SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition A chronic granulomatous infection and its...

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SEMINAR SEMINAR Dr. AAKANKSHA GODIYAL Dr. AAKANKSHA GODIYAL

Transcript of SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition A chronic granulomatous infection and its...

Page 1: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

SEMINAR SEMINAR

Dr. AAKANKSHA GODIYALDr. AAKANKSHA GODIYAL

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LEPROSYLEPROSY

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DefinitionDefinition

A chronic granulomatous infection A chronic granulomatous infection and its sequalae caused by and its sequalae caused by Mycobacterium leprae affecting skin Mycobacterium leprae affecting skin and nerves primarilyand nerves primarily

More than 800,000 new cases More than 800,000 new cases detected worldwise annually detected worldwise annually

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HistoryHistory

Ancient diseaseAncient disease Writings from India describe similar Writings from India describe similar

illness as early as 6illness as early as 6thth century BC century BC Imported to Europe by troops of Imported to Europe by troops of

Alexander.European epidemic Alexander.European epidemic peaked in 13peaked in 13thth century century

French settlers took it to Canada and French settlers took it to Canada and black slaves to America black slaves to America

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ARMAUER HANSEN of Norway ARMAUER HANSEN of Norway attributed it to M.leprae in 1873attributed it to M.leprae in 1873

Effective chemotherapy began with Effective chemotherapy began with sulfones in 1943sulfones in 1943

Rifampicin introduced in 1970Rifampicin introduced in 1970 Grown in mouse footpad in 1961 & in Grown in mouse footpad in 1961 & in

nine banded armadillo in 1971nine banded armadillo in 1971 Sequence of genome published in Sequence of genome published in

2001 2001

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EPIDEMIOLOGYEPIDEMIOLOGY

Endemic in all continents except Endemic in all continents except AntarcticaAntarctica

2/32/3rdrd of world’s leprosy burden in of world’s leprosy burden in Indian subcontinentIndian subcontinent

Incidence:800,000 per yearIncidence:800,000 per year Not always a tropical disease; was Not always a tropical disease; was

endemic in norway till 20endemic in norway till 20thth century century IP=2 to 5 yr for tuberculoid & 8 to 12 IP=2 to 5 yr for tuberculoid & 8 to 12

yr for lepromatous cases yr for lepromatous cases

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Loss of 1 million disability adjusted Loss of 1 million disability adjusted life yearslife years

Majority of cases before 35 yr age Majority of cases before 35 yr age with median age less for tuberculoid with median age less for tuberculoid casescases

Excess of male cases regularly foundExcess of male cases regularly found Clustering of cases well recognisedClustering of cases well recognised HLA associationHLA association

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HLA-DR2/DR3 occur at higher frequency in HLA-DR2/DR3 occur at higher frequency in TT/BT patientsTT/BT patients

HLA-DQ1 increase susceptibility to BL/LL HLA-DQ1 increase susceptibility to BL/LL leprosyleprosy

In South India assoc. found between In South India assoc. found between paucibacillary leprosy and 10p13 paucibacillary leprosy and 10p13 chromosomechromosome

Main source of infection- nasal discharge Main source of infection- nasal discharge from untreated LL patientsfrom untreated LL patients

Not excreted by skin Not excreted by skin

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AETIOLOGYAETIOLOGY Classified under order Actinomycetalis and Classified under order Actinomycetalis and

family Mycobacteriacaefamily Mycobacteriacae Straight/slightly curved rods with parallel Straight/slightly curved rods with parallel

sides and round endssides and round ends Gram +ve, acid fast. Characteristically Gram +ve, acid fast. Characteristically

acid fastness lost with pyridine extractionacid fastness lost with pyridine extraction Obligate intracellular parasite especially in Obligate intracellular parasite especially in

macrophagesmacrophages Noncultivable.. Grows at 30-33 degree Noncultivable.. Grows at 30-33 degree

centigradecentigrade Doubling time= 12-13 daysDoubling time= 12-13 days

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Genome of M.leprae(3.27 Genome of M.leprae(3.27 megabases) shorter than megabases) shorter than M.tuberculosis(4.41 megabases)M.tuberculosis(4.41 megabases)

ULTRASTRUCTUREULTRASTRUCTURE Following components from inside Following components from inside

out:out: Cytoplasm Cytoplasm Trilaminar plasma membraneTrilaminar plasma membrane

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Cell wall is 20 nm thick & electron dense. Cell wall is 20 nm thick & electron dense. Consists of peptidoglycan covalently Consists of peptidoglycan covalently attached to arabinogalactan polymer attached to arabinogalactan polymer modified by branched chain mycolic acid. modified by branched chain mycolic acid. Lipoarabinomannan is another important Lipoarabinomannan is another important componentcomponent

Capsule is electron transparent due to Capsule is electron transparent due to copious amounts of lipid- phthiocerol copious amounts of lipid- phthiocerol dimycocerosate & phenolic glycolipiddimycocerosate & phenolic glycolipid

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PGL-1 is species specific and PGL-1 is species specific and immunogenicimmunogenic

Entry into nerves mediated by Entry into nerves mediated by binding of PGL1 to laminin 2 in basal binding of PGL1 to laminin 2 in basal lamina of Schwann cellslamina of Schwann cells

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PATHOLOGIC SPECTRUM OF PATHOLOGIC SPECTRUM OF LEPROSYLEPROSY

Lepra bacilli is non toxic. Clinicopathologic Lepra bacilli is non toxic. Clinicopathologic manifestations are result of manifestations are result of immunopathologyimmunopathology

RIDLEY JOPLING ciassification defines RIDLEY JOPLING ciassification defines categories along a spectrum depending on categories along a spectrum depending on clinical,histopathological, immunological clinical,histopathological, immunological indices:indices:

TT,BT,BB,BL,LLTT,BT,BB,BL,LL TT and polar LL patients are stable while TT and polar LL patients are stable while

others move in either direction according others move in either direction according to host response & treatmentto host response & treatment

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PATHOLOGYPATHOLOGY

TUBERCULOID LEPROSY:Tuberculoid TUBERCULOID LEPROSY:Tuberculoid granulomas found which tend to collect in granulomas found which tend to collect in foci around neurovascular elements.foci around neurovascular elements.

Each focus cosists of epitheloid cells at Each focus cosists of epitheloid cells at centre with surrounding zone of centre with surrounding zone of lymphocytes. Langhans giant cells lymphocytes. Langhans giant cells sometimes foundsometimes found

Some of the foci invade papillary zone and Some of the foci invade papillary zone and even erode basal layereven erode basal layer

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Bacilli not seenBacilli not seen Dermal nerves are either completely Dermal nerves are either completely

destroyed or swollen by epithelioid destroyed or swollen by epithelioid cell granuloma. Occasionally there cell granuloma. Occasionally there may be caseation within a nervemay be caseation within a nerve

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BORDERLINE LEPROSYBORDERLINE LEPROSY

BT:BT: Epithelioid cell granuloma more diffuse Epithelioid cell granuloma more diffuse

with a free but narrow papillary zone. with a free but narrow papillary zone. Giant cells tend to be of foreign body type.Giant cells tend to be of foreign body type.

Dermal nerves are moderately swollen by Dermal nerves are moderately swollen by cellular infiltration or schwann cell cellular infiltration or schwann cell proliferationproliferation

Bacilli absent from dermis but likely to be Bacilli absent from dermis but likely to be found witin dermal nervesfound witin dermal nerves

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Granulomas follow neurovascular Granulomas follow neurovascular bundles, sweat glands or erector pili bundles, sweat glands or erector pili musclesmuscles

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BB BB

Diffuse epithlioid cell granuloma with Diffuse epithlioid cell granuloma with scanty lymphocytes and no giant scanty lymphocytes and no giant cellscells

Papillary zone is clear and dermal Papillary zone is clear and dermal nerves show slight swelling with nerves show slight swelling with cellular infiltratecellular infiltrate

Bacilli present within dermal nerves Bacilli present within dermal nerves & dermis in modest numbers& dermis in modest numbers

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BLBL

There is macrophage granuloma in There is macrophage granuloma in which some cells show slight foamy which some cells show slight foamy changes. Lymphocytes present in changes. Lymphocytes present in dense clumps or widely distributed in dense clumps or widely distributed in parts of granuloma. Occasionally parts of granuloma. Occasionally epithelioid cells seenepithelioid cells seen

Papillary zone is freePapillary zone is free Bacilli are plentiful-singly or in Bacilli are plentiful-singly or in

clumpsclumps

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Dermal nerves contain some cellular Dermal nerves contain some cellular infiltrate & perineurium may have infiltrate & perineurium may have laminated appearance(onion skin laminated appearance(onion skin perineurium)perineurium)

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LEPROMATOUS LEPROSYLEPROMATOUS LEPROSY

Extensive cellular infiltrate that is Extensive cellular infiltrate that is separated from flattened epidermis by separated from flattened epidermis by Grenz zone of normal collagenGrenz zone of normal collagen

In early lesions macrophages have mixed In early lesions macrophages have mixed population of solid and fragmented population of solid and fragmented bacilli.Solid bacilli are packed like cigars. bacilli.Solid bacilli are packed like cigars. Bacilli may be seen in endothelial cellsBacilli may be seen in endothelial cells

No epithelioid cells. Lymphocytes not No epithelioid cells. Lymphocytes not prominent. Plasma cells may be seenprominent. Plasma cells may be seen

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In time degenerated bacilli In time degenerated bacilli accumulate in macrophages so accumulate in macrophages so called virchow or lepra cells which called virchow or lepra cells which have foamy cytoplasm. In chronic have foamy cytoplasm. In chronic lesions bacilli are disposed in large lesions bacilli are disposed in large basiophilic clumps called globibasiophilic clumps called globi

Dermal nerves are well preserved & Dermal nerves are well preserved & contain large no of bacilli. Slowly contain large no of bacilli. Slowly become fibroticbecome fibrotic

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LUCIO LEPROSYLUCIO LEPROSY

Show similar histological features but Show similar histological features but with characteristic heavy bacillation with characteristic heavy bacillation of small blood vessels in skin causing of small blood vessels in skin causing obliterative angiitis and ischaemic obliterative angiitis and ischaemic epidermal necrosisepidermal necrosis

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Histoid leprosyHistoid leprosy

Shows highest load of bacilli & Shows highest load of bacilli & majority are solid staining arranged majority are solid staining arranged in clumps like sheaves of wheat. in clumps like sheaves of wheat. Macrophages show storiform pattern Macrophages show storiform pattern similar to fibrohistiocytomasimilar to fibrohistiocytoma

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Type 1 reactionType 1 reaction

Edema within & around granuloma Edema within & around granuloma In upgrading reaction granulomas In upgrading reaction granulomas

become more epithelioid & langhans become more epithelioid & langhans giant cells are larger. There may be giant cells are larger. There may be erosion of granuloma into lower erosion of granuloma into lower epidermis epidermis

Fibrinoid necrosis within granulomas Fibrinoid necrosis within granulomas or in dermal nervesor in dermal nerves

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Downgrading reaction: necrosis less Downgrading reaction: necrosis less common. Density of bacilli increasescommon. Density of bacilli increases

Multibacillary patients who upgrade Multibacillary patients who upgrade on therapy show old foamy on therapy show old foamy macrophages mixed with new macrophages mixed with new epithelioid cell granulomasepithelioid cell granulomas

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Type2 reaction(ENL)Type2 reaction(ENL)

Lesions are foci of acute Lesions are foci of acute inflammation on chronic inflammation on chronic multibacillary leprosy. Neutrophils multibacillary leprosy. Neutrophils may be scanty or abundant to form may be scanty or abundant to form abscess. Foamy macrophages with abscess. Foamy macrophages with fragmented bacilli abundantfragmented bacilli abundant

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Lucio reactionLucio reaction

Endothelial proliferation leading to Endothelial proliferation leading to luminal obliteration seen with luminal obliteration seen with thrombosis of medium sized vessels thrombosis of medium sized vessels in dermis & subcutisin dermis & subcutis

Dense aggregates of AFB in walls of Dense aggregates of AFB in walls of normal appearing vessels & vessels normal appearing vessels & vessels with proliferative changeswith proliferative changes

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Indeterminate leprosyIndeterminate leprosy

Mild lymphocyte & macrophage Mild lymphocyte & macrophage accumulation around neurovascular accumulation around neurovascular bundles, sweat glands & erector pili bundles, sweat glands & erector pili muscles. No epithelioid cell granulomas. muscles. No epithelioid cell granulomas. Schwann cell hyperplasia may be seenSchwann cell hyperplasia may be seen

Diagnosis rests on finding 1/more AFB in Diagnosis rests on finding 1/more AFB in sites of predilection- erector pili muscles, sites of predilection- erector pili muscles, just beneath epidermis or around a vesseljust beneath epidermis or around a vessel

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CLINICAL FEATURESCLINICAL FEATURES Indeterminate LeprosyIndeterminate Leprosy: Lesions found : Lesions found

on face, extensor surface of limbs or on face, extensor surface of limbs or buttocks.buttocks.

Consist of one or more slightly Consist of one or more slightly hyperpigmented or erythematous hyperpigmented or erythematous machules with poorly defined margins.machules with poorly defined margins.

Hair growth and nerve functions are Hair growth and nerve functions are normal.normal.

Found usually in children whose immune Found usually in children whose immune status is yet to be determined. Smears are status is yet to be determined. Smears are negative. Sometimes thickened nerve is negative. Sometimes thickened nerve is palpable.palpable.

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POLAR TUBERCULOIDPOLAR TUBERCULOID

Primary lesion is a plaque assuming Primary lesion is a plaque assuming an annular configuration. Both the an annular configuration. Both the borders are sharply marginated.borders are sharply marginated.

Lesion is indurated, elevated, scaly, Lesion is indurated, elevated, scaly, dry, hairless and hypopigmented.dry, hairless and hypopigmented.

Nearby sensory nerve may or may Nearby sensory nerve may or may not be enlarged but the lesion is not be enlarged but the lesion is anaesthetic & anhidrotic.anaesthetic & anhidrotic.

Usually solitary.Usually solitary.

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Spontaneous cure is known.Spontaneous cure is known. Upper limit of 10 cm on lesion size.Upper limit of 10 cm on lesion size.

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BORDERLINE TUBERCULOIDBORDERLINE TUBERCULOID

Primary lesion are plaques and papules.Primary lesion are plaques and papules. Annular configuration is common with both Annular configuration is common with both

borders sharply defined but incomplete borders sharply defined but incomplete annular lesions may be seen. Satellite annular lesions may be seen. Satellite lesions present.lesions present.

Little scaling, less erythema, less Little scaling, less erythema, less induration, size more than 10 cm.induration, size more than 10 cm.

Multiple asymmetric lesions are a rule.Multiple asymmetric lesions are a rule. Loss of sensation in skin lesion, nerve Loss of sensation in skin lesion, nerve

trunk enlargement and palsies seen.trunk enlargement and palsies seen.

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Nerve trunk involvement asymmetric Nerve trunk involvement asymmetric and not more than 2.and not more than 2.

Nerve abscesses are most often seen Nerve abscesses are most often seen in males with BT disease.in males with BT disease.

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MID BORDERLINEMID BORDERLINE

Immunological midpoint Immunological midpoint Characteristic lesions are annular with Characteristic lesions are annular with

sharply marginated interior and exterior sharply marginated interior and exterior margins or large plaques with islands of margins or large plaques with islands of normal skin within the plaque giving a normal skin within the plaque giving a Swiss cheese appearance or a classic Swiss cheese appearance or a classic dimorphic lesion.dimorphic lesion.

Because of instability BB is shortlived and Because of instability BB is shortlived and rarely seen. rarely seen.

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BORDERLINE LEPROMATOUSBORDERLINE LEPROMATOUS

Highly variable in clinical expressionHighly variable in clinical expression In 1/3In 1/3rdrd patients classic dimorphic lesion patients classic dimorphic lesion

seen. Annular lesions with both borders seen. Annular lesions with both borders sharply defined uncommon but when they sharply defined uncommon but when they occur lepromatous like papules & nodules occur lepromatous like papules & nodules seen.seen.

Lesions may be hypoaesthetic/anaesthetic Lesions may be hypoaesthetic/anaesthetic but not necessarily so.but not necessarily so.

Nerve trunk palsies have highest Nerve trunk palsies have highest prevalence in BL. prevalence in BL.

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Untreated BL has a relentless Untreated BL has a relentless progression. When disease is progression. When disease is extensive acral distal symmetric extensive acral distal symmetric anaesthesia may be present. anaesthesia may be present.

Course altered by reactional states.Course altered by reactional states.

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LEPROMATOUS LEPROSYLEPROMATOUS LEPROSY

Poorly defined skin coloured nodules are Poorly defined skin coloured nodules are the most characteristic & symmetrically the most characteristic & symmetrically distributed.distributed.

Diffuse dermal infiltration is always Diffuse dermal infiltration is always present, manifested by widening of nasal present, manifested by widening of nasal root and fusiform swelling of fingers.root and fusiform swelling of fingers.

Skin over forehead thickened causing Skin over forehead thickened causing Leonine facies. Earlobes thickened.Leonine facies. Earlobes thickened.

Ciliary or superciliary madarosis +Ciliary or superciliary madarosis +

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Macules are small, numerous, Macules are small, numerous, erythematous, vague and shiny with no erythematous, vague and shiny with no loss of sensation or hair growth. Papules loss of sensation or hair growth. Papules may also be present may also be present

Sometimes, all 3 lesions may be present Sometimes, all 3 lesions may be present Chronic edema of leg is usualChronic edema of leg is usual Nerve trunk palsies less common than in Nerve trunk palsies less common than in

BL. Acral distal symmetric(glove & BL. Acral distal symmetric(glove & stocking) anaesthesia seen stocking) anaesthesia seen

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Systemic associationsSystemic associations Nose may collapse with septal perforation, Nose may collapse with septal perforation,

voice may become hoarse, upper incisor voice may become hoarse, upper incisor teeth may loosen and fall offteeth may loosen and fall off

Shortening of fingers and toes due to Shortening of fingers and toes due to repeated traumarepeated trauma

Anaesthetic skin liable to blister and Anaesthetic skin liable to blister and ulcerationulceration

Eyes- superficial punctate keratitis, Eyes- superficial punctate keratitis, insidious iritis (beginning as iris pearls) insidious iritis (beginning as iris pearls)

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Damage to cranial nerve V & VII- corneal Damage to cranial nerve V & VII- corneal insensitivity and lag ophthalmos insensitivity and lag ophthalmos predispose cornea to injuriespredispose cornea to injuries

Bone changes occur late- periostitis of Bone changes occur late- periostitis of bones of leg and forearms, atrophy of bones of leg and forearms, atrophy of distal phalanges of hands & atrophy of distal phalanges of hands & atrophy of phalanges, metatarsal & tarsal bones of phalanges, metatarsal & tarsal bones of feetfeet

In skull atrophy of anterior nasal spine and In skull atrophy of anterior nasal spine and of maxillary alveolar process lead to facies of maxillary alveolar process lead to facies leprosaleprosa

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Kidney- renal damage important Kidney- renal damage important cause of morbidity & mortality (renal cause of morbidity & mortality (renal amyloidosis, glomerulonephritis, etc)amyloidosis, glomerulonephritis, etc)

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PREGNANCY & LEPROSYPREGNANCY & LEPROSY

1. Worsening of leprosy status (third 1. Worsening of leprosy status (third trimester of pregnancy)trimester of pregnancy)

2. Type I reaction (during first six 2. Type I reaction (during first six months of lactation)months of lactation)

3. Type II reaction (in third trimester 3. Type II reaction (in third trimester & first six months of lactation)& first six months of lactation)

50 % suffer deterioration of nerve 50 % suffer deterioration of nerve function function

Page 73: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

LUCIO LEPROSYLUCIO LEPROSY Diffuse non nodular type. Described by Diffuse non nodular type. Described by

Lucio and Alvarado in Mexico.Lucio and Alvarado in Mexico. hands, wide spread sensory loss. Shiny hands, wide spread sensory loss. Shiny

thickened skin, body hair loss, puffy thickened skin, body hair loss, puffy Eyes- shiny appearenceEyes- shiny appearence Anaemia, edema, ulceration of both legsAnaemia, edema, ulceration of both legs Nasal symptoms presentNasal symptoms present No motor palsies and eyes not damaged.No motor palsies and eyes not damaged.

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HISTOID HANSENSHISTOID HANSENS

Term introduced by Wade Term introduced by Wade Applied to firm erythematous round shiny Applied to firm erythematous round shiny

glistering nodules which appear on skin of glistering nodules which appear on skin of patients whose disease is relapsing either patients whose disease is relapsing either because they have stopped treatment or because they have stopped treatment or because M. leprae has become drug because M. leprae has become drug resistantresistant

Histoid nodules contain elongated spindle Histoid nodules contain elongated spindle shaped histiocytes with bacilli within themshaped histiocytes with bacilli within them

Page 76: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

TYPE I LEPRA REACTIONTYPE I LEPRA REACTION

Delayed (type IV) hypersensitivity Delayed (type IV) hypersensitivity reaction reaction

Antigens from breaking down of Antigens from breaking down of lepra bacilli react with T-lymphocyteslepra bacilli react with T-lymphocytes

Seen in borderline patientsSeen in borderline patients Increase in CMI associated with Increase in CMI associated with

treatment known as reversal reaction treatment known as reversal reaction whereas reduction in immunity called whereas reduction in immunity called downgrading reactiondowngrading reaction

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Upgrading most common during first Upgrading most common during first six months of treatmentsix months of treatment

LLs->BL->BB->BT->TTsLLs->BL->BB->BT->TTs Rapidly developing change in Rapidly developing change in

some/all skin lesions- become some/all skin lesions- become erythematous, shiny, warm and erythematous, shiny, warm and tender. Sometimes necrosis occurs tender. Sometimes necrosis occurs leading to ulceration. Lesions leading to ulceration. Lesions desquamate as the subsidedesquamate as the subside

Page 78: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

Systemic features less commonSystemic features less common Nerve involvement- swelling of one Nerve involvement- swelling of one

or more nerves with pain and or more nerves with pain and tendernesstenderness

Most serious complication is motor Most serious complication is motor disturbance and nerves at risk are disturbance and nerves at risk are ulnar, lateral popliteal and facialulnar, lateral popliteal and facial

Paralysis can recover with prompt Paralysis can recover with prompt treatment treatment

Page 79: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

Edema of hands, feet or faceEdema of hands, feet or face Tenderness of palms and solesTenderness of palms and soles

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TYPE II LEPRA REACTION TYPE II LEPRA REACTION

Is an immune complex syndrome (type III) Is an immune complex syndrome (type III) hypersensitivity reactionhypersensitivity reaction

Occurs almost always LLp/LLs and Occurs almost always LLp/LLs and occasionally in BLoccasionally in BL

No change of leprosy lesions but crops of No change of leprosy lesions but crops of brightly red nodules seen which come and brightly red nodules seen which come and gogo

Systemic disturbance usualSystemic disturbance usual Unusual for it to occur during first six Unusual for it to occur during first six

months of treatment months of treatment

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ENL lesions tend to be bilaterally ENL lesions tend to be bilaterally symmetrical. Often tender and blanch with symmetrical. Often tender and blanch with light pressure. Evanescent lasting two-light pressure. Evanescent lasting two-three daysthree days

Commonly on face, arms and thighs. Commonly on face, arms and thighs. Leave bluish stain as they fadeLeave bluish stain as they fade

In severe reactions lesions may become In severe reactions lesions may become vesicular and break down (erythema vesicular and break down (erythema necroticans)necroticans)

Other features- neuritis, myositis, arthritis, Other features- neuritis, myositis, arthritis, rhinitis, acute iritis, dactylitis, rhinitis, acute iritis, dactylitis, epididymoorchitis and proteinuria epididymoorchitis and proteinuria

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Factors precipitating ENLFactors precipitating ENL Physical/ mental stressPhysical/ mental stress Immunization Immunization Intercurrent infection/injury/surgeryIntercurrent infection/injury/surgery Pregnancy/parturitionPregnancy/parturition Ingestion of KI besides anti-leprosy Ingestion of KI besides anti-leprosy

drugs drugs

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LUCIO PHENOMENONLUCIO PHENOMENON Confined to diffuse non-nodular LL leprosy Confined to diffuse non-nodular LL leprosy

chiefly encountered in Mexicanschiefly encountered in Mexicans Unique feature- seen only in untreated Unique feature- seen only in untreated

patientspatients Painful red patches appear on the skin Painful red patches appear on the skin

especially extremities, become purpuric, especially extremities, become purpuric, ulcerate and finally develop an eschar ulcerate and finally develop an eschar which falls off to leave a superficial which falls off to leave a superficial atrophic scaratrophic scar

Face and trunk are spared. Patients Face and trunk are spared. Patients afebrile afebrile

Page 88: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

Steroids- show good effectSteroids- show good effect Thalidomide of no valueThalidomide of no value Good results with DapsoneGood results with Dapsone Excellent results with RifampicinExcellent results with Rifampicin

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DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

I. Neurological conditionsI. Neurological conditions

PALPABLE N. THICKENING WITHOUT PALPABLE N. THICKENING WITHOUT ANAESTHESIA/SIGNS OF N. DAMAGEANAESTHESIA/SIGNS OF N. DAMAGE

1. Excessive muscular development 1. Excessive muscular development

2. Pachydermoperiostitis2. Pachydermoperiostitis

Page 91: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

PALPABLE N. THICKENING WITH PALPABLE N. THICKENING WITH REGIONAL ANAESTHESIA REGIONAL ANAESTHESIA WITH/WITHOUT MUSCLE WASTING WITH/WITHOUT MUSCLE WASTING

1.1. Primary amyloidosis of peripheral Primary amyloidosis of peripheral nerves nerves

2.2. Familial hypertrophic interstitial Familial hypertrophic interstitial neuritisneuritis

Page 92: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

REGIONAL ANAESTHESIA REGIONAL ANAESTHESIA WITH/WITHOUT MUSCLE WASTING WITH/WITHOUT MUSCLE WASTING BUT WITH NERVE THICKENINGBUT WITH NERVE THICKENING

1.1. Recurrent/chronic progressive Recurrent/chronic progressive polyneuritis polyneuritis

2.2. Peroneal muscular atrophy Peroneal muscular atrophy (charcot-marie-tooth syndrome) (charcot-marie-tooth syndrome)

Page 93: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

REGIONAL ANAESTHESIA REGIONAL ANAESTHESIA WITH/WITHOUT MUSCLE WASTING WITH/WITHOUT MUSCLE WASTING BUT WITHOUT NERVE THICKENINGBUT WITHOUT NERVE THICKENING

1.1. SyringomyeliaSyringomyelia2.2. Tabes dorsalisTabes dorsalis3.3. Peripheral neuropathies Peripheral neuropathies 4.4. Hereditary sensory radicular Hereditary sensory radicular

neuropathyneuropathy5.5. Congenital indifference to painCongenital indifference to pain

Page 94: SEMINAR Dr. AAKANKSHA GODIYAL. LEPROSY Definition  A chronic granulomatous infection and its sequalae caused by Mycobacterium leprae affecting skin.

II. DERMATOLOGICAL CONDITIONSII. DERMATOLOGICAL CONDITIONS None of the conditions listed below have None of the conditions listed below have

all the three features of sensory loss, all the three features of sensory loss, nerve thickening and +ve skin smear for nerve thickening and +ve skin smear for AFBAFB

A.A. Flat and hypopigmented lesionsFlat and hypopigmented lesions1.1. MorphoeaMorphoea2.2. OnchocerciasisOnchocerciasis3.3. Pityriasis albaPityriasis alba4.4. Tenia versicolorTenia versicolor

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5. Post kala azar dermal leishmaniasis5. Post kala azar dermal leishmaniasis6. Yaws 6. Yaws 7. Vitiligo7. VitiligoB. Raised and pigmented lesionsB. Raised and pigmented lesions1.1. Follicular mucinosis (alopecia mucinosa)Follicular mucinosis (alopecia mucinosa)2.2. Granuloma annulareGranuloma annulare3.3. Granuloma multiformeGranuloma multiforme4.4. Gyrate erythemasGyrate erythemas5.5. Kaposi’s sarcomaKaposi’s sarcoma6.6. Cutaneous leishmaniasisCutaneous leishmaniasis7.7. Lupus erythematosusLupus erythematosus

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8. Lupus vulgaris8. Lupus vulgaris9. Mycobacterium marinum infection9. Mycobacterium marinum infection10. Mycosis fungoides10. Mycosis fungoides11. Neurofibromatosis11. Neurofibromatosis12. P.rosea12. P.rosea13. Tinea corporis13. Tinea corporisc. Generalized thickening of skinc. Generalized thickening of skin1.1. Systemic sclerosisSystemic sclerosis2.2. MyxoedemaMyxoedema3.3. PachydermoperiostosisPachydermoperiostosis

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CONCLUSIONCONCLUSION

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