LEPROSY

Hansen`s Disease

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it is the oldest disease in history and still worldwide sociomedical and economic problem, chronic infectious diseaseas it can lead to deformities and can leave a crippled patient burdens on his family and his society .

It principally affects the skin and peripheral nerves.

Leprosy (Hansen’s disease)

Epidemiology It is endemic in tropical and subtropical

countries .It is common and endemic in certain area in upper

Egypt e .g Assiut & Giza In lower Egypt e.g Sharkeia & Gharbeia .

M. leprae is discovered by Hansen from Norway in 1873

Aetiology

-Mycobacterium leprae .-discovered by Hansen in Norway 1873 -It is can not be cultured in artificial media .- it can be growen in mouse footpad and armadillo .

BACTERILOGY (cont.)

. M . Leprae are straight or slightly curved rod like bacteria

They look like M. tuberculosis stained with Ziehl – Neelsan stain . But are less acid fast and alcohol fast .

bacteriological index is the density of M leprae in smear it includes living and dead

bacilli . morphological index.is percentage of living bacilli it indicates if patient is infectious or not and response to treatment

Mode of transmission droplet infection is main mode of infection There is personal susceptibility which decides if a contact will be

infected or not The majority of human beings have no susceptibiity

Contact through the skin (rare). Arthropod-born infection (rare). Through placenta and milk.

Leprosy is not STD or directly inherited.

Predisposing or risk factors

Age : in all age groups .Peak of onset is 10-20 years Children are more susceptible

Sex : male to female ratio in adult was 2 : 1 last years it is going to be equal .

1.Residence in an endemic area.2.Having a blood relative with leprosy.3.Poverty (malnutrition).4.Contact with affected armadillo.

pathogenesis

ClassificationBased on the clinical, ibacteriologic,immunologic and histopathologicfeaturesleprosy is classified into maintypes

1.Paucibacillaryexample: (Tuberculoidleprosy) (TL) (with scanty or absent bacilli) -Skin lesions, loss of

sensation.2.Multibacillary(Border line) (with numerous bacilli)---

numerous skin lesions, loss of sensation, can go to3.Multibacillary(lepromatousleprosy) (LL).Nodules and

plaques, thickened dermis, loss of sensation, neuronal damage, nasal congestion, epistaxis.

Classification & Clinical Presentation

Jopling Classification

Based on Host Immunity

TT BL LL

BT BB BL

Classification & Clinical Classification & Clinical PresentationPresentation

WHO Classification

Based on Bacterial Load

Paucibacillary Multibacillary

Slit Skin Smear

Positive Negative

LEPROSY

Paucibacillary (PB) Multibacillary (MB)

Indeterminate Leprosy (IL)

Tuberculoid Leprosy (TL)

Borderline Tuberculoid (BT)

Borderline Borderline (BB)

Borderline Lepromatous(BL)

Lepromatous Leprosy (LL)

CLINICAL PICTURE

Indeterminate Leprosy

Tuberculoid Leprosy

Borderline Leprosy

BT BB BL

Lepromatous Leprosy

Indeterminate Leprosy (IL)

Usually single (multiple) macule / patche. Hypopigmented or faintly erythematous. Sensation normal but sometimes imparied. The peripheral nerves normal. Slit skin smear negative.

Indeterminate leprosy :Hypopigmented patch, sensation normal, no palpable peripheral nerve and slit skin smear negative.

Tuberculoid Leprosy (TL)

Usually single but may be few (<5). Hypopigmented / erythematous plaque. Well defined borders. Sensation markedly imparied. Enlarged peripheral nerve. Slit skin smear negative

Tuberculoid leprosy: Two hypopigmented patches, hypoastheticwell defined borders, palpable peripheral nerve and SSS negative.

Tuberculoid Leprosy: Annular, erythematous, anasthetic patch with well defined and raised borders and SSS Negative.

Borderline Leprosy (BL)(BT,BB,BL)

Few / many asymmetrical patches. Partly well-defined borders. Sensory impairments range from slight to marked. Slit skin smear usually positive. P. nerves asymmetrically enlarged. Annular plaque in leprosy are most commonly border line with

characteristic punched out internal border and sloping outer border(swiss cheese appearance)

Borderline Tuberculoid Leprosy: Well-defined large anaesthetic patcheswith satellite lesions. SSS Negative.

Borderline Borderline Leprosy: Less defined, asymmetrically distributed hypoaesthetic patches. SSS positive.

Borderline Lepromatous Leprosy: Numerous, hypoaesthetic almost symmetrically distributed patches . SSS positive.

Lepromatous Leprosy (LL)

Very numerous ill defined lesions. (macules, patches, papules,and nodules). Symmetrically distributed allover the body Loss of eyebrows and eyelashes. Leonina facies. No sensory impairments in lesions . Peripheral nerves symmetrically enlarged. Slit skin smear always positive.

Lepromatous Leprosy: Leonine Face

Mucous membrane involvement

Very common Usually epistaxisDryness ,rhinorrhea

and obstruction of Nose

Nerve involvementBilateral symmetrical

thickening of all nerves

Nerve fibrosis is very late in lepromatous leprosy, so loss of

sensation is also very late.Bilateral Glove and

stocking anasthesia is present in all advanced cases

of lepromatous leprosy.

Diagnosis of Leprosy

1. Clinical Examination.2. Slit Skin Smear.3. Skin Biopsy.

1.Clinical examination: What are the cardinal skin signs of leprosy?

1. Hypopigmented or erythematus patch / plaque

2. Complete / partial loss of sensation.3. Thickening of peripheral nerves.

1 .History

Familial or extrafamilial contactParasthesiaNumbnessEpistaxis

2 .Clinical examination

.I1 (E, Temperature & painxamine all modalities of sensation in the skin lesion “Touch”

.II2 (Examine peripheral nerves for Tenderness, consistency and size:

.IIIHead: Supraorbital

.IVNeck: Great auricular n..VElbows: Ulnar n.

.VIWrists: Superficial radial cut. And median n..VIIPopliteal fossa: Common peroneal n..VIIIThey may be cord-like, beaded, thinned or may show

abscess formation .

3 .Investigations

(1 )Nicotinic acid test:“Professor Dr. El-Arini”

15 mg nicotinic acid IM injection Hypopigmented macule becomes erythematous within 10-15 mins.

*Value:Quick office test for early detection of hypopigmented lesions of leprosy.

(2 )Bacteriological examination :

Nasal smearNot recommended) ? (:

Painful for the patient & may reveal +ve results in contacts

On treatment, lepra bacilli disappear from nasal mucus earlier than from skin.

It’s very difficult to prove the bacilli ara M. Leprae

2.Slit Skin Smear Simple and valuable test. It is needed for diagnosis. Monitor the progress of the treatment.

Slit Skin Smear (method).

Pinch the site tight. Incise. Scrape & collect material Smear on a slide. Air dry & fix. Stain (Z-N method)

Slit Skin Smear (site). Ear lobe. Forehead. Gluteal region. Active edge of patch.

Slit Skin Smear (Reporting the smear).

Bacteriological indexBacteriological index00 – – no bacilli in 100 fieldsno bacilli in 100 fields

11 :+ :+1-101-10 bacilli in 100 fieldsbacilli in 100 fields22 :+ :+1-101-10 bacilli in 10 fieldsbacilli in 10 fields

33 :+ :+1-101-10 bacilli in 1 fieldbacilli in 1 field44 :+ :+10-10010-100 bacilli in 1 fieldbacilli in 1 field

55 :+ :+100-1000100-1000 in 1 fieldin 1 field66> :+> :+10001000 bacilli field )globi(bacilli field )globi(..

Morphological indexMorphological indexThe percentage of living bacilli to the total number of bacilli in the smear.

3 . Skin biopsy- TO verify the diagnosis & classification - The biopsy must be deep enough to include dermis

It’s a sudden change in the clinical picture of the disease because of conflict between the bacilli and the immune system of the host.

What are the precipitating factors ?

1.1. Effective treatment.Effective treatment.2.2. Intercurrent infection.Intercurrent infection.3.3. Physical stress.Physical stress.4.4. Surgical operation.Surgical operation.5.5. Pregnancy.Pregnancy.6.6. Sometimes Sometimes

spontaneously.spontaneously.

LEPROSY REACTIONLEPROSY REACTION

TYPES OF LEPRA REACTIONS

Type I•Change in host CMI •Seen in borderlines•Skin and nerve lesions

Type II•Antigen antibody•Seen in LL & BL leprosy•Skin, nerve & systemic involvement

Type I Lepra Reaction(Reversal Reaction)

Seen in BT, BB & BL. Sudden onset. Eythematous & odematous changes in old

lesions. Appearing of new lesions. Tenderness & swelling of peripheral nerves.

Treatment of type I Reaction:1. Continue MDT.2. NSAID.3. Systemic corticosteroid.

Type II Lepra Reaction (ENL) Acute onset of constitutional symptoms. Appearance of ENL-like skin lesions. Visceral manifestations includes :- Iridocyclitis, hepato-splenomegaly, epididmo-orchitis, nephritis, pleuritis, lymphadenitis & neuritis. Treatment of type II Reaction:1. Continue MDT.2. NSAID3. Thalidoamide ( clofazimine, corticosteroid )

Lucio phenomena

Unusual reaction seen exclusively in patien from Mexico

Having diffusue leprometus form of leprometus leprosy ,who left untreated

•Patient develop recurrent crops of large and sharply marginal ulcerative lesion (lower extremities)

COMLICATIONS OF LEPROSY

COMLICATIONS OF LEPROSY

1. Reactions.2. Complications of peripheral nerves.3. Complications of eyes4. Complication of bones

COMPLICATIONS OF PERIPHERAL NERVES

Peripheral nerves

Sensory Motor Autonomic

Hypoaestesia / anaestesia Muscle paralysis Lack of sweating & sebum

Ulcers Ulnar nerve Claw handRadial nerve Wrist dropLt. popliteal Foot dropPost. tibial Claw toesFacial lagophthalmous

Dry skinCracked skin

Ulcers

COMPLICATIONS OF EYE

Involvment of the ophthalmic division of the (5th.) trigeminal nerve

Corneal sensation imparment

Patients ignore injuries

keratitis, conjunctivitis and ulcers

Involvment of zygomatic & temporal braches of the (7th.) facial nerve .

Lagophthalmos

Unable to close the eye (unbliking stare)

Complications Of Bones

Bone damage in Leprosy is confined to bones of hand , feet & skull.

In the skull two pathognomonic changes occurs

1 -Atrophy of anterior nasal spine.

Nasal collapse

2 -Atrophy of maxillary alveolar process.

Loss of upper central incisors

These two skull changes known as “facies leprosa ”

TREATMENT

LEPROSY IS A CURABLE DISEASE

Leprosy treatment is simple, & the drugs are supplied in backs

All you have to do is decide which course of treatment the patient needs and make

sure that he take it regularly.

Drugs used in Leprosy treatmentWhat are the three commonly used drugs?

1. Dapson.2. Rifampicine.3. Clofazimine.

The combination of these three drugs is known as Multi Drug Therapy )MDT(

Rifampicin is highly bactericidal 99.999% of bacilli will be killed within 3 monthly doses.

Dapsone & clofazimine are weekly bactericidal, but in combination will

kill 99.999% of bacilli within 3 months.

MDT )Chemotherapy( renders Leprosy patients non-infectious.

MDT for PB leprosy6 months

Monthly dose Rifampicin 600mgDapsone 100 mg

Daily doseDapsone 100 mg

Multidrug Therapy )MDT( for Paucibacillary Leprosy )PB(

MDT for MB leprosy24 months

Monthly doseRifampicin 600mgClofazimine 300 mgDapsone 100 mg

Daily doseDapsone 100mg Clofazimine 50 mg

Multidrug Therapy )MDT( for Multibacillary Leprosy )MB(

Multi Drug Multi Drug TherapyTherapy

24 months

6 months

“

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Prophylaxis

1 -BCG Vaccine : it has protective effect as it can help until a vaccine becomes available.

3 -small dose of Dapsone can be used as prophylactic for contacts at risk , though it is liable to give chance for dapsone resistance .

But now it is the only available prophylactic measure for contacts at risk.

2- Armadillo Derived Vaccine )heat killed M.lepra( + BCG and vaccines derived from cultivable mycobacteria are now under trials .

thanks