Hansen’s Disease

1. Hansen’s Disease

2. History • Definition: Leprosy is a chronic systemic disease caused by Mycobacterium lepraemanifesting 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, ‘Kushtharoga’

3. 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

4. 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

5. Immunity and Leprosy Clinical manifestation No infection Subclinical infection with spontaneous regression Indeterminate, pure neuritic, tuberculoid Mid-borderline, borderline-lepromatous Lepromatous Host resistance • Excellent • Good • Fair • Poor • Very poor

6. 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.

7. Classification The Ridley Jopling classification • Indeterminate • Tuberculoid • Borderline: borderline-tuberculoid, mid-borderline, borderline-lepromatous • Lepromatous Pure neural, Maculoanaesthetic - Indian classification

8. 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.

9. 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.

10. 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.

11. Borderline Tuberculoid (BT) • Few asymmetric, hypopigmented or skin colouredmacules, plaques with ill defined margins • Presence of satellite lesion near the advancing margin of patch • Sensory impairment - Marked • Nerve involvement - Marked and asymmetrical

12. 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.

13. 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

14. 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

15. 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.

16. 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.

17. 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.

18. 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

19. 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

20. 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.

21. Other features • Nasal stuffiness / crusting • Epistaxis • Hoarseness of voice • Gynaecomastia • Saddle nose • Bone resorption • Lymphadenopathy

22. Differential diagnosis of leprosy Macular lesions • Vitiligo • Occupational leucoderma • Tineaversicolor • Pityriasis alba • Post kalaazar dermal leishmaniasis • Naevusdepigmentosus • Scars

23. Differential diagnosis of leprosy Infiltrated lesions • Lupus vulgaris • Lupus erythematosus • Granulomaannulare • Annular syphilides • Post kalaazar dermal leismaniasis (infiltrated lesions) • Sarcoidosis • Psoriasis

24. Differential diagnosis of leprosy Nodular lesions • Post kalaazar dermal leismaniasis • Cutaneousleismaniasis • Syphilis • Onchocerciasis • Sarcoidosis • Leukaemia cutis • Mycosis Fungoides • Nodules of neruofibromatosis

25. 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 • Tabesdorsalis • Thoracic outlet syndrome • Alcoholic neuropathy

26. 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

27. Clinical examination • Type and number of skin lesions • Sensory impairment • Motor examination • Nerve examination • Sweating • Loss of hair

28. 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

29. 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.

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

31. 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- Indicates density of leprosy bacilli (live & dead) in the smears and ranges from 0 to 6+ Morphological index- It is the percentage of presumably living bacilli in relation to total number of bacilli in the smear

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

33. 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

34. 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

35. 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

36. 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

37. Newer Drugs / Regimens • RO - 28 • Fluoroquinlones - Nalidixic acid • Macrolides (Clarithromycin) • Ansamycin-Rifabutin, Rifapentine • Dihydrofolatereductase inhibitors-Brodimoprim, K-130 • Fusidic acid • Beta-lactam antibiotics • Cephalosporins • Quinolones (Pefloxacin and Sparfloxacin

38. 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

39. 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

40. Precipitating factors • Physiological conditions like pregnancy • Drugs: anti-leprosy drugs, iodides • Severe physical or mental stress • Infections

41. 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

42. 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

43. Type II Reaction • Occurs in BL and LL cases • Type III hypersensitivity reaction • ErythemaNodosumLeprosum-crops of painful, recurrent, erythematous, papulonodular lesions. • Fever and malaise • Iridocyclitis, episcleritis, epididymo-orchitis, arthritis, neuritis, lymphadenitis

44. 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

45. 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

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

47. 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.

48. 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

49. Primary deformities • Leonine Facies • Loss of eyebrows and eyelashes • Depressed nose • Gynaecomastia • Palatal Perforation

50. Secondary deformities • Corneal ulcers and opacities • Plantar ulcers • Palmar ulcers and ulcers on tips of fingers • Resorption • Charcot joints