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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