Hansen’s Disease

History

 Definition: Leprosy is a chronic systemic disease caused by

Mycobacterium leprae

manifesting as development of specific granulomatous or neurotrophic lesions in the skin, mucous membrane, eyes nerves, bones and viscera.

 Oldest infection known to mankind  Synonyms: Hansen’s disease, ‘Kushtha roga’

Transmission of Leprosy

 Respiratory route: inhalation of bacilli-laden droplets  Cutaneous: skin to skin contact   GIT : ingestion of food Intradermal : inoculation by tattoos } Not Yet Proven

Epidemiological factors

 Occurs at all age groups  Peak age of onset : Between 10 – 20 years  Males > Females  Children most susceptible  Immune status ( host resistance)  Overcrowding  Low socioeconomic status

Immunity and Leprosy

Host resistance  Excellent  Good  Fair  Poor  Very poor Clinical manifestation No infection Subclinical infection with spontaneous regression Indeterminate, pure neuritic, tuberculoid Mid-borderline, borderline-lepromatous Lepromatous

Mycobacterium Leprae

 Obligate, intracellular, acid-fast bacillus  Affinity for skin, nerves and muscle tissue  Found in macrophages, histiocytes and Schwann cells.

  Non cultivable Grown in animal models  Closely resembles

M.tuberculosis

, but less acid-fast.

 Multiplies in 11-13 days.

Classification

The Ridley Jopling classification  Indeterminate  Tuberculoid  Borderline: borderline-tuberculoid, mid-borderline, borderline-lepromatous  Lepromatous Pure neural, Maculoanaesthetic - Indian classification

Classification

 Paucibacillary Leprosy(PB): Indeterminate leprosy (I) Tuberculoid leprosy (TT) Borderline tuberculoid (BT)  Pure neuritic (PN)* Multibacillary Leprosy(MB): Midborderline leprosy (BB) Borderline lepromatous (BL) Lepromatous leprosy (LL) * Asymmetric nerve involvement with no skin lesion and usually of tuberculoid origin.

Tuberculoid (TT)

 Single or few, asymmetrical, well-defined, erythematous or copper-coloured patches  Sensations - Absent  Nerves - thickened, presence of feeding nerves, abscesses  Skin smears - Negative  Lepromin test - Strongly positive  Course - Relative benign and stable, with good prognosis.

Borderline leprosy

 Common type of leprosy Subdivided into BT, BB & BL.

 Course - Unstable with variable prognosis , may progress to sub-polar LL leprosy.   Most prone to reactions.

Lepromin test -Negative ,weakly positive in BT.

Borderline Tuberculoid (BT)

 Few asymmetric, hypopigmented or skin coloured macules, plaques with ill defined margins  Presence of satellite lesion near the advancing margin of patch  Sensory impairment - Marked  Nerve involvement - Marked and asymmetrical

Midborderline leprosy (BB)

 Unstable form, reactions frequent  Annular lesions with characteristic punched out appearance (inverted saucer shaped)   Sensory impairment - Moderate.

Nerve involvement - Marked and asymmetrical.

Borderline lepromatous leprosy (BL)

 Multiple shiny macules, papules, nodules and plaques with sloping edges  Sensory impairment - Slight  Nerve involvement - Widespread and less asymmetrical.

 Glove & stocking hypoaesthesia

Lepromatous leprosy

 Hypopigmented, erythematous or coppery, shiny macules, papules, nodules  Lesions symmetrically distributed, small, multiple, shiny with normal or mild sensory loss  Leonine facies: Infiltration of skin with nodules, loss of eyebrows and eyelashes  Nerve involvement symmetrical; glove & stocking anaesthesia  Lepromin test - Negative

Indeterminate leprosy

 Asymmetrical, single /multiple hypopigmented, or faintly erythematous and ill-defined macules.

 Sensation - Normal or slightly impaired  Peripheral nerves - Normal  Skin smears - Negative  Lepromin test - Unpredictable and variable  Course - Usually self limiting ,may progress to other forms of leprosy.

Pure Neuritic leprosy

 Neuritic manifestations -Tingling, heaviness and numbness, paresis, hypotonia, atrophy, claw hand and toes, wrist-drop, foot-drop. No skin lesion.

 Other changes-Anhidrotic, dry glossy skin, blisters, neuropathic ulcers, decalcification, bone resorption.

Pure Neuritic leprosy

 Lepromin test -Slightly positive.

 Course-Spontaneous regression or progression to TT leprosy.

 Silent neuritis (silent neuropathy) Sensory or motor impairment without skin signs of reversal reaction or ENL ,tenderness, paraesthesiae or numbness.

Special forms of Leprosy

 Lucio Leprosy: Rare form of lepromatous leprosy, described in Mexico. Diffuse widespread infiltration of skin, loss of body hair, loss of eyebrows & eyelashes, and widespread sensory loss.

 ‘Lepra Bonita’ (Pretty leprosy) Elderly persons with diffuse infiltration of face smoothes out wrinkles, giving youthful appearance.  Histoid leprosy: BL patients with irregular or poor treatment compliance Drug resistant cases

Eye Involvement in Leprosy

 Lagophthalmos (partial/complete, unilateral/bilateral)  Conjunctivitis  Exposure keratitis and corneal ulcers  Madarosis, trichiasis leading to corneal vascularity and opacity  Dacryocystitis (acute,subacute or chronic)  Nodules on sclera, episcleritis, scleritis  Corneal nodules and lepromatous pearls  Microlepromata, nodules on iris and ciliary body

Nerve Involvement in Leprosy

 Sensory involvement - Anaesthesia in hands & feet, glove and stocking anaesthesia, repeated trauma  Motor involvement- Wasting and paralysis of muscles  Autonomic involvement -Icthyosis, loss of hair and sweating.

Other features

 Nasal stuffiness / crusting  Epistaxis  Hoarseness of voice  Gynaecomastia  Saddle nose  Bone resorption  Lymphadenopathy

Differential diagnosis of leprosy

Macular lesions  Vitiligo       Occupational leucoderma Tinea versicolor Pityriasis alba Post kala azar dermal leishmaniasis Naevus depigmentosus Scars

Differential diagnosis of leprosy

Infiltrated lesions  Lupus vulgaris  Lupus erythematosus  Granuloma annulare  Annular syphilides  Post kala azar dermal leismaniasis (infiltrated lesions)  Sarcoidosis  Psoriasis

Differential diagnosis of leprosy

Nodular lesions  Post kala azar dermal leismaniasis  Cutaneous leismaniasis  Syphilis  Onchocerciasis  Sarcoidosis  Leukaemia cutis  Mycosis Fungoides  Nodules of neruofibromatosis

Differential diagnosis of neurological conditions

Sensory impairment with or without muscle wasting  Peripheral neuropathy  Diabetic neuropathy  Primary amyloidosis of peripheral nerves   Congential sensory neuropathy Syringomyelia   Tabes dorsalis Thoracic outlet syndrome  Alcoholic neuropathy

Diagnosis

Cardinal signs of leprosy  Sensory impairment in affected areas  Enlargement of peripheral nerves associated with signs of peripheral nerve damage  Finding acid-fast bacilli in the lesions

Clinical examination

 Type and number of skin lesions  Sensory impairment  Motor examination  Nerve examination  Sweating  Loss of hair

Clinical examination: Sensory

 Touch Tested with wisp of cotton,nylon thread or feather.

 Temperature Tested with two test tubes – one containing hot water and other cold  Pain Tested by pin prick

Clinical examination : Motor

 Testing of motor power- Done clinically  Electro-diagnosis - Employed in very early cases. Electrical stimulator using faradic and galvanic current used to test muscle power.

Nerves

 Supra/ infraorbital  Greater auricular  Clavicular  Radial  Sup. Radial cut  Ulnar   Median Lateral popliteal  Posterior tibial  Anterior tibial  Sural

Investigations for M. Leprae

Bacteriological examination Skin smears: Made by slit and scrape method from the most active looking edge of skin lesion and stained with Ziehl-Neelsen method.

Reading of smears:

Bacteriological index-

bacilli (live & dead) in the smears and ranges from 0 to 6+ Indicates density of leprosy

Morphological index-

presumably living bacilli in relation to total number of bacilli in the smear It is the percentage of

Investigations

 Histopathological examination  Nerve biopsy  Sweat function test  Lepromin test  Animal Models: Armadillo, Thymectomised, irradiated nude mice, Korean chipmunk etc.

Newer Investigations

 Serological assays: FLA-ABS, RIA, ELISA  PGL, PCR  Other techniques: Chemical, Immunological, Molecular biological, Bioluminescent techniques, Strain specific probes Indications: - To confirm diagnosis in c/o inconclusive histopathological/smear reports.

- To distinguish between reaction and relapse - To demonstrate M. leprae or its components - To elicit strain differentiation for molecular epidemiology - To detect drug susceptibility or resistance

MDT-WHO

 Paucibacillary leprosy (6 months) - Cap. Rifampicin (600 mg) monthly, supervised - Tab. Dapsone (100 mg) daily  Multibacillary leprosy (1 year) - Cap. Rifampicin (600mg) monthly, supervised - Cap. Clofazimine (300mg) monthly, supervised - Tab. Dapsone (100mg) daily - Cap. Clofazimine (50mg) daily

Blister packets for MDT

 Easy to use, handy and of convenient size  Provide complete treatment  Improve clinical attendance  Drugs are better protected against moisture,heat and accidental damage  Ensures quicker dispensing of the drugs  Can be dispensed by non medical person

Other Regimens

 ROM Comprises Rifampicin - 600 mgs, Ofloxacin - 400 mgs, Minocycline - 100mg Single dose – single patch (WHO accepted) ROM -6 (Monthly for 6 months) - Paucibacillary ROM -12 (Monthly for 12 months) – Multibacillary

Newer Drugs / Regimens

 RO - 28  Fluoroquinlones - Nalidixic acid  Macrolides (Clarithromycin)  Ansamycin-Rifabutin, Rifapentine  Dihydrofolate reductase inhibitors-Brodimoprim, K-130  Fusidic acid  Beta-lactam antibiotics  Cephalosporins  Quinolones (Pefloxacin and Sparfloxacin

Immunomodulatory Drugs

 Drugs- Levamisole, Zinc  Antigenically related mycobacteria- B.C.G vaccine, M.leprae +B.C.G vaccine, Mycobacterium welchii vaccine, ICRC vaccine.

 Other immunomodulators-Gamma interferons,interleukin

Lepra Reactions

 Acute episodes or bouts of exacerbations occurring in course of chronic disease  Sudden increase in activity of existing lesions, appearance of fresh lesions with or without constitutional symptoms  Type I reaction - all borderline cases (BT, BB,BL)  Type II reaction - BL & LL cases

Precipitating factors

 Physiological conditions like pregnancy  Drugs: anti-leprosy drugs, iodides  Severe physical or mental stress  Infections

Type I Reaction

 Sub-types - Upgrading (Reversal) - Downgrading  Type IV hypersensitivity reaction.

 Existing lesions worsen/New lesions may appear  Neuritis / Nerve abscesses  Systemic disturbances: Unusual

Type I reaction - complications

 Neuritis  Dactylitis, edema of hands & feet, inflammation of small joints of fingers  Corneal anesthesia, Conjunctivitis  Sudden occurrence of claw hand, foot-drop, facial palsy

Type II Reaction

 Occurs in BL and LL cases  Type III hypersensitivity reaction  Erythema Nodosum Leprosum-crops of painful, recurrent, erythematous, papulonodular lesions.

 Fever and malaise  Iridocyclitis, episcleritis, epididymo-orchitis, arthritis, neuritis, lymphadenitis

Type II Reaction - complications

 Frozen hand  Laryngitis  Non-paralytic deformity  Polyarthritis/ RA-like syndrome  Multiple dactylitis  Leucocytosis, Anaemia, raised ESR  Albuminuria/ nephrotic syndrome  Liver/spleen enlargement  Epididimytis/ orchitis, Testicular atrophy/sterility  Gynaecomastia  Adrenal gland hypofunction  Eye involvement

Treatment of Lepra reactions

Principles of treatment  Early initiation of treatment for reaction  Continuation / initiation of MDT  Removal of precipitating factor  Rest, physical and mental

Treatment modalities

 Analgesics  Corticosteroids  Antimalarials  Clofazimine  Thalidomide  Miscellaneous – colchicine, zinc, cetrizine, antimonials  Supportive management – for eye complications, splints etc.

Deformities in leprosy

 Primary: Are caused by the tissue reaction to infection with M.Lepra e.g. leonine facies, flat-nose, claw hand.

 Secondary: Occur as a result of damage to the anesthetic parts of the body e.g. planter ulcers, corneal ulcers.

Grading of Deformities/Disabilities: WHO Classification

 Grade 0 No anaesthesia, no visible deformity or damage in hands and feet, or no problems in eye or no visual loss  Grade 1 Anaesthesia present, but no visible deformity or damage, eye problems present but vision 6/60 or better.

 Grade 2 Visible deformity or damage present in hands or feet , and vision worse than 6/60

Primary deformities

 Leonine Facies  Loss of eyebrows and eyelashes  Depressed nose  Gynaecomastia  Palatal Perforation

Secondary deformities

 Corneal ulcers and opacities  Plantar ulcers  Palmar ulcers and ulcers on tips of fingers  Resorption  Charcot joints

Deformities: Nerve damage

 Claw hand (ulnar, median)  Clawing of the toes (posterior tibial)  Wrist-drop (radial)  Foot-drop (lateral popliteal)  Lagophthalmos, facial palsy (facial)

Trophic ulcer: stages

 Threatened ulcer- slight puffiness and warmth in region of metatarsal head with associated tenderness  Concealed ulcer - Necrosis, blisters at the site of damage.

 Open ulceration - Frank ulcer  Types of ulcers - Acute ulcer Chronic ulcer Complicated ulcer

Prevention of deformities

 Early detection of nerve damage  Adequate treatment of leprosy patient  Use of protective footwear  Adequate hydration of skin  Physiotherapy

Management of deformities

 Education of patient regarding prevention of injuries  Daily examination of hands and feet and prompt treatment for minor injuries  Using adapted tools and appliances after training  Reconstructive surgery  Rehabilitation

Physiotherapy

Oil massage/Wax Therapy-Uses  To make the skin soft and supple and loosen stiff joints  As a preliminary to exercises  To strengthen muscles and keep joints mobile  To reduce pain in acute neuritis  To stimulate innervated sweat glands to increase blood flow

Physiotherapy

Splints-Indication  Acute neuritis  Mobile deformities(to prevent fixed deformities),  Fixed deformities(to correct the deformities). Splints used  For radial neuritis-Static or dynamic wrist drop splint  For mobile deformity- Static or dynamic splint  For fixed deformity-Gutter splints,finger loops etc.

Physiotherapy

Electric stimulation - Uses  To maintain the tone of denervated muscle  Helpful in breaking post operative adhesions  After tendon surgery could be used as a means of documenting nerve damage and the progress of the nerve recovery with treatment.

Prevention and Control of leprosy

 Prevention of leprosy Early detection through survey and initiation treatment Families of patients to be kept under surveillance Immunoprophylaxis -Use of leprosy vaccines Improvement in socio-economic conditions  Control of leprosy Three activities of a leprosy control unit Case detection Case holding, including treatment Health education of public and patients

Prevention and Control of leprosy

 Leprosy Organizations UNICEF LEPRA , DANIDA, SIDA ,CIDA ,Leprosy mission, American leprosy mission, German leprosy relief association  Leprosy control Programmes National leprosy control programme (NLCP)1954 Triad of survey, education and treatment (S.E.T).

National leprosy eradication programme (NLEP)1982

Prevention and Control of leprosy

National Leprosy Eradication Programme (NLEP),1982  Eradicate leprosy from the country by 2000  ‘Vertical’ health programme- In areas where prevalence of leprosy is more than 5 per 1000.

 ‘Horizontal’ programme- In areas where the prevalence rate is less than 5 per 1000

NLEP

Three main units for programme operation:  Basic tier- Survey, education and treatment unit, leprosy control unit and urban leprosy control unit.

 Second tier-District/zonal leprosy office  Third tier-Leprosy division of the state directorate of the health services.

Rehabilitation in leprosy

 Rehabilitation: Physical and mental restoration of patients to normal activities, so that they are able to assume their place in the home, society and industry.

 Treatment of physical disability  Education of patient, family and public  Rehabilitation in special homes or institutional rehabilitation  Community based rehabilitation

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