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LEPROSY

LEPROSY. EPIDEMIOLOGY. 8 MILLION PATIENTS 60,000 NEW CASES EACH YEAR WORLD WIDE LEPROSY IS ENDEMIC IN ASIA & AFRICA APPOX. 80% CASES IN THESE AREAS IN THE WORLD. Global Leprosy Situation in 2005. WHO Region Point Prevalence

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LEPROSY

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  1. LEPROSY

  2. EPIDEMIOLOGY • 8 MILLION PATIENTS • 60,000 NEW CASES EACH YEAR WORLD WIDE • LEPROSY IS ENDEMIC IN ASIA & AFRICA • APPOX. 80% CASES IN THESE AREAS IN THE WORLD

  3. Global Leprosy Situation in 2005 • WHO Region Point Prevalence • Africa 47 596 • Americas 36 977 • East Mediterranean 5 398 • South East Asia 186 182 • Western Pacific 10 010 • World 286 063

  4. PERVALENCE OF LEPROSY INSEA REGION WHO 2003

  5. DEFINITION • LEPROSY IS A NONFATAL ,CHRONIC INFECTIOUS DISEASE CAUSED BY MYCOBACTRIUM LEPRAE (By G.A.Hansen 1873) • INVOLVING - • SKIN • PERIPHERAL NERVOUS SYSTEM • UPPER RESPIRATORY TRACT • EYES • TESTES • M.LEPRAE HAS UNIQE TROPISM FOR PEIPHRAL NERVES • REACTIONAL STATE RESPONSIBLE FOR MORBIDITY & • DISEASE IF NOT TREATED LEADS TO CHARECTERSTIC DEFORMITY & PROFOUND SOCIAL STIGMA

  6. DEFORMITY

  7. M. LEPRAE • NONCULTIVABLE IN MEDIUM • FACULTATIVE OBLIAGTE INTRACELLULAR ORGANISM • GRAM-POSITIVE • ACID-FAST BACILUS • PEPTIDOGLYCAN-BACK BONE • ARABINOGALACTAN & MYCOLIC ACID • PHENOLIC GLYCOLIPD-1 (PGL-1) ATTACHED TO LAMININ 2 OF SCHWANN CELLS • MHC CLASS II FOR DISEASE EXPRESSION NOT FOR SUSCEPTIBILITY OF DISEASE • SUSCEPTIBILTY GENE LOCUS ON CHROMOSOME-10P13

  8. The route of transmission • Not been definitively established, • Although human-to-human aerosol spread of nasal secretions is the most likely mode of transmission in most cases. • The disease is not spread by touch, since the mycobacteria are incapable of crossing intact skin. • Living near people with leprosy is associated with increased transmission. Among household contacts, the relative risk for leprosy is increased 8- to 10-fold in multibacillary and 2- to 4-fold in paucibacillary forms. • Animal reservoirs do exist (armadillos, certain nonhuman primates), and cases of suspected zoonotic transmission have been reported.

  9. Two indices which depend on observation of M. leprae in smears from skin or nasal smears are useful in assessing • the amount of infection, • the viability of the organisms • the progress of the patient under treatment. They are - the morphological index (MI) the bacteriological index (BI).

  10. 1. The bacteriological index (BI) • This is an expression of the extent of bacterial loads. It is calculated by counting six to eight stained smears under the 100 x oil immersion lens. • A smear stained by the Ziehl-Neelsen method and decolorized (but not completely) which 1% acid alcohol. • The results are expressed on a logarithmic scale.

  11. BACTERIOLOGICAL INDEX • LOGAITHMIC SCALE AS TO NUMBER OF BACILLI PER OIL IMMERSION FIELD(OIF) • BI OF 6 IS 1000 OR MORE BACILLI/OIF • BI OF 5 IS 100 TO 1000/OIF • BI OF 4 IS 10 TO 100 BACILLI/OIF • BI OF 3 IS 1 TO 10 BACILLI/OIF • BI OF 2 IS 1 BACILLUS/1 TO 10 OIFS • BI OF 1 IS 1 BACILLUS/ 10 TO 100 OIFS • BI OF 0 IS NO BACILLUS PER 100 OIFS

  12. MORPHOLOGICAL INDEX PERCENTAGE OF SOLIDLY STAINED BACILLI IN STAINED SMEAR • Only the solid-staining bacilli are viable. • It is not unusual for solid-staining M. leprae to reappear for short periods in patients being successfully treated with drugs. • It is important to recognize that measurement of MI is liable for observer variations and therefore not always reliable.

  13. IS LEPROSY HOST IMMUNE DEPENDENT DISEASE? YES…. CMI OR ANTIBODY MEDIATED ?

  14. CLINICAL PRESENTATION • LEPROSY HAS SPECTRUM OF DISEASE • Ridley-Jopling classification system • TT BT BB BL LL • INDICIES FOR SPECTRUM • BACTERIOLOGICAL • IMMUNOLOGICAL • CLINICAL • HISTOPATHOLOGICAL

  15. WHO Classification system: • The WHO recommends classifying leprosy according to the number of lesions and the presence of bacilli on a skin smear. . • Paucibacillary (PB) leprosy is characterized by 5 or fewer lesions with absence of organisms on smear. • Includes the tuberculoid and borderline tuberculoid leprosy categories from the Ridley-Jopling system. • Multibacillary (MB) leprosy is marked by 6 or more lesions with possible visualization of bacilli on smear. • Includes Lepromatous, borderline lepromatous, and midborderline on the Ridley-Jopling scale .

  16. The cardinal signs of leprosy • Hypoesthesia, • skin lesions, • loss of hair and sweating & peripheral neuropathy. • The first physical signs of leprosy are usually cutaneous. • The subtype of leprosy often determines the degree of skin involvement.

  17. Physical examination • Evaluation of skin lesions • Careful sensory and motor examination • Palpation of peripheral nerves for pain or enlargement. Particular attention should be paid to the following locations: • Elbows - Ulnar nerve • Wrist - Superficial radial cutaneous and median nerves • Popliteal fossa - Common peroneal nerve • Neck - Great auricular nerve

  18. TUBERCULOID LEPROSY • Single or few Lesions • Erythematous plaques • Hypopigmanted • Hypoasthatic • Macular lesions • Cutaneous nerve • thickened

  19. TUBERCULOID LEPROSY

  20. TUBERCULOID LEPROSY

  21. TUBERCULOID LEPROSY

  22. BORDERLINE LEPROSY

  23. BORDERLINE LEPROSY

  24. POSTERIOR AURICULAR NERVE THICKNING

  25. LEPROMATOUS LEPROSY

  26. LEPROMATOUS LEPROSY

  27. LEPROMATOUS LEPROSY

  28. POSTERIOR AURICULR NERVE

  29. NODULR LESION AT PINNA

  30. NODULAR LESION AT PINNA

  31. DIFFUSE INFILTRATIONINLEPROMATOUS LEPROSY

  32. DIFFUSE INFILTRATIONINLEPROMATOUS LEPROSY

  33. BORDERLINE LEPROMATOUS LEPROSY

  34. LEFT ULNER NERVE PALSY

  35. ANESTHATIC HAND

  36. SKIN SMEAR FROM LESION SHOWS AFB++

  37. HISTPATHOLOGY

  38. Immunologic tests • Lepromin skin test • Not diagnostic of exposure or infection with M leprae • Assesses a patient's ability to mount a granulomatous response against a skin injection of killed M leprae. • Patients with tuberculoid or borderline lepromatous leprosy typically have a positive response (>5 mm). • Patients with lepromatous leprosy typically have no response.

  39. Detection of antibodies to phenolic glycolipid-1 (PGL-1) This is a specific serologic test. • This test has a sensitivity of 95% for the detection of lepromatous disease but only 30% for tuberculoid disease. PCR and recombinant DNA technology development of gene probes with M leprae–specific sequences. This technology can be used to identify the mycobacterium in biopsy samples, skin and nasal smears, and blood and tissue sections. • Lymphocyte migration inhibition test (LMIT): As determined by a lymphocyte transformation and LMIT, • cell-mediated immunity to M leprae is absent in the lepromatous form of disease but present in the tuberculoid form of disease. • Contact or family screening for history of leprosy

  40. REACTIONIONAL SATES IN LEPROSY

  41. TYPE -1 REACTION OCCURS IN BORDERLINE CASES NOT IN POLAR LEPROSY • ACTIVATION OF PREVIOUSLY INVOLVED SKIN LESIONS • PANFUL & TENDER NERVES • DOWNGREADING REACTION - WHEN OCCURS BEFORE INITIATON OF CHEMOTHERAPY • REVERSAL REACTION AFTER INITIATION OF CHEMOTHERAPY- TH1 RESPONSES WITH IFN-GAMMA,IL2 • CORTICOSTEROID TREATMENT OF CHOICE

  42. TYPE -1 REACTION

  43. TYPE -2 REACTION • OCCURS IN BL/LL FORM OF LEPROSY • IN 50% OF CASES • IN 90% OF CASES MAY BE PRESENTING SYMPTOM OF DISEASE • ENL- ERYTHEMA NODOSUM LEPROSUM • FEVER • ARTHRITIS • UVEITIS • ORCHITIS • GLOMERULONEPHRITIS • TNF- PLAYS CETERAL ROLE, • IMMUNE COMPLEX DEPOSITION • TH2 CYTOKINE PROFIL-IL6,IL8 • THALIDOMIDE IS CHOICE OF TREATMENT

  44. ERYTHEMA NODOSUM LEPROSUM

  45. Erythema Nodosum

  46. TREATMENT-MDT • MULTI • DRUG • THERAPY

  47. What is WHO MDT? • Multi drug therapy (MDT) is a key element of the elimination strategy. • MDT is available free of charge from WHO • The drugs used in WHO-MDT are a combination of Rifampicin, • clofazimine and Dapsone for MB leprosy patients and • Rifampicin and Dapsone for PB leprosy patients. • Treatment of leprosy with only one anti leprosy drug will always result in development of drug resistance. • Treatment with Dapsone or any other anti leprosy drug used as monotherapy should be considered as unethical practice.

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