LEPROSY (HANSEN’S DISEASE)

Mikias Woldetensay
Dermatology & venereology
October 2021
 LEPROSY
INTRODUCTION
• A chronic granulomatous infection, caused by
Mycobacterium leprae, primarily involving the skin
& peripheral nerves.
• Also affects other organs & systems like the eyes,
respiratory tract, lymph nodes, testicles & joints.
• A deforming, disabling & stigmatizing disease.
 LEPROSY
EPIDEMIOLOGY
• Common in tropical & subtropical regions of Asia,
Africa, & Central & South America.
• Brazil , India, and Indonesia account for 76% of all
cases worldwide.
• Africa – mainly found in the Sub-Saharan belt &
lower prevalence in northern & southern part of the
continent
 LEPROSY
EPIDEMIOLOGY
• LEPROSY IN ETHIOPIA
– In amharic language ‘sega dewe’.
– The first infectious disease that Ethiopia launched
National Control Program supported by
UNICEF(1948,Emperor Haileselassie I).
– Additional modified new buildings & renamed All
African Leprosy Rehabilitation & Training center
(ALERT) in 1965.
 LEPROSY
EPIDEMIOLOGY
• Armauer Hansen Research Institiute (AHRI) was
built in 1966 to broaden the knowledge of leprosy
through research.
• Rank of disease burden in Sub saharan Africa
2nd15.3% in Ethiopia.
• 4,611 new cases per annum, ~5000.
• Over 30,000 people are living with permanent
leprosy-related disability.
 LEPROSY
ETIOLOGY
• Mycobacterium leprae:
– A non-toxic & non- motile gram-
positive obligate intracellular acid-fast
bacillus.
– A slightly curved rod.
– Divides by binary fission (generation
time 12.5 days).
– Phenolic glycolipid I is a major species-
specific & immunogenic constituent of
the highly non polar outer layer of the
bacillus.
 LEPROSY
ETIOLOGY

• PGL-1:
– May play a role in suppressing the T-cell response
& IFN-γ production.
– Entry into nerves is mediated by the binding of the
species-specific trisaccharide in phenolic
glycolipid I to G domain of the alpha-chain of
laminin 2 (found only in peripheral nerves) in the
basal lamina of Schwann cell axon units.
– M. leprae is the only bacterium known to invade
peripheral nerves.
 LEPROSY
ETIOLOGY
• The primarily affected sites:
– Peripheral nerves, skin, mucous membranes, bones &
viscera (e.g. liver, testes).
• In infected tissues, the leprosy bacillus favors
intracellular locations, within macrophages & nerves.
• Preference for
– Cooler regions of the body (e.g. nose, testicles & ear
lobes) &
– Where the peripheral nerves are close to the skin.
• Relatively spares the perineum, groin, axilla & scalp.
 LEPROSY
PATHOGENESIS
• Transmission
– Predominantly via nasal & oral droplets from the
bacilliferous patient.
– Nasal discharges from untreated LL patients are the
main source of infection
– The bacillus is still viable in dried secretions..1-7
days
 LEPROSY
PATHOGENESIS
• Three requirements for the spread of leprosy:
– A contagious patient
– A susceptible person
– A close or intimate contact
• Incubation period:
– Average incubation time of 2–5 years for
tuberculoid cases.
– 8–12 years for lepromatous cases.
 LEPROSY
PATHOGENESIS
• Immune response to M. leprae determines whether
disease will develop & also which type of leprosy the
patient develops.
• Depending upon the level of specific cell-mediated
immunity , the disease can
– Progress without restraint.
– Limit itself.
– Resolve spontaneously.
 LEPROSY
PATHOGENESIS
• Eventhough the cell mediated immunity is the most
important determinant of acquiring/ developing
leprsoy, the innate immunity also plays an important
role in conferring the susceptibility to leprosy, as well
as determines the propensity to develop clinical
disease.
– Family history in close contacts.
– Various gene loci
• PARKG, vit D receptor gene, PCRG gene,
TLR1/2 heterodimer defects, etc…
 LEPROSY
PATHOGENESIS
• 4 major causes of tissue damage:
– The degree to which cell-mediated immunity
(CMI) is expressed:
• Lepromatous leprosy- Failure of CMI specifically
towards M. leprae with resultant bacillary
multiplication, spread.
• Tuberculoid leprosy- CMI is strongly expressed,
so that the infection is restricted to one or a few
skin sites & peripheral nerves.
 LEPROSY
PATHOGENESIS
• The extent of bacillary spread and multiplication.
– In LL, haematogenous spread of bacilli occurs &
bacilli are readily found.
– In tuberculoid leprosy, bacillary multiplication is
restricted to a few sites & bacilli are not readily
found.
 LEPROSY
PATHOGENESIS
• The appearance of tissue-damaging
immunological complications.
– Lepra reactions
• Tissue damaging immunologic reactions that
may result in new onset acute nerve damage, or
result in organ damage.
 LEPROSY
PATHOGENESIS
• The development of nerve damage & its
complications:
– In skin lesions:
• Small dermal sensory & autonomic nerve fibres
are damaged, causing local sensory loss & loss
of sweating within the area of the skin lesions.
 LEPROSY
PATHOGENESIS
• In peripheral nerve trunks:
– Sensory loss & weakness of muscles ,contracture,
deformities & autonomic dysfunction.
– Trauma, bruising, burns, cuts & especially, tissue
necrosis from repetitive trauma.
– Ulceration, secondary cellulitis ,osteomyelitis &
loss of tissue.
 LEPROSY
CLASSIFICATION
• WHO Classification for treatment Purpose (Ethiopian
guideline for HIV/TB/L, 5th edition)
• 1. Paucibacillary (PB) Leprosy
– One to five leprosy skin lesions.
• 2. Multibacillary (MB) Leprosy
– Six or more skin lesions.
– Less than six skin lesions, which have a positive
slit skin smear result.
 LEPROSY
CLASSIFICATION
• Pure Neural Leprosy
– These are patients who do not have any skin
lesion, but who have clearly thickened nerves with
or without signs of nerve damage.
– Patients with pure neural leprosy should be
reported & treated as a MB case.
 LEPROSY
CLASSIFICATION
• Ridley-Jopling classification
– Takes in to account clinical appearance/lesions,
immunologic status & histopathologic features.
 LEPROSY
CLINICAL FEATURES
History
– Birth or residence in an endemic area.
– A blood relative with leprosy.
– Painless, non- pruritic skin lesions.
– Complaints referable to peripheral neuropathy:
• Numbness, tingling, loss of temprature & pain,
frequent traumatic wounds unnoticed by the
patient.
– Persistent nasal stuffiness, ocular symptoms.
 LEPROSY
CLINICAL FEATURES
• Indeteminate leprosy (early indeterminate
leprosy).
– An early lesion, appearing before the host makes a
definitive immunologic commitment to cure or to
an overt granulomatous response.
– Hypopigmented macule or patch, with or without
an associated sensory deficit in or nearby.
– Spontaneous resolution.
 LEPROSY
CLINICAL FEATURES
Polar tuberculoid leprosy (TT)
• An annular
erythematous/hypopigmentedplaq
ue with peripheral propagation &
central clearing.
• The border is sharply marginated.
• The surface is Scaly, dry, hairless.
• Immunity is strong, as manifested
by spontaneous cure & absence of
downgrading .
 LEPROSY
CLINICAL FEATURES
Borderline tuberculoid leprosy

 Multiple, asymmetric lesions

are the rule, but solitary plaques
are not rare.
 In contrast to TT, typically there
is little or no scaling, less
erythema, induration &
elevation.
 Nerve trunk involvement,
enlargement, or palsies.
 LEPROSY
CLINICAL FEATURES
Borderline borderline leprosy
• Multiple
hypopigmented/erythematous
plaques.
• Usually asymmetric.
• Severity depends upon whether
the patient is ‘leaning’ towards
the lepromatous pole (BL) or the
tuberculoid pole.
• Because of immunologic
instability, the BB posture is
short-lived, and such patients are
rarely seen.
 LEPROSY
CLINICAL FEATURES
Borderline lepromatous leprosy

• Lesions range in number from solitary to numerous

and widespread.
• Multiple plaques, lepromatous like noules.
• Lesions are asymmetric.
• Bilateral ulnar nerve enlargement.
 CLINICAL
FEATURES
Lepromatous Leprosy
• Lack of CMI----unrestricted bacillary replication &
widely disseminated multi organ disease.
• Multiple, poorly defined, erythematous macules,
papules, nodules and plaques.
• Lesions are widespread, usually symmetric in
distribution.
• Affects the face, buttocks and lower extremities.
 LEPROSY
CLINICAL FEATURES
Lepromatous Leprosy
• Infiltration of the skin of the forehead can lead to a
leonine facies
• Hair loss is common on the eyebrows, where it may
progress laterally to medially, or be patchy.
 LEPROSY
CLINICAL FEATURES
Nerve involvement
• Leprosy affects peripheral nerve trunks causing
enlargement & nerve function impairement.
– Sensory loss.
– Motor function loss…weakness, atrophy.
– Autonomic nn impairement….loss in
sweating..dryness of the palms, soles & skin
lesions as well as the skin in general.
 LEPROSY
DIAGNOSIS
• Detailed hx & P/E
• P/E
– Skin lesions…sensory test ( cotton whisp test)
– Peripheral nn trunks…enlargement & tenderness.
 LEPROSY
DIAGNOSIS
• Assesment of nerve function:
– Autonomic function : loss of sweating, hair loss,
dry brittle skin, cracks.
– Sensory function (ST).
• In the areas of affected nerve.
– Motor function.
• Power of muscles (VMT) : Assessing the
strength of movement of the voluntary muscles
supplied by the nerve.
 LEPROSY
DIAGNOSIS
• WHO diagnostic criteria
• One or more is diagnostic:
– Anesthetic or hypoaesthetic
hypopigmented/erythematous skin lesion.
– Thickened peripheral nerve with loss of sensation
&/or weakness of muscles supplied.
– Identified M.leprae in skin samples from skin slit
smear.
 LEPROSY
DIAGNOSIS

• Cotton whisp test for sensation.

 LEPROSY
DIAGNOSIS

• Slit-skin smear
– An incision 5 mm long and 3 mm deep is made
with a small-bladed scalpel.
– From lesions, forehead, eyebrows, earlobes, chin,
extensor surface of the forearm, buttocks and
trunk.
– At least from 2 sites.
– Modified Ziehl–Neelson staining the bacilli gives
a bright red color
 LEPROSY
DIAGNOSIS

• Skin biopsy for histopathology:

– Tuberculoid leprosy:
• A dermal granulomatous infiltrate with
epithelioid cells and Langhans giant cells are
surrounded by lymphocytes.
• Absence of bacilli, even with special stains.
 LEPROSY
DIAGNOSIS

• Skin biopsy for histopathology:

– In lepromatous leprosy:
• Numerous bacilli.
• No/less granuloma.
• An infiltrate is seen in the dermis& subcutis
contains Virchow cells, which are macrophages
with numerous bacilli & lipid droplets in their
cytoplasm
 LEPROSY
TREATMENT
• There are five main principles:
– Stop the infection with chemotherapy[ (multidrug
therapy(MDT)].
– Treat lepra reactions and reduce the risk of nerve
damage.
– Educate the patient to cope with existing nerve
damage, in particular anesthesia.
– Treat the complications of nerve damage.
– Rehabilitate the patient socially and psychologically.
 LEPROSY
TREATMENT
• The first-line anti leprosy drugs are
– Rifampicin
– Dapsone
– Clofazimine
• PB leprosy is treated for 6 months.
• MB leprosy is treated for 12 months.
 LEPROSY
TREATMENT