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Classification & Clinical Features of Leprosy
Classification & Clinical Features of Leprosy
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HANSEN's DISEASE
FORMAT
INTRODUCTION/DEFINITION BASIS FOR CLASSIFICATION OF HANSENS DISEASE CLASSIFICATION OF HANSENS DISEASE PECULIARITIES OF EACH SUBCLASSIFICATION CLINICAL FEATURES OF HANSENS DISEASE COMPLICATIONS DIAGNOSIS OF LEPROSY
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HANSENs disease
Leprosy is a chronic infectious systemic disease that primarily affects the peripheral nerves & skin.other organs that can be affected in long term include nasal mucosa,larynx,eyes,testis,liver,kidney etc. The disease is caused by a rod shaped bacillus called mycobacterium laprae & mycobacterium lepromatosus M laprae was discovered by G.A Hansen in Norway in 1873.Its a slowly growing obligate intracellular pathogen incapable of independent existance outside its host.
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HANSEN's DISEASE
contd
The organism cant be grown in the laboratory Can only be grown in armadillos & immunocompromised mice Mycobacterium leprae has an average doubling time of 12-14 days Human, Armadillos,& 3 species of monkeys(chimpanzee, Sooty mangabey & Cynomolgus macaques) are the only known sufferers from Hansens disease. Disease can take up to 20-40 years before manifesting. Above constitutes many impediments to the study, prevention & control of this ancient disease.
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HANSEN's DISEASE
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HANSEN's DISEASE
Lepers colony
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HANSEN's DISEASE
The disease
Mode of transmission of disease is via respiratory system, through nasal droplets. Transmission via broken skin is a possibility. The primary sites that are affected by leprosy are superficial site of the skin & peripheral nerves as the bacteria have predilection for low temperature sites as it enhances its survival
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MULTIBACILLARY
BORDERLINE
MULTIBACILLARY
LEPROMATOUS
NEGATIVE
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HANSEN's DISEASE
An understanding of the spectral concept help in the understanding of the multiplicity of the clinical manifestation of this disease Tuberculoid leprosy is associated with severe nerve damage, lepromatous leprosy with chronicity & long term complications Spontaneous healing can occur near the tuberculoid pole but almost never in the lepromatous pole Polar disease are immunologically stable & are unlikely to change their position on the spectrum,meaning that ,type 1 reactional states are not often encountered. Borderline disease are unstable & may move either way along the spectrum.Such a shift in cellular immunity is often associated with an acute reaction(type 1),which may occur spontaneously or with treatment causing severe multiple nerve damage. Lepromatous leprosy are likely to suffer reactions mediated by antigen antibody complex(type 2 reaction) Classification of leprosy provides a basis for matching of research patients Classification of patients seen during a survey may help in the planning of control measures
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Value of classification
Classification of leprosy
Leprosy usually affects the skin,nerve & nasal mucosa but presents with varying degree of clinical presentation. Leprosy is a spectrum disease.the point any patient occupy on the spectrum is reflective of the balance btwn cellular immunity(Th1) & bacilli multiplication & consequent tissue invasion.
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Classification of leprosy
Pts with good celluler immunity develop tuberculoid leprosy(TT) limited to the skin & nerve. Those with poor immunity develop lepromatous leprosy(LL) xterised by symmetric skin lesions,nodules,plaques & thickened dermis and also have widespread systemic affectation. In btwn these two extremes are the borderline disease-borderline tuberculoid(BT),mid borderline or borderline borderline(BB) & border line lepromatous.
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Classification continued
Indeterminate leprosy=earliest form of lesion,presents as 1 or 2 hypopigmented anaesthetic patch which can heal spontaneously or progress and enter the spectrum. Tuberculoid leprosy(TT) Borderline tuberculoid(BT) Midborderline/Borderline borderline(BB) Borderline lepromatous(BL) Lepromatous leprosy(LL) TT-------------BT---------------BB--------------BL------------------LL
polar dx
----------borderline disease----------
polar dx
Moving from left to right,the following occurs Decrease in cellular immunity & failure of disease containment Increase in bacillary multiplication & tissue invasion. Increase in number of skin lesion but decreased definition of lesion Positive to negative lepromin test Increase in bacillary index & morphogenic index
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Can be either one large red patch with welldefined raised borders or a large hypopigmented asymmetrical spot Lesions become dry and hairless Loss of sensation may occur at site of some lesions bcs of damaged peripheral nerves Tender, thickened nerves with subsequent loss of function are common Spontaneous resolution may occur in a few years or it may progress to borderline or rarely lepromatous types
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Similar to tuberculoid type except that lesions are smaller and more numerous Disease may stay in this stage or convert back to tuberculoid form, or progress
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BORDERLINE BORDERLINE
Disease is of intermediate severity,most common form of leprosy. Skin lesions resembles tuberculoid leprosy but are more numerous & irregularly shaped. Large patch may affect a whole limb Peripheral nerve involvement with weakness & loss of sensation is common. Sensory loss is moderate Midborderline disease is characterised by instability,disease may advanced towards the lepromatous pole(downgrading) or undergo a reversal reaction becoming more like a tuberculoid leprosy(upgrading)
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Borderline lepromatous
Numerous lesions of all kinds, plaques, macules, papules and nodules. Lesions looking like inverted saucers are common Hair growth and sensation are usually not impaired over the lesions
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Lepromatous leprosy
Characterised by presence of symmetric skin lesions of various types Lesions like plaques, macules, papules and nodules are seen Early symptoms include nasal stuffiness, discharge and bleeding, and swelling of the legs and ankles Left untreated, the following problems may occur:
Skin thickens over forehead (leonine facies), eyebrows and eyelashes are lost(madarosis), nose becomes misshapen or collapses, ear lobes thicken, upper incisor teeth fall out Eye involvement causing photophobia (light sensitivity), glaucoma and blindness Skin on legs thickens and forms ulcers when nodules break down Testicles shrivel causing sterility and enlarged breasts (males) Internal organ infection causing enlarged liver and lymph nodes Voice becomes hoarse due to involvement of the larynx
Slow scarring of peripheral nerves resulting in nerve thickening and sensory loss. Fingers and toes become deformed due to painless repeated trauma. HANSEN's DISEASE 11/17/2013 18
Elevation of margin
Colour of lesion in dark skin Surface character Central healing Sweating & hair growth Loss of sensation Nerve enlargement & damage Bacillary index Lepromin test Natural outcome
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never
Slight hypopigmentation Smooth,shiny None Impaired late late late Many (5 or 6+) negative progression
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common
Marked hypopigmentation Dry,scaly Common Impaired early Early & marked Early & marked absent positive Healing
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ENT
Larynx Kidneys Generalised lymphadenopathy 11/17/2013
Upper sternomastoid Proximal to the flexor retinaculum Upper humerus below deltoid insertion
Anterior tibial
Supra-orbital
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Diagnosis of HD
Anaesthesia-of individual patch or in the distribution of the large peripheral nerve. Charcateristic skin lesion-hypopigmented macules,patches etc Thickened nerves at the site of predilection Acid fast bacilli in skin smear in lepromatous & borderline leprosy Nb-at least 2 of the 1st 3 cardinal signs or the 4th should be present for the diagnosis of leprosy to be made.
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AFB detection
Mycobacterium laprae on ZN stain
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Bacillary index -is a measure of the density of afb on ZN staining 6+= >1000 bacilli in an average field 5+= 100-1000 bacilli in an average field 4+= 10-100 bacillin in an average field 3+= 1-10 bacilli in an average field 2+= 1-10 bacilli in 10 fields 1+= 1-10 bacilli in 100 fields Morphological index Defined as the % of solid(morphologically normal)staining bacilli. MI is a useful index of progress under treatment & changes more rapidly than the bacillary index
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Lepromin test
Lepromin test is a crude semi standardised preparation of bacilli from a lepromatous nodule or an armadillo liver. It is a delayed hypersensitivity reaction to the bacilli 0.1ml is injected intradermally & the site is inspected after 72hrs(Fernandez reaction) & 3 or 4 wks(Mitsuda reaction) for a palpable nodule whose diameter is measured & graded thus No nodule negative 1-2mm doubtful 3-5mm + Over 5mm ++ Ulceration +++ The test is not specific to leprosy & hence cant be relied solely on for diagnosis. Its positive in borderline & tuberculoid leprosy & negative in lepromatous/borderline lepromatous.Hence can be used to distinguish btwn the 2.
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Hansens disease
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Differential diagnosis
As leprosy is a disease with wide spread manifestation,its differential diagnosis is myriad and needs careful consideration given the stigma attached to leprosy(leprophobia!) Good history,physicals including test for sensation is key to distinghising these conditions from hansens disease.
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Macular lesions Birth marks -usually present from birth,usually dont alter their appearance over the course of time.They have irreguler bizarre edge,there is varying degree of hypopigmentation even within the same lesion.The texture & character of the skin is normal,no aneasthesia. Vitiligo-complete depigmentation of any size & shape,no anaesthesia. Post-inflammatory hypopigmentation from previous skin disease eg endemic syphilis,yaws,onchocerciasis,contact dermatitis,burns. Tinea vesicolor - can be hypopigmented,hyperpigmentred or erythematous & covered with white floury scales.Individual lesions are discrete with clear edge.They often fuse to cover large areas.lesions are usually confined to an area of the skin,usually the trunk.Lesion are not anaesthetic,no AFB seen.
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Differential diagnosis
Cutaneous tuberculosis-(lupus vulgaris)-characterised by extensive tissue destruction, presence of scar tissue.lesion show sign of healing from one end & extension at the other end. Cutaneous leishmaniasis Kaposi sarcoma-lesion are usually purplish,highly vascular & easily bleeds,usually associated with lymphedema.patient have evidence of immunosupppression. Other condition-molluscum contagiosum,neurofibromatosis,blastomycosis.
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Differential diagnosis of HD
Conditions causing thickened nerves Peroneal muscular dystrophy(Charcot Mary tooth disaese Amyloidosis Refsums disease Dejerine Sotters disease
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Borderline tuberculoid
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Regime
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