CLASSIFICATION & CLINICAL FEATURES OF LEPROSY

By DR UMAR,F.A. RHEUMATOLOGY UNIT DEPT OF MEDICINE. ABUTH-ZARIA.

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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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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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World distribution of leprosy

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Magnitude of the problem

The incidence of leprosy in low endemic area like USA= 100 case/year New case may reach 5000-10,000/year in high endemic areas(Angola, Brazil, Central Africa Republic, DR Congo, India, Madagascar, Mozambique, Nepal & Tanzania) Global prevalence of leprosy according WHO 2008 report is still > 1case/10,000 population. 2008 global registered prevalence from 118 countries =212,802. N@ of new cases in 2007 is a staggering 254,525. representing 4% drop compared to 2006. Over 50 million have been treated & cured of the disease. Over 3 million have been permanently deformed by the disease, resulting in loss of job, societal stigmatization & isolation. 11/17/2013 HANSEN's DISEASE 6

Lepers colony

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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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Classification schemes for HD

WHO PAUCIBACILLARY RIDLEY-JOPLING INDETERMINATE(I) TUBERCULOID LEPROSY(TT) BORDERLINE TUBERCULOID(BT) MeSH TUBERCULOID LEPROMIN TEST POSITIVE

MULTIBACILLARY

MID BORDERLINE OR BORDERLINE BORDERLINE(BB) LEPROMATOUS LEPROSY(LL) BORDERLINE LEPROMATOUS(BL)

BORDERLINE

MULTIBACILLARY

LEPROMATOUS

NEGATIVE

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 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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Clinical features of tuberculoid leprosy-TT

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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BORDERLINE TUBERCULOID LEPROSY

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

Summary of characteristics of leprosy at the poles

manifestations Lepromatous leprosy Tuberculoid leprosy
One or few sites assymetrical Number & distribution of skin Widely disseminated & nerve lesions symmetrical Definition of skin lesions Clarity of margin poor good

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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Summary of systemic manifestation of HD

System Musculoskeletal Mechanism-sensory neuropathy -immobility -chronic ulceration Eyes Mechanism-1.Anaesthesia & exposure 2.Bacillary invasion ,infection 3.Hypersensitivity Neurologic Mechanism-reactional states/bacillary inv Endocrine Manifestation Indolent ulcers,marjolins ulcer,osteomyelitis,septic arthritis,acroosteolysis,osteoporosis,pathological fractures,charcot arthropathy,myopathy. Conjuctivitis,keratitis,uveitis,triachiasis,ectropi on,entropion,lagophtalmos,glaucoma,cataract, anterior/posterior synaechea,sicca symptoms,optic atrophy. Sensory, Autonomic & Motor dysfunction Testiculer damage/atrophy,secondary gynaecomastia.Azoospermia,sterility

ENT
Larynx Kidneys Generalised lymphadenopathy 11/17/2013

Sinusitis,septal/palate perforation & obstructn.

Hoarseness of the voice,stridor. Immune complex nephritis,interstitial nephritis,Amyloid kidney 90% lepromatous HANSEN's DISEASE pts,70% tuberculoid pts 20

Sites of predilection of nerves swelling/injury

Nerve Ulnar nerve Posterior tibial nerve Site of predilection to injury Above the olecranon groove Behind the medial maleoli

Common peroneal(lateral popliteal )

Facial nerve Great auricular nerve Median nerve Radial nerve

Lateral popliteal fossa

Upper sternomastoid Proximal to the flexor retinaculum Upper humerus below deltoid insertion

Anterior tibial
Supra-orbital

Lateral to Halucis longus tendon

Above the supra-orbital margin

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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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Assesment of bacillary & morphogenic indices

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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Differential diagnosis of leprosy

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 contd

Papules /Plaques /annular lesion Ring worm-have preference for warm moist area(cf with Mb laprae prefence for cold part areas of the body) has raised edge when inflammed which contain vesicles & crust not seen HD.Lesion is usually itchy & not anaesthetic. Granuloma multiforme- characterised by cincinate lesions of varying size,lesions have infiltrated border,No hypopigmentation,normal sweat & hair growth. Seborrheic dermatitis-occurs mostly in children.Presents as dry scaly,hypopigmented macules affecting the cheeks,alae nasi,eyebrow,hair line & scalp.lesions show no anaesthesia. Sarcoidosis-can present with macules,papules,nodules & may be difficult to differentiate from tuberculoid leprosy.Lesion are not anaesthetic,there are signs & symptoms of systemic disease.pt may have erythema nodosum & kveim test is positive
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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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Differential diagnosis of hansens disease

Disease presenting with clawing of the digit Dupuytrens contracture-starts with 4th finger 1st . Congenital flexion deformity-5th finger usually. Tertiary yaws-affects 3rd ,4th & 5th fingers. Diseases presenting with plantar ulcers Syringomyelia Diabetic neuropathy Hereditary sensory neuropathy
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Differential diagnosis of leprosy`

Ectropion,entropion,trichiasis-commonly due to trauchoma. Iridocyclitis-onchocercaisis,connective tissue disease,tuberculosis.

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Differential diagnosis of hansens disease

Conditions causing anaesthetic limb/patch Traumatic nerve damage Tropical ataxic neuropathy-xt by weakness,ataxia & optic neuropathy. Syringomyelia-characterised by dissociated anaesthesia,lower motor neuron sign in the upper limb & upper motor neuron sign in the lower limb.

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Borderline tuberculoid

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Borderline tuberculoid leprosy

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WHO MDT regime for adults with HD

Type of leprosy Classification Paucibacillary leprosy Indeterminate,tuberculoid, borderline tuberculoid Multibacillary leprosy Borderline tuberculoid,borderline borderline,bordeline lepromatous,lepromatous Supervised rifampicin 600mg monthly + Supervised clofazimine 300mg monthly + Daily unsupervised Dapsone 100mg & Daily unsupervised Clofazimine 50mg

Regime

Supervised Rifampicin 600mg monthly + Daily unsupervised dapsone 100mg

Duration of treatment 11/17/2013

6 months HANSEN's DISEASE

At least 1-2 years.

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Principles of mgt & control of leprosy

Adequate dissemination of information Early diagnosis & appropriate management Ensure availability of drug regimes used in leprosy Early & appropiate mangement of reactional states Adequate supportive measures

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O no! Too late for MDT to salvage! florid leprosy deformities.

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