leprocy

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By: drvivekshimla (19 month(s) ago)

Hello Doctor, Nice presentation indeed!. Can I download?

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LEPROSY : 

By. Dr. Deepak Kumar Talreja LEPROSY

DEFINITION : 

DEFINITION A chronic granulomatous disease Caused by Mycobacterium leprae Affecting peripheral nerves and skin.

BACKGROUND : 

BACKGROUND Armauer Hansen Discovered M leprae in Norway in 1873 First bacillus to be Associated with human disease Humans Primary reservoir Animal reservoirs 9-banded armadillos Chimpanzees Mangabey monkeys Every year January 27 is World Leprosy Day

GEOGRAPHICAL DISTRIBUTION : 

GEOGRAPHICAL DISTRIBUTION Endemic in Asia Africa Latin America India 70% of the world’s leprosy cases

EPIDEMIOLOGY : 

EPIDEMIOLOGY Incidence Around 800 000 Prevalence 4 million people Age 10-14 years 35-44 years Sex In adults (lepromatous type) Male-to-female ratio of 2:1 In children (tuberculoid form)

PATHOGENESIS : 

PATHOGENESIS M leprae Intracellular Acid-fast Gram-positive Bacillus Transmission Aerosol Spread Abraded Skin Incubation Time Tuberculoid Cases 2–5 Years Lepromatous Cases 8–12 Years

PATHOGENESIS : 

PATHOGENESIS (HLA) associations Tuberculoid HLA-DR2 and HLADR3 Lepromatous HLA-DQ1 Susceptible loci on chromosome10p13 and chromosome 6 Prefer cool parts Superficial peripheral nerves Skin Mucous membranes of U.R.T Anterior chamber of the eyes Testes

PATHOGENESIS : 

PATHOGENESIS

PATHOGENESIS : 

PATHOGENESIS 4 principle causes of tissue damage The degree to which CMI is expressed The extent of bacillary spread & multiplication Lepra reactions The nerve damage & its complications

PATHOGENESIS : 

PATHOGENESIS

CLINICAL SPECTRUM : 

CLINICAL SPECTRUM

HISTOLOGY TUBERCULOID LEPROSY (TT) : 

HISTOLOGY TUBERCULOID LEPROSY (TT) Granulomas Surround N.V. elements in foci Papillary zone may be invaded Bacilli not visible Cutaneous nerves swollen

HISTOLOGY TUBERCULOID LEPROSY (TT) : 

HISTOLOGY TUBERCULOID LEPROSY (TT)

HISTOLOGY TUBERCULOID LEPROSY (TT) : 

HISTOLOGY TUBERCULOID LEPROSY (TT)

HISTOLOGY TUBERCULOID LEPROSY (TT) : 

HISTOLOGY TUBERCULOID LEPROSY (TT)

HISTOLOGY BORDERLINE LEPROSY : 

HISTOLOGY BORDERLINE LEPROSY In BT Granuloma is more diffuse Fine but narrow papillary zone FB giant cells Dermal nerves moderately swollen AFB’s absent or scanty In mid BB Lymphocytes scanty No giant cells Nerves slightly swollen AFB’s in moderate number

HISTOLOGY BORDERLINE LEPROSY : 

HISTOLOGY BORDERLINE LEPROSY In BL Macrophages slightly foamy Occasional epitheloid cells Scanty cellular infiltrate Clear papillary zone Leprosy bacilli plentiful, Singly or in clumps

HISTOLOGY LEPROMATOUS LEPROSY (LL) : 

HISTOLOGY LEPROMATOUS LEPROSY (LL) Epidermis thin Rete ridges flattened Papillary layer appear as a clear band Typical diffuse leproma Lymphocytes & plasma cells few Enormous no. of AFB’s Singly In clumps Globi

HISTOLOGY LEPROMATOUS LEPROSY (LL) : 

HISTOLOGY LEPROMATOUS LEPROSY (LL)

HISTOLOGY LEPROMATOUS LEPROSY (LL) : 

HISTOLOGY LEPROMATOUS LEPROSY (LL)

HISTOLOGY LEPROMATOUS LEPROSY (LL) : 

HISTOLOGY LEPROMATOUS LEPROSY (LL)

HISTOLOGY LEPROMATOUS LEPROSY (LL) : 

HISTOLOGY LEPROMATOUS LEPROSY (LL)

HISTOLOGY INDETERMINATE LEPROSY : 

HISTOLOGY INDETERMINATE LEPROSY Scattered infiltrate Histiocytic lymphocytic Rarely single bacillus In a dermal nerve

CLINICAL FEATURES : 

CLINICAL FEATURES Early lesions Classic lesion is that of IL Macules (character) One or more Few cms. in diameter Margins poorly defined Sites Face Extensor surface (limbs) Alternatively, features of One of est. forms

CLINICAL FEATURESTUBERCULOID LEPROSY (TT) : 

CLINICAL FEATURESTUBERCULOID LEPROSY (TT) Few lesions often solitary Typically a plaque Copper coloured or purple Edges raised, slope towards centre Surface Dry Hairless Insensitive Sometimes purely neural Pain & swelling of the affected nerve Anaesthesia &/or muscle weakness & wasting

CLINICAL FEATURES TUBERCULOID LEPROSY (TT) : 

CLINICAL FEATURES TUBERCULOID LEPROSY (TT)

CLINICAL FEATURESLEPROMATOUS LEPROSY (LL) : 

CLINICAL FEATURESLEPROMATOUS LEPROSY (LL) Early symptoms Dermal Macules, Diffuse papules Nodules Or all three Nasal Stuffiness Discharge Epistaxis Oedema of legs

CLINICAL FEATURESLEPROMATOUS LEPROSY (LL) : 

CLINICAL FEATURESLEPROMATOUS LEPROSY (LL) Skin lesions Multiple Bilaterally symmetrical Sites Face Arms Legs Spared areas Scalp Axillae Groins Perineum

CLINICAL FEATURESLEPROMATOUS LEPROSY (LL) : 

CLINICAL FEATURESLEPROMATOUS LEPROSY (LL) Oral lesions Papules on lips Nodules on Palate Uvula Tongue Gums

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY Untreated advanced disease Thickened facial skin “Leonine facies” Thickened ear lobes Collapsed nose Loss of eye brows Hoarseness Fall of upper incisor teeth Nerve involvement Icthyosis

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY Nerve involvement Skin manifestations antedate nerve damage Thickening of nerves Sensory or motor dysfunction Blistering of anaesthetic skin

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY Eye changes 2 means Leprous deposits Keratitis Iridocyclitis (uveitis) Iris atrophy Lagophthalmos Exposure keratitis Blindness

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY Bone changes Osteoporosis in the phalanges Small osteolytic cysts Compression fractures Crooked or short fingers Nails Thin & brittle Testicular atrophy

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY LUCIO LEPROSY A pure diffuse type of LL Described in 1852 (Mexico) Initial symptoms Sensory impairment Hands and feet Gradual loss of Eyebrows Eyelashes Body hair. Diffuse scleroderma like skin Absent nodules & plaques

CLINICAL FEATURES LEPROMATOUS LEPROSY : 

CLINICAL FEATURES LEPROMATOUS LEPROSY HISTOID LESIONS Distinct cutaneous nodules Characteristic of relapse after treatment

CLINICAL FEATURES BORDER LINE LEPROSY : 

CLINICAL FEATURES BORDER LINE LEPROSY Commonest type Lesions Macules Plaques Annular Bizarre-shaped bands Intermediate in number Asymmetrical distribution In the middle of the spectrum Characteristic plaques with a “punched out” appearance

CLINICAL FEATURES BORDER LINE LEPROSY : 

CLINICAL FEATURES BORDER LINE LEPROSY DIMORPHIC

CLINICAL FEATURES BORDER LINE LEPROSY : 

CLINICAL FEATURES BORDER LINE LEPROSY Tuberculoid end of the spectrum Lesions few and dry More hair loss & anhidrosis Fewer bacilli in smears & biopsies And vice versa towards the lepromatous pole One or more nerves thickened Border line disease is unstable

CLINICAL FEATURES BORDERLINE TUBERCULOID : 

CLINICAL FEATURES BORDERLINE TUBERCULOID Tuberculoid leprosy

CLINICAL FEATURES BORDERLINE LEPROMATOUS : 

CLINICAL FEATURES BORDERLINE LEPROMATOUS Borderline tuberculoid

CLINICAL FEATURES PURE NEURITIC LEPROSY : 

CLINICAL FEATURES PURE NEURITIC LEPROSY Accounts for 5-10% of patients Seen in India & Nepal Peripheral nerve lesions Asymmetrical No visible skin lesions

REACTIONS : 

REACTIONS Type I reactions Occur in border line disease Seen after starting treatment Cell mediated Presents with Acute neuritis Acutely inflamed skin lesions Nerves Tender Loss of function Sensory & motor New lesions may appear Can occur spontaneously

REACTIONS : 

REACTIONS Type II ENL reactions A systemic disorder Antibody mediated Occur in MB disease (LL & BL) Spontaneous On treatment No change in existing lesions Presents Fever Painful red nodules Face & limbs Superficial or deep Ulceration

LUCIO REACTIONS : 

LUCIO REACTIONS Occurs only in lucio leprosy Untreated pts presents Severe systemic upset Can be fatal Erythematous patches Bullae & necrosis Deep painful ulcers

PROGNOSIS : 

PROGNOSIS Nerve damage Cannot be reversed Severe disability Esp. When all 4 limbs & both eyes are affected Anti-bacterial treatment Highly effective Low relapse rates

DIAGNOSIS : 

DIAGNOSIS Usually clinical (on the basis of 2 out of 3) Anaesthesia of a skin lesion Thickened nerves Typical skin lesions Slit skin smears Bacteriological index Morphological index Skin biopsy Nerve biopsy

BACTERIOLOGICAL INDEX (BI) : 

BACTERIOLOGICAL INDEX (BI)

MORPHOLOGICAL INDEX : 

MORPHOLOGICAL INDEX Percentage of solid staining bacteria

DIAGNOSIS : 

DIAGNOSIS Lepromin skin test Analogous to the Tuberculin test Positive at 48 hours = Fernandez reaction Delayed hypersensitivity Positive again at 3 - 4 weeks = Mitsuda reaction Granulomatous response Strongly positive in TT Weakly positive in BT Negative in BB, BL and LL Unpredictable in indeterminate leprosy

DIFFERENTIAL DIAGNOSES : 

DIFFERENTIAL DIAGNOSES Macular lesions Birth marks Vitiligo Pityriasis Alba Pityriasis Versicolor Tinea Corporis Post inflammatory hypochromia

DIFFERENTIAL DIAGNOSESMacular lesions : 

DIFFERENTIAL DIAGNOSESMacular lesions Birthmark Single or few in number Present from birth Unchanging Normal sweating and sensation

DIFFERENTIAL DIAGNOSESMacular lesions : 

DIFFERENTIAL DIAGNOSESMacular lesions Post-inflammatory hypochromia Hypochromia at site of inflamation Take a history examine for loss of sensation

DIFFERENTIAL DIAGNOSESMacular lesions : 

DIFFERENTIAL DIAGNOSESMacular lesions Tinea versicolor Well-defined, scaly lesions Over the trunk, neck and limbs Sensation and sweating normal Fungal elements seen under microscope

DIFFERENTIAL DIAGNOSESMacular lesions : 

DIFFERENTIAL DIAGNOSESMacular lesions Tinea corporis Prominent, scaly lesion Respond to antifungal Sensation and sweating normal

DIFFERENTIAL DIAGNOSESMacular lesions : 

DIFFERENTIAL DIAGNOSESMacular lesions Vitiligo White lesions (de-pigmentation) Sensation, sweating are normal

DIFFERENTIAL DIAGNOSES : 

DIFFERENTIAL DIAGNOSES Plaques & annular lesions Ring worm Granuloma Multiforme Sarcoidosis Cutaneous TB Granuloma annulare

DIFFERENTIAL DIAGNOSES Plaques & annular : 

DIFFERENTIAL DIAGNOSES Plaques & annular Granuloma annulare Affects mainly children and young adults Papules or nodules in a annular pattern Symptomless No enlarged peripheral nerves Sensation and sweating are normal

DIFFERENTIAL DIAGNOSES Plaques & annular : 

DIFFERENTIAL DIAGNOSES Plaques & annular Granuloma multiforme Occurs mainly in nigeria Variant of granuloma annulare Initialy itching Disappear spontaneously Sensation, sweating and peripheral nerves –all normal

DIFFERENTIAL DIAGNOSES Plaques & annular : 

DIFFERENTIAL DIAGNOSES Plaques & annular Sarcoidosis Sensation normal No enlargement of Nerves

DIFFERENTIAL DIAGNOSES Plaques & annular : 

DIFFERENTIAL DIAGNOSES Plaques & annular Lupus Vulgaris Tendency to ulceration and scar formation Nerves are not involved Sensation is normal

DIFFERENTIAL DIAGNOSES : 

DIFFERENTIAL DIAGNOSES Nodules Cutaneous Leishmaniasis Post-kala-azar dermal leishmaniasis (PKDL)

DIFFERENTIAL DIAGNOSES : 

DIFFERENTIAL DIAGNOSES Nerves Hereditary sensory motor neuropathy type III Amyloidosis Peroneal muscular atrophy (CMTD) Causes of other polyneuropathies AIDS DM Alcoholism Heavy metal poisoning

DIFFERENTIAL DIAGNOSES : 

DIFFERENTIAL DIAGNOSES Eye disease Trachoma Onchocerciasis which causes uveitis

TREATMENT : 

TREATMENT 5 main principles of treatment Stop the infection with chemotherapy Treat reactions & reduce risk of N. damage Educate the pt. to cope with existing N. dam. Treat the complications of N. damage Rehabilitation Social Psychological

TREATMENT : 

TREATMENT WHO multidrug therapy regimen

TREATMENT : 

TREATMENT Relapse Low relapse rates Relapsed MB pts. treated with triple therapy

REACTION & NEURITIS : 

REACTION & NEURITIS Peak time for reversal reactions First 6 months Patient warning important Treatment aim Controlling acute inflammation Easing pain Reversing nerve & eye damage Reassuring the patient MDR continued

TREATMENT : 

TREATMENT Lepra Type 1 Reactions Prednisolone 40-60 mg/d Taper in 2- to 3-month period Indications for prednisone Neuritis Lesions appearing at face Ulcerating lesions

TREATMENT : 

TREATMENT Lepra Type 2 Reactions ENL High dose steroids 80 mg/d, Rapid tapering Recurrent ENL Thalidomide 400 mg daily Clofazimine 300 mg daily Acute Iridocyclitis Hydrocortisone 1% eye drops, 4 hrly Atropine 1% eye drops, 12 hrly

COMPLICATIONS OF NERVE DAMAGE : 

COMPLICATIONS OF NERVE DAMAGE Preventable Early diagnosis Self awareness of the patient Appropriate shoe protection Special shoe for deformities Rest in case of plantar ulceration Weakness & Paralysis Physiotherapy

VACCINES AGAINST LEPROSY : 

VACCINES AGAINST LEPROSY Cross-reactivity b/w BCG & M. Leprae BCG agianst leprosy Statistically significant but variable protection

THANK YOU : 

THANK YOU