LEPROSY
HAN SE N ' S D I S E A SE
FOR HEALTH OFFICER STUDENTS
SOLOMON H ( MD, DVR )
Introduction
A chronic granulomatous infection caused by M. leprae
Nowadays, mostly a disease of developing countries
In Ethiopia 5,000 cases are estimated to be infected each year,
presented densely populated in the highlands of Shoa, Gojjam,
s.wollo and Arsi
A disabling, deforming and stigmatizing disease
Primarily affects the skin & nerves
Etiology
M. leprae:
non-cultivable
Gram +ve
non-motile
aerobic
obligate Intera Cellular acid fast bacilli
Don’t grow on
artificial media
Humans – primary
reservoir
Animal reservoir
armadillos
monkeys
chimpanzee
Transmission
Not fully understood
o nasal droplets
○ skin-to-skin contact – not considered as an important
route
Pathogenesis
- M. leprae- lives within cells /macrophages, Schwann cell/
Requires a temperature of ~ 27-33ºC
For disease susceptibility- genetic & environmental factors
Source of infection- untreated multibacillary pts
Inhaled M. leprae multiply
Has brief bacteremic phase before binding to macrophages or Schwann
cells
Depending on the specific CMI, disease can progress or resolve
spontaneously
Less than 5% of pts develop the disease
In the majority, the immunological defense kills all the bacill
Macrophage- play an important role
Classification
Classification determined by clinical & histological changes
1. Tuberculoid Leprosy (TT)
2. Borderline Tuberculoid Leprosy (BT)
3. Mid-Borderline Leprosy (BB)
4. Borderline Lepromatous Leprosy (BL)
5. Lepromatous Leprosy( LL)
-TT & LLp are stable poles
- In all cases of TT & most BT – AFB cannot be found
WHO proposed classification
● paucibacillary/PB/
● multibacillary/MB/
PB leprosy ׃/TT, IL & smear –ve BT/
few lesions /< 5 /
Only one nerve trunk enlarged
AFB negative
MB leprosy ׃/smear +ve BT, BB , BL & LL/
multiple / ≥ 5/
more than one nerve trunk enlarged
AFB positive
Polar tuberculoid leprosy
Immunity is strong
Plaque having annular
configuration
both border is sharply
marginated
Indurated, erythematous,
scaly
solitary
Borderline tuberculoid leprosy
Immunity is strong to restrain
infection
Somewhat unstable
Plaque, papular
Annular configuration
Both borders are sharply
demarcated
Little or no scaling, less
erythema, less induration & less
elevation
may have sharply marginated
satellite papules
Multiple, asymmetric
Borderline leprosy
mid-zone of the granulomatous
spectrum
- most unstable
- patient quickly down
regulates or upregulate
- annular lesion
Patient are rarely seen
Borderline lepromatous leprosy
Generally, annular & plaque
lesion- numerous, asymmetric
Lepromatous like nodules- if
numerous symmetric
Nerve trunk palsies → motor
& sensory deficit
Lepromatous leprosy
Lack of CMI → unrestricted
proliferation & widely
disseminated multiorgan
disease
Poorly defined nodules- most
common lesion/up to 2cm/
Symmetrical distribution
Diffuse dermal infiltration
In diffuse non-nodular LL-
enlargement of ear lobes,
widening of the nasal root &
fusiform swelling of the
fingers
Indeterminate leprosy
An early lesion appearing before
the host makes a commitment to
cure or to an overt
granulomatous response
Hypopigmented macule or patch
Associated sensory deficit may or
may not be present
AFB , if present in small number
Have neither typical tuberculoid
or lepromatous histological
patterns
More than 75% will heal
spontaneously
Pure neural leprosy
Involve only the nerves without skin changes
Sensory, motor deficit
Thickened nerve with or without nerve damage
Skin smears are negative
As PB if only one nerve & nerve biopsy smear is –ve
As MB if ≥ 2 nerves & nerve biopsy smear is +ve
Clinical findings
History
Risk for those born or resident in endemic area
Duration of symptoms, history of previous Rx
Peripheral neuropathy, nasal stuffiness, ocular symptoms
- 90% Hx of numbness first→ sometimes years before the skin lesions
appear
/Temp. → light touch → pain → deep pressure/
Especially apparent in hands & feet
complaint of burn or an ulcer
Examination ׃
• Cutaneous lesions
- character, number & distribution
- Hypopigmented macule, plaque
- may or may not be hypoesthetic
• Neuropathies
- assess for hypoesthesia /light touch, pinprick/, anhidrosis
-ulnar, peroneal N, median, radial cutaneous N, post tibial N
- enlargement , tenderness
Palpating the nerves:
Peripheral nerves are
examined for:
√ Enlargement or
thickening
√ Tenderness
When palpating a nerve
always use 2 or 3 fingers.
The nerve should be rolled
over the surface of the
underlying bone.
The left and right side must
always be compared.
Palpating the
nerves:
- enlargement /tenderness
- use 2 or 3 fingers should be
rolled over the surface of
the
underlying bone
- The left and right side must
always be compared
Eye involvement
Lagophthalmos
- inability to close the eye
- from involvement of the
facial N
▫ Decreased corneal reflex
- ophthalmic branch of
trigeminal N
- dry eye
Corneal ulceration,
scarring, blindness
Extra ocular
Madarosis
Loss of hair (eye brow)
Leonine facies
- The skin is thrown into
folds
Examination of the hands & feet
Look for ○ skin cracks,
wounds
○ clawed fingers
& toes
○ foot drop
○ wrist drop
○ shortening &
scarring of
fingers & toes
Peripheral nerve change ׃
1- Nerve enlargement
2- Sensory impairment
3- Nerve trunk palsies
4- Stocking- glove
pattern of sensory
impairment
5- Anhidrosis
DX
Criteria for Diagnosis
Cardinal signs of leprosy
1. Definite loss of sensation in hypopigmented or
erythematous lesion.
2. Thickened peripheral nerve with loss of sensation &
or weakness of muscles supplied.
3. Presence of AFB in slit skin smear
In smears M. leprae is quantified by the biopsy index (BI), a
logarithmic scale as to the numbers of bacilli per oil immersion
field (OIF)
BI of 6 is 1000 or more bacilli/OIF
5 is 100-1000 bacilli/OIF
4 is 10-100 bacilli/OIF
3 is 1-10 bacilli/OIF
2 is 1 bacillus/OIF
1 is 1 bacillus/ 1-10 OIF
0 is no bacillus/100 OIF
SSS only detect bacilli present at a concentration greater than 10
‘4/ g tissue, and so cannot be used as a test of microbiological cure
Slit skin smear
Lesions and cooler areas of the skin, such as the earlobes ,
elbows, and knees, and stained with acid-fast stains
An incision 5 mm long and 3 mm deep-blade is turned at right
angles to cut
Fluid and pulp from the dermis
is detected by modified Ziehl-Neelsen stain the bacilli a bright
red color
• Biopsies - confirmation of diagnosis & classification of leprosy.
• histologic features of disease correlate with the clinical pattern of
disease.
DIFFERENTIIAL DIAGNOSIS.
consider leprosy as a cause of peripheral neuropathy.
Macular & Patch lesions Papular to Nodular lesions
Birthmarks Cutaneous leishmaniasis
Vitiligo PKDL
pityriasis alba lymphomas
pityriasis versicolor
Plaques and annular lesions
Tinea corporis
granuloma multiforme
sarcoidosis
cutaneous tuberculosis
TREATMENT
Main principles of treatment:
Stop the infection with chemotherapy.
Treat reactions and reduce the risk of nerve damage.
Treat the complications of nerve damage.
Rehabilitate the patient socially and psychologically.
PB – MDT regimen
Child <10yrs
Rifampicin 300mg monthly
Dapsone 25mg daily
10 – 14yrs
Rifampicin 450mg monthly
Dapsone 50mg daily
>15yrs
Rifampicin 600mg monthly
Dapsone 100mg daily
MB – MDT regimen MB – MDT regimen
Child <10yrs 10 – 14yrs
Rifampicin 300mg Rifampicin 450mg
monthly monthly
Clofazimine 100mg Clofazimine 150mg
monthly monthly
Clofazimine 50mg Clofazimine 50mg every
2x/week other day
Dapsone 25mg daily Dapsone 50mg daily
MB – MDT regimen
>15yrs
Rifampicin 600mg monthly
Clofazimine 300mg monthly
Clofazimine 50mg
Dapsone 100mg daily
Reactional state
• Problems – related Leprosy
• Immunologicaly driven distinctive, tissue destructive ,inflammatory processes
that appears suddenly in any form of leprosy
• Precipitating factor
MDT
Pregnancy
Inter current infections
Vaccination (BCG)
• Usually as complications during treatment but they may occur before treatment
or after
• Reactional states
Type-1 (Reversal )
Type-2 (ENL)
Type I
• enhanced T cell-mediated immune response to M. leprae
(Type4 DTH)
• can occur spontaneously.
• Most common in the first year of treatment(peak time 1st 6
months of Rx) or well after treatment has stopped.
• Particularly common in BL patients, but seen in BB , or BT
patient.
Clinically - Existing skin lesions become erythematous or
edematous and may desquamate.
new lesions can appear
In severe cases, ulceration can occur
Often multiple
edema of face, hands or feet is the presenting symptom,
Often associated with neuritis (severe).
• Nerves often become tender with/out loss of sensory and
motor fun
constitutional symptoms are unusual.
ENL reaction
• regarded as an immune complex disorder.
• supported by the presence of immunoglobulin and complement in
the lesions and circulating immune complexes.
• occur before, during, or after chemotherapy.
• median time of onset is 1 year after onset of treatment.
• Clinically-crops of new painful and tender bright-
pink,dermal and subcutaneous nodules arising in clinically
normal skin
• Lesions- targetoid, vesicular, pustular, ulcerative,or necrotic.
• Involvement of both upper and lower extremities is the rule.
• favor the extensor arms and medial thighs, face
systemic symptoms- fever , malaise, anorexia
Arthralgias and arthritis are more common in ENL than --
neuritis, adenitis,
orchitis/ epididymitis, uveitis & iritis.
In severely involved patients, episodes can be frequent to virtually
unremitting. -Chronic ENL .
TREATMENT
AIM
controlling acute inflammation.
easing pain.
reversing progressive nerve and eye damage
reassuring the patient.
Multidrug therapy should be continued.
Type-1 (Reversal) Reaction
mild reactions –(oedema & erythma skin lesions only) without neurologic complications or severe systemic
symptoms or findings.
Treatment may be supportive.
Bed rest /ASA or NSAID
Examined P 1 week- Sn of rxn –Rx should Continued for another week
Mild rxn > 6 weeks –severe
Type I reaction RX
Severe rxn
prednisone starting at a dose of 40 to 60 mg/day(0.5-1 mg/kg)
duration are determined by the clinical course of the reaction.
Once reaction is controlled, the prednisone may need to be tapered
slowly-over months
• Nerve abscesses - need to be surgically drained immediately
• response of neuritis is not prompt rest & splinting of affected
extremities
Type II
Thalidomide treatment of choice.
• initial recommended dosage is up to 400 mg/day (>50 kg)
• milder cases, treatment may be started (100 to 200 mg/day)
Systemic corticosteroids are also effective
• high-dose steroids (80-mg daily,tapered down rapidly)
• ENL frequently recurs, steroid dependency can easily develop
Clofazimine in higher doses (up to 300 mg/day) is effective in ENL,
and may be used alone or to reduce corticosteroid or thalidomide
doses.
