LEPROSY

Leprosy I Leprosy I Introduction Introduction Epidemiology Epidemiology Bacteriology Bacteriology Classification Classification Clinical features Clinical features Leprosy II Reactions Diagnosis Treatment Rehabilitation

Introduction Chronic granulomatous disease Chronic granulomatous disease Caused by Mycobacterium leprae Caused by Mycobacterium leprae Mainly involves the peripheral nerves and skin Mainly involves the peripheral nerves and skin Other organs may involve: Other organs may involve: Mucosa of mouth Upper respiratory tract EyesBones Testes etc

Historical aspect of leprosy Oldest disease known to mankind Oldest disease known to mankind Word leper comes from Greek word “scaling” Word leper comes from Greek word “scaling” Earliest description from India in 600BC Earliest description from India in 600BC Kustha Roga & attributed punishment or curse of God Kustha Roga & attributed punishment or curse of God M. leprae discovered in 1873 by Armauer Hansen M. leprae discovered in 1873 by Armauer Hansen Referred as Hansen ’ s disease Referred as Hansen ’ s disease

Epidemiology

Distribution Prevalence Prevalence Wide distribution world-wide Wide distribution world-wide Out of 122 countries, only 2 countries still have to reach the elimination goal Out of 122 countries, only 2 countries still have to reach the elimination goal Brazil and East Timor Brazil and East Timor

Leprosy status in districts March Districts with Prevalence rate less than 1 per 10, Districts with PR more than 1 per 10,000

Cases under treatment at the end of the year Year

Bacteriology

Lepra bacilli Obligate intracellular Gram positive and acid fast bacilli Obligate intracellular Gram positive and acid fast bacilli Short, thick, pink stained rods Short, thick, pink stained rods Size: 5  X 0.5  Size: 5  X 0.5  Arrangement: Single or in cigar-shaped bundles or in “ globi ” Arrangement: Single or in cigar-shaped bundles or in “ globi ” Affinity for Schwan cells & cells of R-E system Affinity for Schwan cells & cells of R-E system Cannot grow in vitro but can grow in Cannot grow in vitro but can grow in mice and mice and nine banded armadillos nine banded armadillos

The Leprosy Bacteria

Reservoir of infection Main reservoir: Human being Main reservoir: Human being Lepromatous case> Non lepromatous cases Lepromatous case> Non lepromatous cases Animal reservoirs Animal reservoirs 9-banded armadillos 9-banded armadillos Chimpanzees Chimpanzees Mangabey monkeys Mangabey monkeys

Portal of exit Major portal of exit: Nose Major portal of exit: Nose LL cases harbour millions of M. leprae in their nasal mucosa LL cases harbour millions of M. leprae in their nasal mucosa Ulcerated or broken skin of bacteriologically positive cases Ulcerated or broken skin of bacteriologically positive cases

Mode of transmission Transmission by inhalation Transmission by inhalation Droplet infection Droplet infection Transmission by contact Transmission by contact Skin to skin contact with infectious cases Skin to skin contact with infectious cases Skin contact with soil & fomites Skin contact with soil & fomites

Incubation period Long incubation period Long incubation period Ranged: 6 months-40 years or more Ranged: 6 months-40 years or more Average: 2-5 years Average: 2-5 years

Environmental factors Humidity favors survival of M. leprae in environment Humidity favors survival of M. leprae in environment M. leprae remain viable in M. leprae remain viable in Dried nasal secretions for 9 days Dried nasal secretions for 9 days Moist soil at room temperature for 46 days Moist soil at room temperature for 46 days Overcrowding & lack of ventilation within households Overcrowding & lack of ventilation within households

Social factors Often called a “social disease” Often called a “social disease” Social factors: Social factors: Poverty Poverty Poverty related circumstances Poverty related circumstances Overcrowding Overcrowding Poor housing Poor housing Lack of personal hygiene Lack of personal hygiene

CLASSIFICATION OF LEPROSY

IMPORTANCE OF CLASSIFICATION Identify the infectious cases – Epidemiological importance - Principal targets for treatment Identify the infectious cases – Epidemiological importance - Principal targets for treatment Identify the patients likely to develop the deformities and determine the prognosis Identify the patients likely to develop the deformities and determine the prognosis Frame the line of treatment Frame the line of treatment Helpful in planning and evaluation of leprosy control activities Helpful in planning and evaluation of leprosy control activities

Ridley-Jopling 1966 (Research purposes) Most widely accepted Most widely accepted Based on clinical, bacteriological, immunological and histopathological parameters, which divide the leprosy into five recognizable groups Based on clinical, bacteriological, immunological and histopathological parameters, which divide the leprosy into five recognizable groups  Exhibits a spectral disease with varied clinical characteristics due to varied host immune response to bacilli

RIDLEY-JOPLING Tuberculoid (TT) Tuberculoid (TT) Borderline Tuberculoid (BT) Borderline Tuberculoid (BT) Borderline Borderline (BB) Borderline Borderline (BB) Borderline Lepromatous (BL) Borderline Lepromatous (BL) Lepromatous (LL) Lepromatous (LL)

Indeterminate leprosy Indeterminate leprosy

Immunity in leprosy (-) (+) LLHD BLHD BBHDBTHDTTHD TT -paucibacillary state, few lesions due to high immune response LL - multibacillary state with multiple lesions due to low immune response

Contd.. Borderline forms (BB, BT and BL) lie between these two poles and are immunologically unstable, tending to move towards one of the polar forms Borderline forms (BB, BT and BL) lie between these two poles and are immunologically unstable, tending to move towards one of the polar forms

Immunology & bacteriology in leprosy (spectrum) Bacilli (-) (+) (++) (+++) (++) (+) (-) Immunity LLHD BLHDBBHDBTHDTTHD

Clinical Feature on Skin Lesion Pauci bacillary LeprosyPB Multi Bacillary Leprosy MB Including macular flat lesion, papules & nodules 1 to 5 lesion 1 to 5 lesion Asymmetrical distribution Asymmetrical distribution Definite loss of sensation Definite loss of sensation More than 5 lesion More than 5 lesion Symmetrical distribution Symmetrical distribution Loss of sensation Loss of sensation may or may not be present WHO Classification

W H O classification (For chemotherapy – M. leprae) Paucibacillary Indeterminate - I Indeterminate - I Tuberculoid – TT Tuberculoid – TT Borderline Tuberculoid – BT Borderline Tuberculoid – BT If any of these have positive bacterial index they should be classified as multibacillary for multidrug therapy If any of these have positive bacterial index they should be classified as multibacillary for multidrug therapy Multibacillary Mid borderline – BB Mid borderline – BB Borderline Lepromatous – BL Borderline Lepromatous – BL Lepromatous – LL Lepromatous – LL All smear positive cases All smear positive cases

Clinical Feature

Indeterminate Leprosy  Earliest & transitory stage  Hypopigmented macule with indistinct margins

Indeterminate Leprosy If untreated may progress towards tuberculoid, borderline or lepromatous leprosy If untreated may progress towards tuberculoid, borderline or lepromatous leprosy Spontaneous regression may occur Spontaneous regression may occur Usually negative for skin smear for AFB Usually negative for skin smear for AFB

TUBERCULOID LEPROSY Single or a few lesions Single or a few lesions Asymmetrically distributed on trunk and limbs Asymmetrically distributed on trunk and limbs Sharply defined, dry, erythematous or hypopigmented, anesthetic macules or plaques Sharply defined, dry, erythematous or hypopigmented, anesthetic macules or plaques One or two nerves may be enlarged near the skin lesion One or two nerves may be enlarged near the skin lesion SS for AFB: Negative SS for AFB: Negative Lepromin test may be strongly positive Lepromin test may be strongly positive

Tuberculoid Leprosy

Borderline Tuberculoid Single or multiple, asymmetrically distributed Single or multiple, asymmetrically distributed Macules or plaques of variable sizes with well- defined margins & satellite lesions Macules or plaques of variable sizes with well- defined margins & satellite lesions Peripheral nerves enlarged asymmetrically Peripheral nerves enlarged asymmetrically Sensation: hyposthesia Sensation: hyposthesia SS for AFB: may be seen SS for AFB: may be seen Lepromin test may be weakly positive Lepromin test may be weakly positive

Borderline tuberculoid

Borderline Borderline Multiple erythematous macules & plaques Multiple erythematous macules & plaques Various sizes and shapes with punched out centre and ill defined slopping outer margin Various sizes and shapes with punched out centre and ill defined slopping outer margin Tend to be symmetrical Tend to be symmetrical Nerves may be asymmetrically enlarged Nerves may be asymmetrically enlarged Sensation:+/- Sensation:+/- SS for AFB: seen +/- SS for AFB: seen +/- Lepromin test-usually negative, may be doubtful Lepromin test-usually negative, may be doubtful

Borderline Borderline

Borderline Lepromatous Numerous, symmetrically distributed lesions Numerous, symmetrically distributed lesions Hypopigmented or erythematous irregularly shaped maculopapules, infiltrative nodules, or plaques, with smooth surfaces & ill defined borders, sloping outwards Hypopigmented or erythematous irregularly shaped maculopapules, infiltrative nodules, or plaques, with smooth surfaces & ill defined borders, sloping outwards Nerves may be symmetrically or asymmetrically enlarged Nerves may be symmetrically or asymmetrically enlarged Sensation:+/- Sensation:+/- SS for AFB: numerous seen SS for AFB: numerous seen Lepromin test -negative Lepromin test -negative

Borderline Lepromatous

Lepromatous Leprosy Numerous macules, plaques, nodules or diffusely infiltrated lesions, symmetrically distributed on face, trunk and extremities with ill-defined margin which may be slightly hypopigmented or erythematous Numerous macules, plaques, nodules or diffusely infiltrated lesions, symmetrically distributed on face, trunk and extremities with ill-defined margin which may be slightly hypopigmented or erythematous Symmetrical nerve enlargement is seen Symmetrical nerve enlargement is seen Sensation: normal Sensation: normal SS for AFB: numerous seen SS for AFB: numerous seen Lepromin test - negative Lepromin test - negative

Lepromatous Leprosy

Ear lobes thickens

. diffuse thickening of the skin, with loss of hair (eyebrows and eyelashes). saddle nose deformity saddle nose deformity leonine facies leonine facies

General Findings Eye The anterior chamber can be invaded in LL with resultant glaucoma and cataract formation. Iritis/Iridocyclitis Testes May be involved in LL with resultant hypogonadism. Systemic involvement – Respiratory, Bones, Kidneys, Lymph glands, etc.

Nerve involvement in Leprosy

M. Leprae : superficial nerve involvement W Britton

Nerve Involvement Neural involvement leads to muscle weakness, muscle atrophy, severe neuritic pain, and contractures of the hands and feet. Ulnar nerve commonly involved Examination for sensations of hot and cold, pain and fine touch

Nerve palpation

Face Facial Nerve  Lagophthalmos  Facial droop Trigeminal Nerve  Corneal anaesthesia

Nerve damage – upper limb

UlnarS  Anaesthesia medial 1/3 palm M  Claw ring and little fingers A  Dryness medial 1/3 palm

Median S  Anaesthesia lateral 2/3 palm M  Claw mid + index + loss Opposition M  Claw mid + index + loss Opposition A  Dryness lateral 2/3 palm A  Dryness lateral 2/3 palm

RadialS  Anaesthesia dorsum hand M  Wrist drop

Nerve damage – lower limb Lateral (common) Popliteal  Foot drop

Posterior Tibial S  Sole anaesthesia M  Claw Toes A  Dryness in sole