-mycobacterial properties,
which may be used if needed as substitutes in the
above current therapeutic programs are rifabutin,
ofloxacin, sparfloxacin, levofloxacin, minocycline,
and clarithromycin.
Although the diagnostic and therapeutic
guidelines advocated by the WHO have significantly
impacted on global prevalence rate, practitioners
especially in the USA should be aware that the Public
Health service recommends the classifications of the
disease into paucibacillary and multibacillary disease
for its recommendations for treatment. In the United
States, MDT has been standard therapy since the
1970s. During this period, 3 years of daily dapsone,
rifampin, and clofazimine was recommended, and if
there was suspicion of dapsone resistance, severe
and widespread neuropathy, or ocular disease, dapsone was recommended to be given indefinitely. In
1990, a relatively short-term therapy was introduced
in the United States under the FDA-approved
protocols which has proven very effective and
without significant toxicity. The current recommended standard treatment regimens for Hansens
disease in the United States are outlined in Table I.
The strengths of MDT are that it obviates and
prevents dapsone resistance, quickly reduces infectivity, and results in fewer relapses and reactions, and
consequently, fewer disabilities. The shortcomings
of MDT are the long duration of therapy with

Table I. Public health service recommendation for

treatment of leprosy
1. Paucibacillary (PB): Dapsone 100 mg daily plus Rifampin
600 mg daily for 1 year
2. Multibacillary (MB): Dapsone 100 mg daily plus Rifampin
600 mg daily plus clofazimine 50 mg daily for 2 years
3. The follow-up: For treated PB disease every 6 months for
5 years and for MB disease every 6 months for 10 years

consequential reduction of compliance, encouragement of default, and creation of operational

difficulties.
Since WHO/MDT appears to have been effective,
safe, and well tolerated and the risk of relapse is low,
unlike the USA, the WHO does not advocate posttherapy surveillance. Patients are advised to report as
soon as they note any skin, eye, or nerve changes.
Fortunately, post-therapy relapse, reaction, and silent neuropathy occur infrequently. Until sufficient
data of post-therapy complications exists, treated
paucibacillary disease should be followed for at least
three to five years, and treated multibacillary disease
should be followed for at least five to ten years. Silent
neuropathy is described as a progressive sensory or
motor impairment which occurs in the absence of
cutaneous type I or II reactions and is characterized
by a lack of involved nerve tenderness and burning
or shooting pain, paresthesias, or numbness.27

VACCINATION
Anti-leprosy vaccination can be immunoprophylactic or immunotherapeutic.28-30 The
immunoprophylactic aim is to restore the host recognition of shared mycobacterial antigens to promote TH1 responses to them, to induce CD8 cytotoxic
cells, and to downregulate the proportion of T cells
producing interleukins 4 and 5. The aim of immunotherapy is to switch off the mechanisms leading to
immunopathology and to increase intracellular
mechanisms by which bacilli are killed. The first
vaccine used was BCG, but its failure to protect certain
populations clearly indicated that an improved vaccine against leprosy was needed. Among the vaccines
being used or explored are Mycobacterium W.
(Talwar, 1978), Mycobacterium ICRC (M avium intracellulare) (Deo et al, 1981), bacillus CalmetteGuerin plus heat-killed M leprae (Convit, 1992),
Mycobacterium tufu (Iushm and Kalianina, 1995),
and Mycobacterium habana (Singh et al, 1997).29
The enthusiasm for use of vaccines has lessened
because of the significantly favorable impact of MDT
on leprosy. However, one cannot deny the potential
usefulness of a proven effective vaccine in highly
endemic countries such as India and Brazil. Some of

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Table II. Characteristic features of reactional leprosy

Type II reaction
(erythema nodosum
leprosum-ENL)

Type I reaction
(Reversal)

Complication of types of - Rare in Tuberculoid

leprosy
- Borderline (BB, BT, BL)
Clinical features

Neuropathic and ocular

changes

Course

Treatment

-Lepromatous leprosy (LL) and

occasionally Borderline
lepromatous (BL)
Occurs during 6 to 18
- Can occur before
months of treatment
treatment but
tends to occur
later in treatment
Constitutional signs and - In severe cases
constitutional signs
symptoms in severe
and symptoms
cases
- Erythematous tender nodules
Lesions become
appear in crops especially
erythematous and
extremities and face
edematous; in severe
cases ulcerate (Lazarine - Vesicular, pustular, suppurative
lesions can occur
leprosy)
- Edema of face, hands, and feet
Edema of affected
extremities and face may - Orchitis, lymphadenitis, dactylitis,
epistaxis, and proteinuria
occur

- Pain and swelling of one

or more nerves; nerve
abscesses can form
- Sudden appearance of
claw hand, foot drop,
facial palsy with or
without Lagophthalmus
and complicating
exposure keratitis
- Usually persists for few
months

- Widespread painful swelling of

peripheral nerves
- Iridocyclitis and scleritis of eyes

- Few days but can

be prolonged,
frequent, and recurrent
- Prednisone, non-steroidal - NSAID, thalidomide,
anti-inflammatory drugs
increase dose
(NSAID)
of clofazimine, systemic
corticosteroids

the best information about vaccination is from India

where the Mycobacterium W and ICR vaccines are
currently being used. In the field, with the use of
vaccine W as an adjunct to chemotherapy, the
following observations were noted:28
1. The vaccine expedited bacterial clearance and
accelerated clinical regression of the lesions.
2. It shortened significantly the period of release
of the patients from treatment.

Lucio
phenomenon

- Diffuse lepromatous leprosy;

endemic Mexico
and Costa Rica
- No constitutional signs or
symptoms

- Pseudomyxedematous
facies
- Madarosis with or without
generalized and diffuse hair
loss
- Extensive areas of anhidrosis
- Early and severe destructive
rhinitis
- Erythema necrotisans-flame
or geographic shaped
necrotic lesions anteceded
sometimes by hemorrhagic
blisters; appear in crops,
favor extremities and
sometimes the face; heal
with superficial scarring
- No orchitis
- Labsanemia, elevated
sedimentation rate,
positive STS
- Acral distal symmetrical
anesthesia usually with no
significant aggravation
- Muscle atrophy and
paralysis late
- Minimal ocular
involvement with
no iritis
- Depends on the severity of
the reaction and response
to MDT
- Systemic corticosteroids
- Thalidomide is ineffective

3. It was effective in inducing a fall in the bacterial

index in multibacillary patients who are poor or
nonresponders to the standard multidrug therapy.
4. It promoted the conversion of the lepromin
from negativity to positivity.
5. There was a histopathologic upgrading.
6. The vaccine was tolerated and the incidence
and severity of the type II reaction was reduced.
7. No increased incidence of neuritis was seen.

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Table III. Ridley-Jopling classification

Characteristics

Cutaneous

Indeterminate

Tuberculoid
(TT-Polar)

One or few
One or few
lesions;
lesions;
hypopigmented sharply
or erythematous marginated;
scaling
macules; favors
macules or
extremities,
buttock, or face plaques;
sometimes
elevated
edges

Neuropathic Lesions slightly

changes
hypesthetic,
no peripheral
nerve
enlargement

Lesions
anesthetic
sometimes
enlarged
nerve in
area
of lesion

Nil
Nil
Bacterial
(paucibacillary)
(paucibacilindex
lary)
(Ridleys
logarithmic
indices)
Lepromin
Variable
Strongly
test
positive
Reactions
Nil
Rare

Borderline
tuberculoid (BT)

Borderline
(BB)

Borderline
lepromatous (BL)

Lepromatous
(LL + Polar)

Multiple and
symmetrical
erythematous
and copper
colored
macules,
later
induration
followed by
nodulation
of face
especially
ears and nose,
extremities
especially
joints,
and trunk;
madarosis
of eyebrows;
nasal
mucosal
ulceration
Usually no
Lesions
Moderate
Lesions
sensory
slightly
anesthetic
anesthetic
impairment in
anesthetic
lesions
asymmetrical
early stages;
widespread
widespread
several
later
and less
and
peripheral
symmetrical
asymmetrical
asymmetrical
nerves
peripheral
peripheral
peripheral
involved
neuropathy
nerve
nerve
of arms and
involvement
involvement
legs with
stocking
and glove
anesthesia
and of the
facial nerve;
Eye
involvement
(conjunctiva,
cornea, and
iris)
2+-3+
4+-5+
6+ and greater
Nil or scarce
(multibacillary) (multibacillary)
(multibacill(paucibacillary)
ary)
or
multibacillary
Several succulent Many,
Few to many
roughly
plaques
erythematosymmetrical
with sharply
hypochromic
shiny
demarcated
plaques with
erythematous
central area
scaling
and
and edges
surface and
sometimes
sloping into
well-defined
hyposurrounding
margins
pigmented
normal skin;
which
macules,
large
may have
papules,
erythematous
small satellite
nodules
irregular
lesions and
with
infiltrated
is usually
sloping
bands with
annular
edges
central
uninvolved
anesthetic
areas

Weakly
positive
Reversal
(Type I)

Negative

Negative

Negative

Reversal
(Type I)

Reversal
(Type I)
and/or
ENL (Type II)

ENL (Type II)

424 Moschella

TREATMENT REACTIONS
Unfortunately in spite of the significant advances
in the chemotherapy of the disease in the last ten
years, the understanding of the etiology and management of reactions has not changed much. MDT
has not significantly impacted on the incidence of the
reversal reaction (Type I). The treatment of the
reaction14,16,31 depends on its severity, the type of
the reaction, the presence of neuritis, facial involvement in Type I reaction, pregnancy, and drug
allergies or adverse drug reactions. If Type I reaction14 is severe or associated with neuritis or facial
lesions are involved, systemic corticosteroids are
indicated. In milder cases without neuritis, the nonsteroid antiinflammatory drugs may be helpful.
With the use of clofazimine in the WHO multidrug
therapy, the frequency of ENL Type II reaction has
been significantly reduced to as low as 5%. In Type II
reaction15,16,31 without significant neuritis, thalidomide is effective, but in the presence of neuritis or
iritis, systemic corticosteroids are necessary. Topical
steroids and atropine are used for reactive iritis.
Clofazimine in higher doses can sometimes permit
the use of lower doses of corticosteroids.
Colchicine32 and pentoxifylline33 have been reported to be effective and may be useful as adjunctive drugs in the treatment of Type II reactions. In
refractory cases immunomodulatory agents such as
cyclosporin A,34 and intravenous IgG might be
considered.
Lucio phenomenon17,18,19 is usually controlled by
systemic corticosteroids. In the late 1940s and early
1950s, Mexican leprologists reported the control of
Lucio phenomenon in untreated patients with the
initiation of dapsone therapy;35 unfortunately in my
limited experience, monotherapy with dapsone in
the past and MDT presently did not make a significant
therapeutic impact on the Lucio phenomenon.
Thalidomide is ineffective. In severe, non-responding cases, immunomodulatory and cytotoxic
drugs, such as Imuran or cyclophosphamide with
systemic corticosteroids with or without plasmapheresis have been used.

PREVENTION OF DISABILITIES AND

REHABILITATION36,37
The socioeconomic impact resulting from the
physical and psychological disabilities of leprosy
continues to be a burden in endemic countries.
25% of leprosy patients have some degree of disability, which are greatest in patients with BL and LL
disease. It is greater in the older age group and males.
Duration of disease and disability are directly proportional to each other. Hands are most frequently

J AM ACAD DERMATOL
SEPTEMBER 2004

involved followed by the feet and eyes. There is

an estimated 3 million people with physical impairments with resultant disabilities. Approximately
4% of newly diagnosed patients present with grade II
disabilities, which are visible deformities or
damages. However, grade I disability, that is the
presence of anesthesia without damage is important
because these patients are at serious risk of developing serious impairments and deformities. The
reported incidences of grade II disabilities are 3% in
patients in Southeast Asia, 11% of patients in Africa,
and 5% of patients in America.
Any leprosy program must incorporate the prevention of disabilities and rehabilitation. Among the
important efforts for prevention are an initial and
periodic measurement of neural impairment, aggressive treatment of reactions, provisions for the therapy
and prevention of injuries to the hand, of foot ulcers,
of eye disease, and their complications, and instructions in self-care.
The best methods for early detection of nerve
impairment are palpation for nerve enlargement and
use of sensory testing. Early detection and treatment
of reactions significantly reduce and prevent nerve
damage with its resultant impairment. The initiation
of systemic steroid therapy depends on the magnitude and duration of nerve function impairment and/
or nerve tenderness. It has been reported that 7% of
new patients have at first examination silent neuropathy; 75% of all silent neuropathy was diagnosed
during the first year of treatment.27 Patients with
extensive skin involvement, more than three enlarged nerves or a positive skin smear, are at a greater
risk to develop silent neuropathy.
The leprous neuropathy responds to a prednisone
taper over about a four to six month period. Patients
treated for early nerve impairment regain 60% of
their nerve function. Neural impairment up to six
months duration may be benefited by systemic
corticosteroid therapy. The role of surgery in the
management of nerve involvement is controversial.
There is no data to suggest that steroids given prophylactically prevent impairment.

CONCLUSIONS
In spite of its relative simplicity, the classification
of leprosy into paucibacillary and multibacillary
disease based on lesion count in conjunction with
an MDT program has proven to be effective in the
reduction of the prevalence without significant relapse or Type I and II reactions. In fact, the incidence
and severity of these reactions, especially Type II,
have been reduced. When facilities are available to
do the necessary laboratory studies, it is advisable to

J AM ACAD DERMATOL
VOLUME 51, NUMBER 3

continue to use the Jopling-Ridley classification for

scientific communications, teaching, and patient
care. Because the incidence appears to have remained stable in spite of MDT, the projection of the
WHO program for the eradication of leprosy by 2005
appears to be unrealistic.
In the absence of a proven cost effective vaccine
and because of the time constraints for vaccine trials,
there is presently no alternative to MDT in trying to
break the chain of transmission of M. leprae, however. I share the pessimism of some leprologists. The
question being asked is: will the repeated postponements of the goal elimination dates undermine the confidence and support of the involved
governments, institutions, and public for this strategy? I would like to echo support for the stance of
Vissehedigk et al2 which is: sustainable leprosy
control which consists of early detection of patients,
adequate treatment, and providing comprehensive
care for the prevention of disabilities and rehabilitation, and continuous research to close the substantial
gaps in the knowledge of leprosy transmission and
epidemiology should be our goal for the foreseeable
future rather than elimination.
Unlike tuberculosis, HIV disease has not yet
demonstrated a significant impact on leprosy and
vice versa. Pregnancy can precipitate relapse, as well
as Type I and Type II reactions. Unfortunately, there
are no skin tests or serologic studies that facilitate the
recognition of carriers, or the diagnosis of
paucibacillary disease. The pathogenesis and treatment of the Lucios phenomenon need to be
established.
In endemic regions, leprosy continues to be
a public health problem. Defining leprosy control
purely by prevalence side-steps the real issues such
as:
1. Control programs need tests such as a skin or
serologic test for recognition of carriers in
order to help reduce the incidence.
2. Research is needed to shorten treatment schedules and to reduce defaulter rates and costs.
3. Treatment of the infection, reactions, and
neuritis is still far from ideal.
4. The use of an effective vaccine may play
a major role in the prophylaxis and therapy
of leprosy in high-risk regions.
5. Research into the true incidence of leprosy in
endemic areas is essential.
6. Control programs need a more detailed understanding of M. leprae to permit more logical
interventions.
7. It is essential to minimize irreversible physical
damage, which leads to disability and stigmatization.

Moschella 425

Although, the prevalence rate should continue to

decline, the major problems such as the disabilities
among the old cured patients and those of the
newly diagnosed patients must continue to be
addressed.
Because of the lack of the knowledge necessary to
reduce the incidence of leprosy, we have to continue
to invest heavily in the effort to reduce the prevalence and hopefully the incidence secondarily. It is
also important to plan for the future by making
leprosy control part of primary health care, which
should promote early detection and adequate treatment as well as disability prevention and management Table II and III.
I acknowledge the exemplary technical support of
Janet Couturier and the library assistance of Carol
Spencer.

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