Post on 26-Dec-2021
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CLINICAL FEATURES OF LEPROSY
Name of the Doctor :-Dr. Manisha Nijhawan
WHAT IS LEPROSY
� It is a chronic granulomatous disease
� World's oldest recorded disease
� Stigmatized disease
� Gerhard Henrick Armauer Hansen
� Word ‘leper’‘leper’ is a Greek word meaning ‘scaly’‘scaly’
� Multibacillary cases ( LL & BL) are the most important
source of infection in the community (doubt about
tuberculoid end)
� Current view is that all patients with active leprosy must
be infectious
VARIOUS NAMES FOR LEPRA
• Leprosy – Europe & U.S.A
• Lepre – France
• Aussatz – Germany
• Lebbra – Italy
• Prokaza – USSR
• Taimafu – China
• Raibyo - Japan
• Kushtha - India
WHAT IS NOT LEPROSY
� Skin patches which
� Have normal feeling
� Are present from birth
� Cause itching
� Are white, black, dark red or silver coloured
� Appear and disappear periodically
� Spread quickly
� Signs of damage to hands/feet/face without loss of
sensation
� Due to other reasons like injury, accidents, burns, birth
defects
� Due to other diseases like arthritis
� Due to other conditions causing paralysis
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CASE DEFINITION
� Individual who has not completed the course of
treatment, and has atleast one of the three cardinal signs
� Hypopigmented or erythematous skin lesion(s) with definite
loss/impairment of sensation
� Involvement of the peripheral nerves, as demonstrated by
definite thickening with sensory impairment
� Skin smear positive for acid-fast bacilli (AFB)
� Patients who relapse after completing a full course of
treatment
� It does not include cured persons with late reactions or
residual disability
WHO ARE AT RISK
� It can affect all ages and both sex
� Mainly affects:
� Skin
� Eyes
� Peripheral nerves
� Mucosa of the upper respiratory tract
PORTAL OF EXIT
� Nose – major portal of exit
� Harbors millions of M.leprae in nasal mucosa
� Can also exit through ulcerated or broken skin of
biologically positive cases
MODE OF TRANSMISSION
� Droplet infection
� Via aerosols
� Skin , GIT & respiratory tract
� Contact transmission
� Direct – Skin to skin
� Indirect – with soil and fomites
� Other
� Via breast milk from lepromatous mother
� Insect vectors (Aedes aegypti)
� Tattooing needles
Good immune
status
Tuberculoid
BTBB
BL
Most labile
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Entry of M.lepra into susceptible host
Primary focus
Spread to different sites
Sites favourable
to M.lepra
Sites unfavourable
to m.lepra
No lesions developAnesthesia, muscle destruction
Nerve destruction
Tuberculoid granuloma formed
Nerves invaded by lymphocytes & histiocytes (become epitheliod cells and groups of these become giant cells)
Intraneural infection is recognised by body
From here bacilli enters neighbouring schwann cells and intraneural infection spreads
Bacilli enter schwann cells & multiply
HISTOPATHOGENESIS OF TUBERCULOID LEPROSY
INDETERMINATE LEPROSY
� Early stage of leprosy evolution
� Observed only in endemic areas especially in children
�� Clinical featuresClinical features
� 1- 3 ill defined hypopigmented macules ranging in size from
1 – 5 cm, commonly seen over the face, trunk & extremities
� Sensations impaired & nerve thickening will be present
� No bacilli in SSS
� Heals spontaneously, but 30 % may progress to determinate
type
Hypopigmented macule of Indeterminate leprosy
TUBERCULOID LEPROSY
� Patient reports early for medical examination due to
nerve involvement
� Types
� Neural
� Dermal
� Mixed
� Neural
� Consists of pain, loss of feeling, tingling, muscle weakness &
paralysis
� Superficial feeder nerve or a single regional nerve may be
nodular
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TUBERCULOID LEPROSY CONT..
� Dermal
� Plaques
� 1 – 3 number, 0.5 – 30 cm in size located anywhere in the body
� Usually asymmetrical & unilateral
� Markedly thickened & edematous with sharply defined edges
� Surface: dry rough & irregular with hair loss & impairment of
sweating
� Macules
� Sharply defined hypopigmented, varying in size from 0.5 cm to
large area, anesthetic
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BODERLINE TUBERCULOID
� Well defined infiltrated plaques with satellite lesionssatellite lesions,
dry surface & anhidrotic
� Lesions may be hypopigmented or slightly erythematous
macules
� Pain & temperature sensations are usually lost or
markedly impaired
� Few nerves aymmetrically thickened, anesthesia &
distribution of the nerve
WELL-DEMARCATED, ANNULAR PATCH OF BORDERLINE
TUBERCULOID LEPROSY AND A SATELLITE LESION
MID BODERLINE
� Very unstable
� 10 – 20 lesions
� Bilateral but asymmetrical infiltrated plaques with a punched
out appearance (inverted saucer appearance(inverted saucer appearance))
� Plaque is depressed in the centre with a sharp inner edge
� Bizarre shape with irregular borders & a geographic
appearance
� Lesions are hypoaesthetic & nerves show asymmetrical
thickening
TYPICAL BORDERLINE LEPROSY LESION WITH CENTRAL
“IMMUNE AREA”
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BODERLINE LEPROMATOUS
� 20 lesions
� Lesions may be macules, annular plaques or even
nodules
� Shiny, copper coloured & more infiltrated in the centre
than in the periphery
� Lesions are hypoaesthetic
� Peripheral nerve involvement is bilateral
Numerous, widespread, shiny, raised patches of
borderline-lepromatous leprosy, sloping
towards the periphery
Numerous, copper-coloured, raised patches of
borderline-
lepromatous leprosy, sloping towards the
periphery
LEPROMATOUS LEPROSY
Depressed cell immunity
Bacilli which enter Schwann cells and Perineural cell multiply
unchecked
Perineural multiplication impairs competency of perineurium to stabilise the
intraneural environment
(onion peel appearance – infiltration of perineurium with histiocytes &
plasma cells)
Some bacilli are also liberated out when schwann cells &
perineural cells are destroyed
Engulfed by histiocytes
Wandering macrophages instead of becoming fixed
epitheliod cells
LEPROMATOUS LEPROSY CONTD…
Bacilli multiply in wandering macrophages while travelling to other
parts of nerve & tissues
Swollen macrophages packed with bacilli known as lepra cells (Virchow cells)
&
Masses of bacilli which accumulate in their cytoplasm called globi
LEPROMATOUS LEPROSY CONTD… LEPROMATOUS LEPROSY CONTD…
� No nerve involvement or skin lesions noticed by the
patient so difficult to diagnose
� 2 early symptoms which helps in diagnosis are
� Nasal ( stuffiness, crust formation & blood stained discharge)
� Bilateral edema of legs & ankles
� Skin lesions are multiple & bilaterally symmetrical
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TYPES OF LL
� Diffuse LL
� True subtype of LL
� Results from gradual coalescing of various numerous vague
macules of macular LL
� Slight infiltration (appreciated well by touch)
� Thickness of skin due to diffuse infiltration
� Ear lobes are shiny and thickened (buddha ears)
� Thinning or loss of eyebrows
� Infiltrated LL
� Areas of marked infiltration
� Advanced stage of macular LL, easily visible infiltration
� Nodular LL
� Disease advances and the nodules appear
� Infiltrated plaques accentuate the skin folds
1.SKIN MANIFESTATIONS
� Macules
� Erythematous or coppery or hyperpigmented, small numerous
� Shiny surface
� Ill defined edges
� Show no loss of sensation or hair growth
� Papules & nodules
� Skin colored or erythematous or coppery firm on palpation of
varing size
� Result of progressive deterioration of macular, diffuse or
infiltrative forms of LL
� Facial lesions
� Face becomes generally thickened
� Nose become swollen & broadened
� Supraciliary madarosis with ciliary madarosis
� Thickening & nodular lesions of both ears
� Ichthyotic changes of thighs, legs & arms
� Woody hard edema of legs
� Hoarse Voice
� Loss of upper incisor teeth
� Glove and stoke anaesthesa (shortening of finger and toes)
Numerous, highly bacilliferous, shiny,
glossy
nodules of lepromatous leprosy
Ill-defined, coalescing, hypopigmented
patches
of multibacillary leprosy studded with
multiple nodules
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LEONINE FACIES
� Thickening of lesions of forehead causing deepening of
natural lines
� Thickening of ear lobes
� Loss of eye brows
� Depression of nasal bridge
2.NERVE INVOLVEMENT
� Sensory, motor & mixed involvement
� Bilaterally symmetrical peripheral nerve thickening
� Thickened nerves feel firm & smooth, localized to more superficial areas of the body
� Sensory impairment
� Light touch, pain & temperature
� Dissociative anesthesia can occur
� Bilateral glove & stocking anesthesia affecting all the 4 limbs
� Slowly developing fibrosis due to reaction against dead bacilli even after the treatment completion
� Leads to blister formation (reflex dilatation of skin capillaries due to damage of dermal nerves)
� Motor involvement
� Facial nerve(facial palsy) (earliest sign is lower lid palsy)
� Ulnar nerve (claw hand or main en griffe)
� Median nerve (ape hand or main de singe)
� Lateral popliteal nerve (Foot drop)
� Posterior tibila nerve (claw toes or hammer toes)
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3.NAILS OF FINGERS & TOES
� Appear dry
� Lusterless
� Shrunken
� Narrowed
� Longitudinally ridged
4.MOUTH, PHARYNX & LARYNX
� Nasal stuffiness, blood stained discharge, crusting
� Irregular mucosa, perforation of nasal septum, nasal
� Cartilage destruction leading to nasal collapse
� Papules on lips, nodules over tongue, palate, uvula(may
ulcerate & perforate)
� Larynx –thickening, nodulation & ulceration & fibrosis
of vocal cords produce hoarseness
5.EYE INVOLVEMENT
� Superficial punctate keratitis
� Pannus formation leading to sclerosing keratitis
� Acute Iritis
� Secondary cataract & glaucoma
� Ciliary staphyloma
� Corneal anesthesia due to 5th nerve palsy
� Corneal ulceration
� Lagophthalmos (7th nerve involvement)
6.BONE INVOLVEMENT
� Hands
� Distal phalanges are atrophied
� Absorption leading to shortening of fingers
� Carpals & metacarpals are spared
� Feet
� Atrophic changes occur in phalanges,metatarsals & tarsals
� Distal phalanges become thinned by rarefying osteitis known as
concentric bone atrophy
� 5th metatarsals – ‘‘penicillingpenicilling’ or ‘sucked candy stick’ ’ or ‘sucked candy stick’
� Skull
� Atrophy of the anterior nasal spine (nasal collapse)
� Atrophy of maxillary alveolar process (loosening of upper
central incisor teeth) gives rise to faciesfacies leprosaleprosa
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7. Testes
� Testicular atrophy leading to impotence
� Gynecomastia
� Female reproductive organs are not affected
8. Kidneys
� Renal amyloidosis, glomerular nephritis, interstitial
nephritis,
� Pyelonephritis & renaltuberculosis (non specific & self
limiting)
10. Muscles10. Muscles
� Firm well defined, nontender masses of various sizes within
the muscles leading to stiffness & discomfort
� Histopathology reveals interstitial myositis with granular
bacilli between muscle bundles
11. Lymph nodes11. Lymph nodes� Generalized lymphadenopathy
12.KIDNEY INVOLVEMENT
� Sparing of renal parenchyma
� Glomerulonephritis (6-50%)
� Membranoproliferative (11-43%)
� Diffuse proliferative
� Rapidly progressive
� Mesangioproliferative
� Chronic glomerulonephritis
� Acute tubular necrosis
� Pyelonephritis
� Interstitial nephritis
� Tubular degeneration
� Hyalinization of small and madium sized renal vessels
� Acute and chronic renal failure
� Amyloid deposition(2-55%)
� Nephrotic syndrome
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VARIANTS OF LL
1)1)HistoidHistoid leprosyleprosy
� Introduced by Wade
� Clinical features
� Firm, erythematous, well demarcated juicy cutaneous
� Subcutaneous shiny glistening nodules which appear on the
patients in relapse
Multiple (in places crusted) nodules of
histoid leprosy
2.L2.LUCIOUCIO LEPROSYLEPROSY
� Described by Lucio & Alvarado in Mexico in 1852
� In Mexico c/d ‘Lepra bonita’
� Diffuse non nodular type
� Polar form
� Thickening of upper eyelids
� Uniform diffuse shiny infiltration of the entire skin of the
body
� 1st symptom- Numbness of the hands or feet or nasal
congestion, epistaxis, hoarseness, edema of feet
� 1st sign - Madarosis
� Type 2 like reaction develops which is severe and
associated with infarcts of the skin (Lucio’s
phenomenon)
� Normocytic normochromic anemia is the rule
PURE NEURAL LEPROSY
� 4.3-10.7 % of cases in India
� M > F
� Age 20-40 years
� Majority of patients present with sensory impairment, some with nerve pain or deformity
� Majority of cases are mono neuritic
� Ulnar, median & lateral popliteal nerve most commonly affected
� Can be diagnosed by thickened nerves, sensory impairment, negative SSS & absence of skin lesions
� Nerve biopsy is diagnostic
� Spectrum ranges from TT - BL
LAZARINE LEPROSY
� Unusual expression of BT leprosy in spontaneous ulceration of skin lesions
� d/t exaggerated hypersensitivity in type 1 reaction
� H/p – Necrosis due to extreme cellular hypersensitivity
� T/t- Antileprosy drugs + Sys corticosteroids
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INOCULATION LEPROSY
� Leprosy following scarification/tattooing, vaccination,
needle stick injury or trauma
� First reported by Lowe and Chatterjee in 1939
� Once the lepra bacilli are introduced, they may initially
remain dormant for a few days to many years
PREGNANCY AND LEPROSY
Effects of pregnancy on the woman with leprosy
� Worsening of the leprosy
� Women already infected with M.leprae & incubating leprosy
� Established leprosy (deterioration of nerve function)
� Intercurrent infections
� Decreased resistance to viral infections & also to
pneumococcal infection & malaria
� Increased incidence of lepra reaction
� Type 1
� During puerperium (upgrading)
� During pregnancy (downgrading)
� Type 2
� In 3rd trimester & puerperium, may also complicate in early
pregnancy because of stress
AFFECT ON INFANTS
� LBW
� High risk of contracting leprosy from
� Mother if she is an open case
� Hidden source of infection in the home
� If child is born in a leprosarium
TYPE 1 LEPRA REACTION
� Type of delayed type hypersensitivity reaction
� Ex- Coombs and Gell Type IV hypersensitivity reaction
� Antigen from breakdown leprosy bacilli reacts with T
lymphocytes
� Associated with a rapid change in CMI
� Seen in borderline patients d/t immunological instability
� REVERSAL REACTION:
� If patient is under treatment, there will be rapid increase in CMI,
it becomes upgrading reaction
� If the reaction is associated with a reduced immunity, it is termed
as downgraded reaction
� Borderline patients may upgrade to tuberculoid type(TT)
but may form a subgroup secondary tuberculoid
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� Upgrading reaction may also occur in sub-polar LL
under treatment
� These patients rapidly regain their lost immunity and the
new skin lesions have the features of BL
� There will be skin manifestations, systemic and nerve
involvement, motor disturbances
Type 1 lepra reaction
Type 1 lepra reaction
TYPE 2 LEPRA REACTION
� Immune comlex syndrome
� It is a humoral antibody syndrome
� Example of type III hypersensitivity
� Occurs exclusively in lepromatous leprosy and
occasionally in BL
� It is unusual for it to occur in first 6months of treatment
Erythema
nodosum leprosum
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LUCIO REACTION
� Lucio reaction
� Mexico and caribbean
� Seen in diffuse form of lepromatous leprosy
� Purplish lesions of the hands and feet
� Irregular shapes erythemtous plaques
� May resolve spontaneously or undergo ulceration or
necrosis
� Histology: ischemic necrosis secondary to endothelial
� Parasitization by AFB; thrombosis in deep vessels
� Systemic corticosteroid is the Tx of choiceLucio reaction
CONCLUSION
� Leprosy- chronic infectious disease caused by Mycobacterium leprae
� Spectrum of manifestations –reflect CMI
� Asymptomatic hypopigmented macule to mutilating deformities
These ancient masks show the deformities that many ancient cultures
had associated with leprosy
THANK YOU
� Silent LL
� Auto aggressive hanseneases
� Panniculitis presented as
� Localised LL