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Leprosy. Dr. Syed Waleem Pasha. Introduction. Chronic granulomatous disease Skin and nerves Mycobacterium leprae Disease depends on degree of cell mediated immunity expressed by the individual towards M. leprae Nerve damage – considerable morbidity Stigmatised - leper. Organism.
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Leprosy Dr. SyedWaleem Pasha
Introduction • Chronic granulomatous disease • Skin and nerves • Mycobacterium leprae • Disease depends on degree of cell mediated immunity expressed by the individual towards M. leprae • Nerve damage – considerable morbidity • Stigmatised - leper
Organism • Mycobacterium leprae (M. leprae) • Cannot be grown in vitro • Doubling time is 12 days • Withstand drying for upto 5 months • Differs from M. tuberculosis by only 29 functional genes
Epidemiology • 4 million people – disabled by leprosy • 750000 new cases annually • Most cases – children • 70% cases in India • Male>female • HIV is not a risk factor for leprosy • Transmission • Untreated lepromatous pt discharge bacilli from nose • Infection acquired through nose (airborne) • Hematogenous spread to skin and nerves
Pathogenesis • Incubation period • Tuberculoid cases: 2-5 years • Lepromatous cases: 5-10 years • Predilection to Schwann cells and skin macrophages • Three aspects of pathogenesis • Immune responses • Nerve damage • Immune mediated reaction • Ridley – Jopling spectrum of responses
Pathogenesis • Tuberculoid Pts • Th1 -type response • IL 2 and IFN g • Positive lepromin test • Clears bacteria • Local tissue destruction • Granuloma formation • Lepromatous Pts • T cell and macrophage anergy • Th2 –type response • Poor reponse • Negative lepromin test
Clinical features • Skin lesion • Effects of peripheral nerve damage • Lepra reaction
Clinical features • Skin • Macules or papules • Anesthesia + loss of sweating • Lepromatous • Papules / nodules • Numerous • Diffuse infilteration of skin • Tuberculoid • Few • Hypopigmentedmacules
Clinical features • Peripheral neuropathy • Sites of predilection • Upper limbs: Ulnar, Median, Radial, Radial cutaneous • Lower limbs: Common peroneal, Posterior tibial, Sural • Head and Neck: Facial, Greater auricular • Characteristic signs • Regional sensory loss • Dysfunction of muscles • Thickened
Clinical features • Secondary complications due to nerve damage • Eye involvement: • Corneal ulceration due to trauma • Damage to facial and trigeminal nerve
Pure neural leprosy • Only in India • No skin lesions • Asymmetrical involvement of peripheral nerves
Investigations • Diagnosis is clinical • Slit skin smears/ skin biopsy • Acid fast bacilli • Histology on biopsy • Bacterial index
Differential Diagnosis • Skin • Pityriasisversicolor • Vitiligo • Post inflammatory depigmentation • Psoriasis • Vitiligo • Nerves • Thickeneing • Charcot-Marie-Tooth disease • Amyloidosis • Polyneuropathy • HIV, alcohol, diabetes, vaculitis, heavy metal poisoning
Treatment - principles • Stop the infection by chemotherapy • Treat reactions • Educate the Pt about leprosy • Prevent disability • Social and psychological support
Chemotherapy • Multi drug combinations • Paucibacillary • 2-5 skin lesions • Tuberculoid (TT) • Slit skin smear negative BT • Multibacillary • >5 skin lesions • Skin smear positive BT • All BB, BL and LL
First line drugs: • Rifampicin • Dapsone • Clofazimine • Second line drugs: • Pefloxacin, ofloxacin • Minocycline • Clarithromycin
Treatment of lepra reactions • Type 1 • steroids • Type 2 • High doses steroids • Thalidomide • Chloroquine