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• بسم هللا الرحمن الرحيم Leprosy (HANSEN'S DISEASE): It is an ancient stigmatizing disease of mainly: 1-Skin 2-Superficial peripheral nerves 3-Nose 4-Eyes 5-Testes It is a notorious cause of the followings:1\Blindness 2\Nasal deformities. 3\Hand deformities. 4\Feet deformities. 5\Nerve deformities. • WHO classification 1) Multi-bacillary (lepromatous) leprosy. 2) Pauci-bacillary (tuberculoid) leprosy • Source of infection 1-Lepromatous patients are main source of the infection. 2-Tubercloid patients are less infectious. Mode of transmission : 1. Infectious nasal discharge or droplet. 2. Intimate skin contact. Host factors: Over 90% of individuals resist leprosy successfully and this may suggest the possible genetic factor: -HLA link • SUSCEPTIBILITY TO INFECTION 1\RACE 2\SEX 3\AGE • GEOGRAPHIC DISTRIBUTION • REGISTERED CASES IN THE WORLD IN 2003 Around 13 million diagnosed and treated in the period from 1985-2003. From the total of about 122 countries about 112 countries got rid of leprosy. • REGISTERED CASES IN SUDAN BY THE END OF 2003 The registered cases where about 999. 906 New cases discovered. 22% of the cases with marked degree of deformity. Children under 15 years represented about 2%. MB : PB 3:1 • TREND IN CASE DETECTION 91-2003 • ASMA 2 • Mycobacterium leprae M.leprae is a rod-like bacillus measuring 2-7 µm in length and 0.3-0.4 µm in width. It was first discovered in 1873, in Norway, by • Armauer Hansen. The organism is sometimes known, therefore, as “Hansen’s bacillus”. It is Acid-Fast and can only be seen under the microscope if it is stained and that is done using the “modified ZeihlNeelsen” Staining Method. M.leprae still cannot be cultivated artificially in laboratory media and this has caused much frustration and delay in finding an effective anti-leprosy vaccine. • In a limited way, it has been made to grow in the foot pads of mice and also in the 9-banded Armadillo. • These techniques have been helpful in testing for drugresistance (Sulphone Drugs) of the organisms • • According to the clinical, pathological and immunological picture presented: • Stable(LL &TL). • Unstable (BL). • This is determined by: • CMI. • Genetic make up • CMI AND THE CLINICAL PICTURE • WHO CLASSIFICATION MULTIBACILLARY PAUCIBACILLARY Routes of infection: Respiratory tract. Abrasion on skin. Gastrointestinal tract. Mechanical transmission . Other uncommon methods : sexual intercourse _blood transfusion_ vaccination. • LEPROMATOUS LEPROSY LL skin ,peripheral nerves, organ systems CMI. humeral immunity. Pt. is very infectious • HISTOPATHLOGICAL APPEARACE Dermis histiocytic granuloma globi /lepra (diagnostic) Space bet.lesion in the dermis & the epidermis. (diagnostic) Large no. of the bacilli. • TUBERCULOID LEPROSY TL Skin & peripheral nerves only. CMI Patients are not infectious. • HISTOPATHOLOGICAL APPEARANCE Dermis epithelioid cell granuloma:- No space between lesions in the dermis & epidermis. Scanty bacilli. • BORDERLINELINE LEPROSY BL DIMORPHUS. Unstable form Intermediate immunity. Mixed features of LL&TL. Less severe. Moderate no. of bacilli. Epitheloid cell infiltrate in the dermis . Without treatment moves towards LL or TL • BUBA STIM Leprosy like all disease have symptoms and signs. Symptoms : what patient complain of. Signs : what medical worker find in examination. • What people think in leprosy • Clinical symptoms Early: A small patch of recent origin either paler or redder than normal skin. Numbness and tingling of hands and/or the feet Burning sensation in the skin Slight weakness of the face hands and feet • WOMEN MARIAM Late: More and larger skin patches. Painless injuries, burns or ulcers. Obvious nodule and or thickening of the skin. More severe weakness or paralyses of muscle of face, hand or feet NOTE: Both early and late cases are very seldom complains of all symptoms that are listed SIGNS: There are 3 cardinal signs of leprosy, usually more than one of these signs is present , but if you are absolutely certain of only one of them this is sufficient to prove the diagnosis. i. Diminished sensation to cotton wool touch in a patch in the skin. NOTE: Loss of sensation in hands and feet due to nerve damage from causes other than leprosy , but diminished sensation in visible patches in the skin is always due to leprosy. ii. Enlargement of the peripheral nerve. NOTE: Severe disease causes nerve tenderness but only leprosy causes nerve enlargement with loss of function. iii. The demonstration of acid fast mycobacterium in skin smear. Mycobacterium leprae can’t grow in cell free culture. Thus laboratory confirmation of leprosy requires histopathological finding consistent with clinical disease & skin test reactivity to lepromin. • LL & borderline lepromatous disease Diagnosed by demonstration of M.leprae in a material obtained by a slit skin smear. Smears are made from skin lesions, earlobes and dorsum of the ring or middle fingers _sites of fast bacillary replication. Nasal mucosa also contains the organism in lepromatous leprosy and is good indication of infectivity. • Tuberculoid leprosy M.leprae is undetectable in skin smears of tuberculoid leprosy In which the cardinal signs and clinical manifestations are used in diagnosis. Biopsy of skin or nerves is seldom necessary except for accurate classification. • Lepromin test Lepromin is a suspension of dead M.leprae. 1-10 lepromin units’ of purified protein derivative in 0.1 ml normal saline intradermally in flexor aspect of the forearm 2-test is positive if after 4 weeks there is at least 5mm induration with surrounding erythema. +ve result indicates good cellular immunity. The test is of no value in establishing the diagnosis of leprosy, but it’s useful in helping to classify the disease, and so determine treatment and prognosis. 600 B.C Hydnocarpus oil Early of 20th century Early1960's Dapsone 1970's Dapson (DDS) Clofazimine (combination with Rifampicin Now cured by MDT (multidrug therapy) cure: Most patients in 6 month More infectious patients within 1 year Basic principles in using MDT: 1-Rifampicin should be one of the components of MDT. 2-Rifampicin 600 mg should be given at least once a month to all patients. • 3-At least 2 anti-leprosy drugs should be used in MB (multi-bacillary) regimen & 1 anti-leprosy drug in PB (pauci-bacillary) regimen in addition to Rifampicin in order to prevent the occurrence of Rifampicin resistant mycobacterium leprae • DRUGS USED IN MDT 1-DAPSON: 2-RIFAMPICIN: 3-CLOFAZIMINE: MDT: can prevent the resistance of M.leprae to anti-leprosy drugs all evidence indicate that it is safe during pregnancy in practice side effects reported from the use of MDT in thousands of patients show that most of them are mild is not contraindicated in patients suffering from TB or HIV infections. • WHO-recommended MDT Regimens • • • RIFAMPICIN • 600 mg ONCE A MONTH • DAPSONE • 100 mg DAILY • CLOFAZIMINE • 3OO mg ONCE A MONTH • • 50 mg DAILY • DURATION • 12 MONTHS • Pauci-bacillary leprosy For adults the standard regimens is: • • • RIFAMPICIN • 600 mg ONCE A MONTH • DAPSONE • 100 mg DAILY • DURATION • 6 MONTHS • • Single Skin Lesion Pauci-bacillary leprosy For adults the standard regimens is a single dose of: Rifampicin Ofloxacin 600 mg 400 mg Minocycline 100 mg • Leprosy reactions 1\Reversal reaction 2\ENL(erythema nodosum leprosum). MANAGEMENT 1-Case finding: To do that the following must be done: 1\ Awareness of health workers. 2\Community awareness. Case finding in developing countries poses special public health problems. For socio-economic reasons case finding is more complex in towns than in rural areas. 2-Treatment of cases: This requires: 1\Proper health centers. Leprosy clinics increased in number from 10 clinics in 1991 to 721 in 2003. 2\ MDT is available free of charge at all health facilities. Leprosy can be completely cured when early and regular treatment prevent deformities. Patients can live completely normal lives during and after their treatment BUT 3-Case follow-up: I. Supervision. a. Making a detailed supervising plan. b. Distributing the supervision lists (sheets). c. Provision of the tools (equipments) of supervision. • II. Patient investigation (assay) and follow-up: – Pt should be investigated regularly even after the complete cure of the case in order to discover recurrence and hypersensitivity reactions early enough. Treatment of reactions: • What should always be kept in mind is the prevention of disabilities which could rise from the disease . • That's by : • 1- provision of the treating drugs • 2- health awareness • 3- training • WHAT IS STIGMA ??? DEFINITION Is fear and rejection of the patient from the society, individuals and even his family • CAUSES OF STIGMA Fear of infection. Multilating deformity Disability results from these deformities. Ulceration , cellulitis , scarring ,bone deformity (seen in LL) . In advanced stages , blindness. • THE RESULT The patient faces considerable employment, housing ,and family problems. The patient may react with denial of the disease, depression and inferiority feeling or may try to hide or travel to another country. • MANAGEMENT • The treatment of leprosy is not only the chemotherapy . • MANAGEMENT REHABILITATION: 1. PHYSICAL 2. SOCIAL AND PSYCHOLOGICAL • PHYSICAL REHABILITATION We have to minimize potential physical deformities and to prevent contracture. The patient needs education in how to deal with anaesthesia of the hands and feet and how to deal with the existing nerve damage. Patients must learn to carefully inspect their extremities or trauma and wear proper foot wear. • SOCIAL &PSYCHOLOGICAL REHABILITATION Firstly , the patient fears must be addressed because of the cultural stigma associated with the disease. The patient may need psychological counseling because they may have difficulty in coming to term with the disease or in feeling rejected by the society. Social workers is essential to assist patients in obtaining housing for homeless patient , job placement and welfare payment for needy patients. • References: • World wide web. • Guide to eliminate leprosy as a public health problem. • Lippincotte pharmacology • Atlas of leprosy. • Leprosy in africans.