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SEMINAR

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SEMINAR Dr. AAKANKSHA GODIYAL Kidney- renal damage important cause of morbidity & mortality (renal amyloidosis, glomerulonephritis, etc) PREGNANCY & LEPROSY 1. – PowerPoint PPT presentation

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Title: SEMINAR


1
  • SEMINAR
  • Dr. AAKANKSHA GODIYAL

2
LEPROSY
3
Definition
  • A chronic granulomatous infection and its
    sequalae caused by Mycobacterium leprae affecting
    skin and nerves primarily
  • More than 800,000 new cases detected worldwise
    annually

4
History
  • Ancient disease
  • Writings from India describe similar illness as
    early as 6th century BC
  • Imported to Europe by troops of
    Alexander.European epidemic peaked in 13th
    century
  • French settlers took it to Canada and black
    slaves to America

5
  • ARMAUER HANSEN of Norway attributed it to
    M.leprae in 1873
  • Effective chemotherapy began with sulfones in
    1943
  • Rifampicin introduced in 1970
  • Grown in mouse footpad in 1961 in nine banded
    armadillo in 1971
  • Sequence of genome published in 2001

6
EPIDEMIOLOGY
  • Endemic in all continents except Antarctica
  • 2/3rd of worlds leprosy burden in Indian
    subcontinent
  • Incidence800,000 per year
  • Not always a tropical disease was endemic in
    norway till 20th century
  • IP2 to 5 yr for tuberculoid 8 to 12 yr for
    lepromatous cases

7
  • Loss of 1 million disability adjusted life years
  • Majority of cases before 35 yr age with median
    age less for tuberculoid cases
  • Excess of male cases regularly found
  • Clustering of cases well recognised
  • HLA association

8
  • HLA-DR2/DR3 occur at higher frequency in TT/BT
    patients
  • HLA-DQ1 increase susceptibility to BL/LL leprosy
  • In South India assoc. found between
    paucibacillary leprosy and 10p13 chromosome
  • Main source of infection- nasal discharge from
    untreated LL patients
  • Not excreted by skin

9
AETIOLOGY
  • Classified under order Actinomycetalis and family
    Mycobacteriacae
  • Straight/slightly curved rods with parallel sides
    and round ends
  • Gram ve, acid fast. Characteristically acid
    fastness lost with pyridine extraction
  • Obligate intracellular parasite especially in
    macrophages
  • Noncultivable.. Grows at 30-33 degree centigrade
  • Doubling time 12-13 days

10
  • Genome of M.leprae(3.27 megabases) shorter than
    M.tuberculosis(4.41 megabases)
  • ULTRASTRUCTURE
  • Following components from inside out
  • Cytoplasm
  • Trilaminar plasma membrane

11
  • Cell wall is 20 nm thick electron dense.
    Consists of peptidoglycan covalently attached to
    arabinogalactan polymer modified by branched
    chain mycolic acid. Lipoarabinomannan is another
    important component
  • Capsule is electron transparent due to copious
    amounts of lipid- phthiocerol dimycocerosate
    phenolic glycolipid

12
  • PGL-1 is species specific and immunogenic
  • Entry into nerves mediated by binding of PGL1 to
    laminin 2 in basal lamina of Schwann cells

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PATHOLOGIC SPECTRUM OF LEPROSY
  • Lepra bacilli is non toxic. Clinicopathologic
    manifestations are result of immunopathology
  • RIDLEY JOPLING ciassification defines categories
    along a spectrum depending on clinical,histopathol
    ogical, immunological indices
  • TT,BT,BB,BL,LL
  • TT and polar LL patients are stable while others
    move in either direction according to host
    response treatment

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PATHOLOGY
  • TUBERCULOID LEPROSYTuberculoid granulomas found
    which tend to collect in foci around
    neurovascular elements.
  • Each focus cosists of epitheloid cells at centre
    with surrounding zone of lymphocytes. Langhans
    giant cells sometimes found
  • Some of the foci invade papillary zone and even
    erode basal layer

19
  • Bacilli not seen
  • Dermal nerves are either completely destroyed or
    swollen by epithelioid cell granuloma.
    Occasionally there may be caseation within a nerve

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BORDERLINE LEPROSY
  • BT
  • Epithelioid cell granuloma more diffuse with a
    free but narrow papillary zone. Giant cells tend
    to be of foreign body type.
  • Dermal nerves are moderately swollen by cellular
    infiltration or schwann cell proliferation
  • Bacilli absent from dermis but likely to be found
    witin dermal nerves

23
  • Granulomas follow neurovascular bundles, sweat
    glands or erector pili muscles

24
BB
  • Diffuse epithlioid cell granuloma with scanty
    lymphocytes and no giant cells
  • Papillary zone is clear and dermal nerves show
    slight swelling with cellular infiltrate
  • Bacilli present within dermal nerves dermis in
    modest numbers

25
BL
  • There is macrophage granuloma in which some cells
    show slight foamy changes. Lymphocytes present in
    dense clumps or widely distributed in parts of
    granuloma. Occasionally epithelioid cells seen
  • Papillary zone is free
  • Bacilli are plentiful-singly or in clumps

26
  • Dermal nerves contain some cellular infiltrate
    perineurium may have laminated appearance(onion
    skin perineurium)

27
LEPROMATOUS LEPROSY
  • Extensive cellular infiltrate that is separated
    from flattened epidermis by Grenz zone of normal
    collagen
  • In early lesions macrophages have mixed
    population of solid and fragmented bacilli.Solid
    bacilli are packed like cigars. Bacilli may be
    seen in endothelial cells
  • No epithelioid cells. Lymphocytes not prominent.
    Plasma cells may be seen

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  • In time degenerated bacilli accumulate in
    macrophages so called virchow or lepra cells
    which have foamy cytoplasm. In chronic lesions
    bacilli are disposed in large basiophilic clumps
    called globi
  • Dermal nerves are well preserved contain large
    no of bacilli. Slowly become fibrotic

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

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

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Type 1 reaction
  • Edema within around granuloma
  • In upgrading reaction granulomas become more
    epithelioid langhans giant cells are larger.
    There may be erosion of granuloma into lower
    epidermis
  • Fibrinoid necrosis within granulomas or in dermal
    nerves

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  • Downgrading reaction necrosis less common.
    Density of bacilli increases
  • Multibacillary patients who upgrade on therapy
    show old foamy macrophages mixed with new
    epithelioid cell granulomas

38
Type2 reaction(ENL)
  • Lesions are foci of acute inflammation on chronic
    multibacillary leprosy. Neutrophils may be scanty
    or abundant to form abscess. Foamy macrophages
    with fragmented bacilli abundant

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Lucio reaction
  • Endothelial proliferation leading to luminal
    obliteration seen with thrombosis of medium sized
    vessels in dermis subcutis
  • Dense aggregates of AFB in walls of normal
    appearing vessels vessels with proliferative
    changes

41
Indeterminate leprosy
  • Mild lymphocyte macrophage accumulation around
    neurovascular bundles, sweat glands erector
    pili muscles. No epithelioid cell granulomas.
    Schwann cell hyperplasia may be seen
  • Diagnosis rests on finding 1/more AFB in sites of
    predilection- erector pili muscles, just beneath
    epidermis or around a vessel

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CLINICAL FEATURES
  • Indeterminate Leprosy Lesions found on face,
    extensor surface of limbs or buttocks.
  • Consist of one or more slightly hyperpigmented or
    erythematous machules with poorly defined
    margins.
  • Hair growth and nerve functions are normal.
  • Found usually in children whose immune status is
    yet to be determined. Smears are negative.
    Sometimes thickened nerve is palpable.

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POLAR TUBERCULOID
  • Primary lesion is a plaque assuming an annular
    configuration. Both the borders are sharply
    marginated.
  • Lesion is indurated, elevated, scaly, dry,
    hairless and hypopigmented.
  • Nearby sensory nerve may or may not be enlarged
    but the lesion is anaesthetic anhidrotic.
  • Usually solitary.

46
  • Spontaneous cure is known.
  • Upper limit of 10 cm on lesion size.

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BORDERLINE TUBERCULOID
  • Primary lesion are plaques and papules.
  • Annular configuration is common with both borders
    sharply defined but incomplete annular lesions
    may be seen. Satellite lesions present.
  • Little scaling, less erythema, less induration,
    size more than 10 cm.
  • Multiple asymmetric lesions are a rule.
  • Loss of sensation in skin lesion, nerve trunk
    enlargement and palsies seen.

50
  • Nerve trunk involvement asymmetric and not more
    than 2.
  • Nerve abscesses are most often seen in males with
    BT disease.

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MID BORDERLINE
  • Immunological midpoint
  • Characteristic lesions are annular with sharply
    marginated interior and exterior margins or large
    plaques with islands of normal skin within the
    plaque giving a Swiss cheese appearance or a
    classic dimorphic lesion.
  • Because of instability BB is shortlived and
    rarely seen.

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BORDERLINE LEPROMATOUS
  • Highly variable in clinical expression
  • In 1/3rd patients classic dimorphic lesion seen.
    Annular lesions with both borders sharply defined
    uncommon but when they occur lepromatous like
    papules nodules seen.
  • Lesions may be hypoaesthetic/anaesthetic but not
    necessarily so.
  • Nerve trunk palsies have highest prevalence in
    BL.

58
  • Untreated BL has a relentless progression. When
    disease is extensive acral distal symmetric
    anaesthesia may be present.
  • Course altered by reactional states.

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LEPROMATOUS LEPROSY
  • Poorly defined skin coloured nodules are the most
    characteristic symmetrically distributed.
  • Diffuse dermal infiltration is always present,
    manifested by widening of nasal root and fusiform
    swelling of fingers.
  • Skin over forehead thickened causing Leonine
    facies. Earlobes thickened.
  • Ciliary or superciliary madarosis

63
  • Macules are small, numerous, erythematous, vague
    and shiny with no loss of sensation or hair
    growth. Papules may also be present
  • Sometimes, all 3 lesions may be present
  • Chronic edema of leg is usual
  • Nerve trunk palsies less common than in BL. Acral
    distal symmetric(glove stocking) anaesthesia
    seen

64
  • Systemic associations
  • Nose may collapse with septal perforation, voice
    may become hoarse, upper incisor teeth may loosen
    and fall off
  • Shortening of fingers and toes due to repeated
    trauma
  • Anaesthetic skin liable to blister and ulceration
  • Eyes- superficial punctate keratitis, insidious
    iritis (beginning as iris pearls)

65
  • Damage to cranial nerve V VII- corneal
    insensitivity and lag ophthalmos predispose
    cornea to injuries
  • Bone changes occur late- periostitis of bones of
    leg and forearms, atrophy of distal phalanges of
    hands atrophy of phalanges, metatarsal tarsal
    bones of feet
  • In skull atrophy of anterior nasal spine and of
    maxillary alveolar process lead to facies leprosa

66
  • Kidney- renal damage important cause of morbidity
    mortality (renal amyloidosis,
    glomerulonephritis, etc)

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PREGNANCY LEPROSY
  • 1. Worsening of leprosy status (third trimester
    of pregnancy)
  • 2. Type I reaction (during first six months of
    lactation)
  • 3. Type II reaction (in third trimester first
    six months of lactation)
  • 50 suffer deterioration of nerve function

73
LUCIO LEPROSY
  • Diffuse non nodular type. Described by Lucio and
    Alvarado in Mexico.
  • hands, wide spread sensory loss. Shiny thickened
    skin, body hair loss, puffy
  • Eyes- shiny appearence
  • Anaemia, edema, ulceration of both legs
  • Nasal symptoms present
  • No motor palsies and eyes not damaged.

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HISTOID HANSENS
  • Term introduced by Wade
  • Applied to firm erythematous round shiny
    glistering nodules which appear on skin of
    patients whose disease is relapsing either
    because they have stopped treatment or because M.
    leprae has become drug resistant
  • Histoid nodules contain elongated spindle shaped
    histiocytes with bacilli within them

76
TYPE I LEPRA REACTION
  • Delayed (type IV) hypersensitivity reaction
  • Antigens from breaking down of lepra bacilli
    react with T-lymphocytes
  • Seen in borderline patients
  • Increase in CMI associated with treatment known
    as reversal reaction whereas reduction in
    immunity called downgrading reaction

77
  • Upgrading most common during first six months of
    treatment
  • LLs-gtBL-gtBB-gtBT-gtTTs
  • Rapidly developing change in some/all skin
    lesions- become erythematous, shiny, warm and
    tender. Sometimes necrosis occurs leading to
    ulceration. Lesions desquamate as the subside

78
  • Systemic features less common
  • Nerve involvement- swelling of one or more nerves
    with pain and tenderness
  • Most serious complication is motor disturbance
    and nerves at risk are ulnar, lateral popliteal
    and facial
  • Paralysis can recover with prompt treatment

79
  • Edema of hands, feet or face
  • Tenderness of palms and soles

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TYPE II LEPRA REACTION
  • Is an immune complex syndrome (type III)
    hypersensitivity reaction
  • Occurs almost always LLp/LLs and occasionally in
    BL
  • No change of leprosy lesions but crops of
    brightly red nodules seen which come and go
  • Systemic disturbance usual
  • Unusual for it to occur during first six months
    of treatment

83
  • ENL lesions tend to be bilaterally symmetrical.
    Often tender and blanch with light pressure.
    Evanescent lasting two-three days
  • Commonly on face, arms and thighs. Leave bluish
    stain as they fade
  • In severe reactions lesions may become vesicular
    and break down (erythema necroticans)
  • Other features- neuritis, myositis, arthritis,
    rhinitis, acute iritis, dactylitis,
    epididymoorchitis and proteinuria

84
  • Factors precipitating ENL
  • Physical/ mental stress
  • Immunization
  • Intercurrent infection/injury/surgery
  • Pregnancy/parturition
  • Ingestion of KI besides anti-leprosy drugs

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LUCIO PHENOMENON
  • Confined to diffuse non-nodular LL leprosy
    chiefly encountered in Mexicans
  • Unique feature- seen only in untreated patients
  • Painful red patches appear on the skin
    especially extremities, become purpuric, ulcerate
    and finally develop an eschar which falls off to
    leave a superficial atrophic scar
  • Face and trunk are spared. Patients afebrile

88
  • Steroids- show good effect
  • Thalidomide of no value
  • Good results with Dapsone
  • Excellent results with Rifampicin

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DIFFERENTIAL DIAGNOSIS
  • I. Neurological conditions
  • PALPABLE N. THICKENING WITHOUT
    ANAESTHESIA/SIGNS OF N. DAMAGE
  • 1. Excessive muscular development
  • 2. Pachydermoperiostitis

91
  • PALPABLE N. THICKENING WITH REGIONAL
    ANAESTHESIA WITH/WITHOUT MUSCLE WASTING
  • Primary amyloidosis of peripheral nerves
  • Familial hypertrophic interstitial neuritis

92
  • REGIONAL ANAESTHESIA WITH/WITHOUT MUSCLE
    WASTING BUT WITH NERVE THICKENING
  • Recurrent/chronic progressive polyneuritis
  • Peroneal muscular atrophy (charcot-marie-tooth
    syndrome)

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  • REGIONAL ANAESTHESIA WITH/WITHOUT MUSCLE
    WASTING BUT WITHOUT NERVE THICKENING
  • Syringomyelia
  • Tabes dorsalis
  • Peripheral neuropathies
  • Hereditary sensory radicular neuropathy
  • Congenital indifference to pain

94
  • II. DERMATOLOGICAL CONDITIONS
  • None of the conditions listed below have all
    the three features of sensory loss, nerve
    thickening and ve skin smear for AFB
  • Flat and hypopigmented lesions
  • Morphoea
  • Onchocerciasis
  • Pityriasis alba
  • Tenia versicolor

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  • 5. Post kala azar dermal leishmaniasis
  • 6. Yaws
  • 7. Vitiligo
  • B. Raised and pigmented lesions
  • Follicular mucinosis (alopecia mucinosa)
  • Granuloma annulare
  • Granuloma multiforme
  • Gyrate erythemas
  • Kaposis sarcoma
  • Cutaneous leishmaniasis
  • Lupus erythematosus

96
  • 8. Lupus vulgaris
  • 9. Mycobacterium marinum infection
  • 10. Mycosis fungoides
  • 11. Neurofibromatosis
  • 12. P.rosea
  • 13. Tinea corporis
  • c. Generalized thickening of skin
  • Systemic sclerosis
  • Myxoedema
  • Pachydermoperiostosis

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CONCLUSION
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THANK YOU
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