LEPROSY
DR.S.AHAMED UWYSE
CONSULTANT DERMATOLOGIST
COLOMBO NORTH TEACHING HOSPITAL
RAGAMA
What is leprosy ?
Chronic granulomatous disorder caused by mycobacterium
leprae which mainly affects the skin and nerves , but other
organs may also be involved .
History and Epidemiology
Considered to be originated in India in 1400 yrs B.C
Highly prevalent in India, south east Asia, central Africa and also in
central and north America.
Not common in Europe.
Prevalence rate in Sri Lanka 0.98%
Age and sex
rare among infants
may occur at any age group
peak age –> 10 – 20 yrs
Male > female .
Incubation period - months to years
Poor socio-economic conditions favours the spread of the
disease.
Transmission of the disease
Mainly by droplet inhalation.
By direct contact - very rare
Spread by clothes and bed linens - very rare
Transmission by arthropods – very rare.
Pathogenesis
Bacilli entering the respiratory system entering the ph. Nerves via the
blood stream multiply within the Schwan cells damaging the
cells and large number of bacilli released may reenter the Schwan cells or
taken up by the macrophages activate T lymphocytes
IL1, IL2 , TNF alpha and other cytokines released induce inflammation
at the site of infection.
Then the bacilli found within the nerve travel through the nerves reaches
skin the causing inflammatory changes in the skin , resulting in the clinical
lesions
If the CMI is good , multiplication of the bacilli within the nerve is limited
and may cause only nerve injury or some bacilli may reach the skin and may
cause skin lesions ( 1- 2 lesions ) - PB leprosy
If the CMI is week , bacilli proliferate in large number within the nerves and
may enter the circulation causing multiple lesions in the skin and other
organs as well - MB leprosy
Classification of Leprosy
(Ridley and Jopling classification )
leprosy
Tuberculoid leprosy Borderline leprosy Lepromatous leprosy
BI 0 BI 5+ 6+
Borderline leprosy
Borderline tuberculoid Borderline borderline Borderline lepromatous
BI 0 , 1+ BI 2+ 3+ BI 3+ 4+
WHO classification
paucibacillary (PB) ( < 5 lesions )
multibacillary (MB) ( > 5 lesions )
Other types of leprosy
Indeterminate leprosy
Histoid leprosy
Lucio leprosy
pure neural leprosy
Tuberculoid Leprosy
Usually a single patch, but 2-3 asymmetrical patches may occur.
Lesions may be macular or plaque or psoriasiform
Patches may be hypopigmented or erythematous or bronze in colour.
Usually lesions are well defined with slightly elevated edge
Patches may look dry and scaly due to impaired sweating
loss of hair on the patches
Impaired sensation (touch, temperature, pain ) over the patches.
facial lesions – sensation may be present due to overlapping of nerve
supply
features of nerve involvement
the nerves affected becomes thickened and tender .
sometimes nerve abscess may be present
nerves entering or leaving the lesions or the regional nerves (ulnar,
median post.tibial, greater auricular, facial , trigeminal and so on)
may be enlarged
Complication due to nerve involvement.
Claw hand, wrist drop, foot drop, lag ophthalmus, corneal ulceration
and so on.
nerve abscess due to caeseation .
trophic ulcers
loss of fingers and toes
Regional LN may be involved and become enlarged
TT usually stable , but may go into BT rarely
Peripheral nerve involvement in leprosy
Supra orbital N
Facial N
Greater auricular N
Supra clavicular N
Radial N
Cut. Branch of the radial N
Median N
Ulnar N
Common peroneal N
Posterior tibial N
Sural N
Features of nerve involvement in leprosy
thickened;
hard;
irregular in consistency;
tender;
spontaneous pain;
nerve function deficit (sensory, motor, autonomic).
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Palpating supra orbital N
Thickened GAN
Cutaneous branch of the
Radial N
Thickened median N
Palpating CPN
Sup PN
Palpating PT N
Borderline leprosy
• Usually unstable, may down grade towards lepromatous pole or may
reverse to tuberculoid pole.
• Nerve involvement – wide spread and asymmetrical
• More prone to get lepra reactions (BB, BL )
• all BL treated with MB treatment except BT
Borderline tuberculoid leprosy
• Number of lesions may be single or multiple (2-3)
• Still margins are well defined, but some part of the margin may be
illdefined,
• Lesions are asymmetrical and more scaly.
• Shape of the lesions similar to TT.
• Satellite lesions found around the main lesions.
• Nerve involvement – multiple and asymmetrical
Boderline Tuberculoid
BB Leprosy
• multiple lesions are present
• asymmetrical
• less well demarcated, somewhat shiny, sloping edge giving the
inverted saucer appearance
• annular lesions are common
• Sensory impairment of the patches is moderate (BT- marked)
• Impairment of hair growth is moderate
• Ph. nerve involvement – widespread and asymmetrical.
• Single large patch ( > 10 cm ) , considered as BL or BB
• Biological index (BI ) - 2 + or 3+
BB leprosy
Single large patch – BB or BL leprosy
BB borderline leprosy
BB Leprosy with multiple burn injury
Borderline lepromatous leprosy
• Many in number, smaller lesions
• Roughly symmetrical
• Plaque lesions shows characteristic inverted saucer appearance (sloped
edge).
• Ph.Nerve involvement – wide spread and less asymmetrical
• Histology – presence of grenz zone ,
diffuse granuloma with more macrophages and less giant cells and
epethiliod cells.
BL Leprosy
In these forms of leprosy sensation can be
normal or impaired over the patches , nerves
may be enlarged or normal, slit-skin smear
should be positive
Lepromatous Leprosy
• Mainly involves skin and mucous membrane , but it may involve
systemic organs as well (eye, testes, liver, spleen, bone marrow, lymph
nodes and so on ).
• CMI impaired , so large number of bacilli seen in the lesions
• LL may downgrade from borderline leprosy or starts as LL itself.
Clinical presentation
it occurs in sub polar type (LLs)
multiple, smaller, ill-defined, symmetrical, hypo pigmented or skin coloured or
copper coloured macules.
sensation intact at early stages.
multiple shiny symmetrical plaques with sloping edge.
it mainly occurs in polar type (LLp)
usually starting on the earlobe, face, limbs and trunk.
multiple, symmetrical , painless , soft or hard , small or large
uninvolved skin may be reddish brown or copper in colour , and dry and
scaly
diffuse infiltration of the skin with
nodules
nasal discharge, septal perforation, saddle nose.
sub fertility , gynaecomastia.
late prsentation
glove and stocking type of sensory loss.
conjunctivitis , keratitis , uveitis
lag ophthalmus , Corneal insensitivity .
due to lymphedema
erythema may be present – mimick cellulits
Sometimes looks eczematised
swelling of the fingers and toes due to inflamation (periostitis) .
bone lesions may be present – osteoprotic , osteolytic
Lepromatous leprosy in a child
It causes no discomfort, and the patient does
not feel sick. It is
“ just there”. No signs of nerve damage or
large nerves. Many bacilli in some lesions
Lepromatous leprosy
(Leprosy bonita)
On palpation diffuse infiltration of the skin of
the whole face except upper lip. A vague edge
is present along the naso-labial fold. (Upper lip
normal skin, rest of face infiltrated.)
Diagnosis was accidentally made on the biopsy
of a nodule on the arm.
Slit-skin smear: - bacteriological index 6+
at all sites!
Diagnosis
Clinical diagnosis
Laboratory investigation
Clinical diagnosis
The 3 cardinal signs of leprosy
1. Skin patch with loss of sensation;
2. enlarged peripheral nerve;
3. positive slit-skin smear.
Testing for loss of senstation
Loss of sweating on the
patch
Lab Investigation
Skin biopsy
• done in doubtful cases
• histology showing well defined tuberculoid granuloma with more epithelioid cells
and giant cells cuffed by lymphocytes in T T leprosy –
epithelioid cell granuloma
• Skin appendages and nerves may be involved.
• In Borderline cases, grenz zone present, granuloma may be slightly diffused .
• In lepromatous leprosy, diffuse granuloma with more macrophages
(Virchow’s cells) than the epetheliod cells and giant cells –
macrophage granuloma , grenz zone present
Histiod leprosy
Slit skin smear
1. Smear
2. Dry
3. Fix
4. Stain (Ziehl-
Neelsen)
solid
granular
BI (bacteriological index)
1+ - 6+
Morphological index
Lepromin test.
Abs test
PGL1abs (phenolic glycolipid ) detecting by RIA or ELISA technique
useful test in detecting early lesions , pure neuritic leprosy
not freely available.
Culture
can not be cultured in artificial media
grown in mouse foot pad , Armadillo and
monkeys.
PCR
Differential diagnosis in leprosy
Pityriasis versicolor, pityriasis alba
Tinea corporis
Vitiligo
Granuloma annulare
Lupus vulgaris
Syphilis
Cutaneous leishmaniasis
Psoriasis
Sarcoidosis
Mycosis fungides
Treatment of leprosy
PB drug therapy for T T and BT leprosy
MB drug therapy for borderline (except BT) and
lepromatous leprosy
PB treatment
• PB adult ( > 14 yrs ) PB child ( 14 – 10 yrs )
Rifampicin 600 mg 1st d ay Rifampicin 450 mg
Dapsone 100 mg Dapsone 50 mg
dapsone 100 mg daily Dapsone 50 mg
• Children < 10 yrs
Rifampicin 10 mg / kg b.w
Dapsone 2 mg / kg b.w
( clofazimine 1 mg / kg b.w)
MB Treatment
MB adult ( > 14 yrs ) MB child (14-10 yr)
Rifampicin 600 mg 1st day Rifampicin 450mg
dapsone 100 mg Dapsone 50mg
clofazimine 300 mg clofazimine 150 mg
dapsone 100 mg daily dapsone 50mg
clofazimine 50 mg clofazimine 50 mg EOD
Alternative drugs in leprosy
Ofloxacin
Clarithromycin
Minocycline
Nerve palsies and deformities
Corticosteroid therapy
Physiotherapy
Splints
Special foot wears
Corrective surgery
Other types of leprosy
• Indeterminate leprosy
• Histoid leprosy
• Lucio leprosy (lepra bonita )
common in Mexico
• Neural leprosy
pure nerve involvement without skin lesions.
single or multiple nerve involvement.
investigation - NCS , sural N biopsy.
Treatment - PB or MB depends on histology.
more than one nerve - MB
Indeterminate Leprosy
Lepra reaction
• It is a immunological reaction.
• May occur spontaneously or after starting treatment.
• All BL and LL may cause usually , but TT usually not.
• Leprosy treatment should not be stopped .
Type 1 reaction
• Type 4 immunological reaction.
• Occurs in BB, BL usually (raely in BT )
• Existing lesions becoming erythematous, thickened and enlarged.
usually no new lesions
• Nerve involvement common
• Swelling of the hand, feet and face
• Reversal reaction – moving towards Tub.pole
down grading reaction – moving towards Lep.Pole.
• Lasting for few weeks to few months
Type 2 reaction (ENL )
• Due to type 3 immun. reaction
• Occurs usually in LL and less commonly in BL
• Multiple tender nodules all over the body
may enlarge and ulcerate.
• Systemic disorder
fever, malaise may be present
any organ may be involved ,
eye, testes, kidney, muscles, bones, liver and spleen, lymph nodes and
so on .
• Swelling of the hands, feet and face.
• May last for few months.
Necrotic ENL
Bullous ENL
Mixed reaction ( type 1 and type 2 ) can also occurs
Treatment
• Continue leprosy Rx .
• Corticosteroid therapy.
• Clofazimine - 100 – 300 mg / day.
• Thalidomide - start with high dose (300 – 400 mg /day ) , then
gradually reduce to 100 mg / day.
• methotrexate
Drug reactions
• Dapsone
haemolytic anaemia ,methaemoglobinaemia, hepatitis , skin rash
dapsone syndrome
Ph. Neuropathy.
• Rifampicin
• Clofazimine
hyper pigmentation ( brownish )
References
• Lockwood DNJ and Bryceson ADM , leprosy , in:
Champion RH,Burton JL, Burns DA,Breathnach SM, eds
Textbook of Dermatology, 6th ed, Oxford-
Blackwell science 1998, 681 -708.
• Yawalkar SJ , leprosy 6th ed , 1994.
Thank You
