UNIVERSITY OF MEDICAL SCIENCES AND TECHNOLOGY

LEPROSY(Hansen's disease)

Presented by;Dr. AlteibYousif

Dec 2012

12/16/2012 9:55 AMDr. Alteib

CONTENT

History and background

Key facts

Types of leprosy

Risk factors

Signs and symptoms

Complications

Diagnosis

Treatment and prevention

Global current situation

Situation in Sudan

Elimination of leprosy

12/16/2012 9:55 AMDr. Alteib

HISTORY & BACKGROUND

Leprosy was recognized

in the ancient civilizations of

China, Egypt and India.

The earliest documented

account of leprosy is around 1550

B.C on Egyptian papyrus.

Throughout history, the badly

affected have often been hated

by their communities and families (stigmatization).

12/16/2012 9:55 AMDr. Alteib

Con.. Although leprosy was

treated differently in the

past, the first advance in

treatment occurred in the

1940s with development

of the drug Dapsone,

which arrested the disease.

But duration of the treatment

was many years, even a lifetime, making it difficult for

patients to follow.

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Con..

In the 1960s, M. leprae started to develop resistance to Dapsone, the world’s only known anti-leprosy drug at that time.

In the early 1960s, Rifampicin and Clofazimine, the other two components of recommended multidrug therapy (MDT), were discovered.

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Con..

In 1981, WHO Study Group recommended MDT.

Since 1995, WHO provides free MDT for all patients in the world, initially through the drug fund provided by the Nippon Foundation and since 2000, through the MDT donation provided by Novartis and the Novartis Foundation for Sustainable Development.

12/16/2012 9:55 AMDr. Alteib

KEY FACTS

Leprosy is a chronic infectious disease caused by an acid-fast, rod-shaped bacillus, Mycobacterium leprae.

Official figures show that almost 182 000 people, mainly in Asia and Africa, were affected at the beginning of 2012, with approximately 219 000 new cases reported during 2011.

12/16/2012 9:55 AMDr. Alteib

Con..

M. leprae multiplies very slowly and the incubation period of the disease is about five years.

Symptoms can take as long as 20 years to appear.

Leprosy is not highly infectious.

Leprosy is transmitted via droplets, from the nose and mouth, during close and frequent contacts with untreated cases.

12/16/2012 9:55 AMDr. Alteib

Con..

Untreated, leprosy can cause progressive and permanent damage to the skin, nerves, limbs and eyes.

The disease mainly affects the skin, the peripheral nerves, mucosa of the upper respiratory tract and also the eyes.

Leprosy is curable and treatment provided in the early stages averts disability.

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RISK FACTORS FOR LEPROSY

People who live in the areas where leprosy is endemic (parts of India, China, Japan, Nepal, Egypt, and other areas) and especially those people in constant physical contact with infected people.

There is some evidence that genetic defects in the immune system may cause certain people to be more likely to become infected (region q25 on chromosome 6).

People who handle certain animals that are known to carry the bacteria (armadillos, chimpanzee,…) are at risk of getting the bacteria from the animals

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CLASSIFICATION OF LEPROSY

Two types of classifications:

Skin smear result classification

Clinical classification

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Con..

SKIN SMEAR RESULTS CLASSIFICATION

1. Paucibacillary leprosy (PB) – few Bacilli;

Two to five skin lesions with negative skin smear results at all sites

2. Multibacillary leprosy (MB);

Any form of leprosy in which the patient shows positive smears at any site

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Con..

CLINICAL CLASSIFICATION

1. Indeterminate leprosy (IL)

2. Tuberculoid leprosy (TT)

3. Lepromatous leprosy (LL)

4. Borderline leprosy (BL):

Borderline tuberculoid leprosy (BT)

Borderline borderline leprosy (BB)

Borderline lepromatous leprosy (BL)

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Con..

Adapted from Ramos-e-Silva M , Castro MCR. Mycobacterial infections.

In: Bolognia JL; Jorizzo JL; Rapini RP. Dermatology. 2nd edition. London: Mosby, 2008; 1109.

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CLASSIFICATION OF LEPROSY

Clinical findings Lepromatous Leprosy Borderline Leprosy Tuberculoid leprosy Indeterminate

leprosy

Type of lesions Macules, papules, nodules,

diffuse infiltration

Macules, papules,

plaques, infiltration

Infiltrated plaques,

oftenhypopigmented

Macules,

oftenhypopigmented

Number Numerous Many One or few (up to 5)

lesions

One or few

Distribution Symmetric Tendency to symmetry Localized,

asymmetric

Variable

Definition Vague, difficult to distinguish

normal versus affected skin

Less well- defined

borders

Well-defined, sharp

borders

Not always defined

Sensation Not affected Diminished Absent Impaired

Bacilli in skin

lesions

Many (globi) Many None detected Usually none

detected

Adapted from Ramos-e-Silva M , Castro MCR. Mycobacterial infections. In: Bolognia JL; Jorizzo JL; Rapini RP. Dermatology. 2nd edition. London: Mosby, 2008; 1109.

SIGNS AND SYMPTOMSTuberculoid leprosy:

Early signs and symptomsof Tuberculoid leprosy caninclude one or more slightred patches of skin thatappear on the trunk orextremities.

12/16/2012 9:55 AMDr. Alteib

Con..Other signs and symptoms of

Tuberculoid leprosy include:

• Skin stiffness and dryness

• Loss of fingers and toes

• Eye problems, which leads

to blindness

• Severe pain

• Muscle weakness, especially

in the hands and feet

• Enlarged nerves, especially

• those around the elbow and knee.12/16/2012 9:55 AMDr. Alteib

Loss of eyebrows and eyelashes

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12/16/2012 9:55 AMDr. Alteib

Hypopigmented Maculein Tuberculoid Leprosy

Nose Deformity Borderline TuberculoidLeprosy Skin Lesion

Con..

It is important to note that not all people withleprosy lose their fingers and toes. With earlydiagnosis and treatment, many of these signsand symptoms of leprosy can be prevented.

Many patients with Tuberculoid disease caneven self-heal without benefit of treatment.

In order to prevent problems with fingers ortoes, people should avoid injury and infectionsto these areas and take their medicines asprescribed.

12/16/2012 9:55 AMDr. Alteib

Con..Lepromatous leprosy:

It is the severe form of

leprosy, signs and

symptoms can Include

a symmetrical skin rash

More commonly found on:

Elbows

Knees

Buttocks

Face

Ears

Wrists.12/16/2012 9:55 AMDr. Alteib

arm nodules in lepromatous leprosy

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Con..

Other signs and symptomsof Lepromatous leprosy:

• Thinning of eyebrows

and eyelashes

• Thickened skin on face

• Nasal stuffiness

• Bloody nose

• Laryngitis

• Collapsing of the nose

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Con..

• Swelling of the lymph nodes in the groin and armpits

• Scarring of the testes that leads to infertility

• Enlargement of male breasts.

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ASSOCIATED COMPLICATIONS

Leprosy is probablythe most common causeof crippling in the handsworldwide. Leprosy complicationscan include:Blindness Loss of fingers or toesfollowing an injury or infection

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DIAGNOSIS

Diagnosis of leprosy is mostcommonly based on the clinical signsand symptoms. These are easy toobserve by any health worker after ashort period of training.

Only in rare instances there is a needto use laboratory and otherinvestigations to confirm a diagnosisof leprosy.

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Con..

In an endemic country or area, anindividual should be regarded ashaving leprosy if he or she showsONE of the following cardinal signs:

skin lesion consistent with leprosyand with definite sensory loss, withor without thickened nerves

positive skin smears

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TREATMENT

Multidrug therapy (MDT)

treatment has been made

available by WHO free of

charge to all patients

worldwide since 1995,

and provides a simple

yet highly effective cure

for all types of leprosy.

12/16/2012 9:55 AMDr. Alteib

Con..

The drugs used in WHO-MDT are a combination of:

Among these Rifampicin is the most important anti leprosy drug and therefore is included in the treatment of both types of leprosy.

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Multi Bacillary (MB) leprosy Paucibacillary (PB) leprosy

Rifampicin + Clofazimine + Dapsone Rifampicin + Dapsone

WHO recommended MDT regimens

12/16/2012 9:55 AMDr. Alteib

Multibacillary (MB) Paucibacillary (PB) Single Skin Lesion (PB)

Adults:Rifampicin: 600 mg/mDapsone: 100 mg/dClofazimine:300mg/m+50mg/d

Adults:Rifampicin: 600mg/mDapsone: 100mg/d

Adults:Rifampicin: 600 mgOfloxacin: 400 mgMinocycline:100mg

Children 10-14 years:Rifampicin: 450mg/mDapsone: 50 mg/dClofazimine: 150mg/m & 50mg/d

Children 10-14 years:Rifampicin: 450mg/mDapsone: 50mg/d

Children 10-14 years:Rifampicin: 300 mgOfloxacin: 200 mgMinocycline: 50 mg

Children less than 10:Rifampicin: 300mg/mDapsone: 25 mg/dClofazimine: 100 mg/m & 50mg twice/w

Children less than 10:Rifampicin: 300mg/mDapsone: 25 mg/d

Children less than 10:Not recommended for pregnant womenand children < 5 yrs

Duration= 12 months Duration= six months Duration= once

Con..

12/16/2012 9:55 AMDr. Alteib

Con..

Treatment of leprosy with only one anti leprosy drug will always result in development of drug resistance to that drug.

Treatment with Dapsone or any other anti leprosy drug used as mono therapy should be considered as unethical practice.

12/16/2012 9:55 AMDr. Alteib

Con..

The role for surgery in the treatment of leprosy occurs after medical treatment (antibiotics) has been completed with negative skin smears (no detectable acid-fast bacilli) and is often only needed in advanced cases.

Surgery is individualized for each patient with the goal to attempt cosmetic improvements and, if possible, to restore limb function and some neural functions that were lost to the disease.

12/16/2012 9:55 AMDr. Alteib

PREVENTION

Prevention of contact with droplets from nasal and other secretions from patients with untreated M. leprae infection.

Treatment of patients with appropriate antibiotics stops the person from spreading the disease.

People who live with individuals who have untreated leprosy are about eight times as likely to develop the disease, because they have close proximity to infectious droplets.

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Con..

Leprosy is not hereditary, but recent findings suggest susceptibility to the disease may have a genetic basis.

There is no commercially available vaccine available to prevent leprosy. However, there are reports of using BCG vaccine, the BCG vaccine along with heat-killed M. lepraeorganisms.

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PROGNOSIS

The prognosis of leprosy varies with the stage of the disease when first diagnosed and treated.

Early diagnosis and treatment limits or prevents tissue damage so the person has a good outcome.

However, if the patient's disease has progressed to more advanced disease, the complications can markedly affect the patient's lifestyle, and thus the condition has a fair to poor prognosis.

12/16/2012 9:55 AMDr. Alteib

GLOBAL CURRENT SITUATION

Leprosy control has improved significantly due to national and sub-national campaigns in most endemic countries.

Integration of primary leprosy services into existing general health services has made diagnosis and treatment of the disease easy.

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Con..

The implementation of the global leprosy strategy 2011–2015 national leprosy programs now focus more on underserved populations and inaccessible areas to improve access and coverage.

Since control strategies are limited, national programs actively improve case holding, contact tracing, monitoring, referrals and record management.

12/16/2012 9:55 AMDr. Alteib

Con..

According to official reports received from 105 countries and territories, the global registered prevalence of leprosy at the beginning of 2012 stood at 181 941 cases.

The number of cases detected during 2011 was 219 075 compared with 228 474 in 2010.

12/16/2012 9:55 AMDr. Alteib

Con..

Pockets of high endemicity still remain in some areas of Brazil, Indonesia, Philippines, Democratic Republic of Congo, India, Madagascar, Mozambique, Nepal, and the United Republic of Tanzania.

All endemic countries remain highly committed to eliminating the disease, and continue to intensify their leprosy control activities.

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SITUATION IN SUDAN

In 2002 the WHO launched a leprosy control program, with rapid simple diagnostic tools and the delivery of multi-drug therapy (MDT).

All efforts are constrained by instability, lack of suitable logistics, climate difficulties and poor roads, lack of resources and changes in health personnel.

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Con.. SITUATION IN SUDAN

In Northern Sudan 725 cases of leprosy were detected in 2008, 553 of which were multi bacillary (MB).

The cure rate for Multi Bacillary cases was 69.5%. However, in the Kordofan and Darfur states the Multi Bacillary cure rate was 57.3% due to instability, population movement and great stigma.

In Khartoum the Multi Bacillary cure rate is 80.7%, which percentage is affected by defaulters, re-registered patients and stigma.

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Con.. SITUATION IN SUDAN

Number of new cases in Sudan detected annually since 2004

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2004 2005 2006 2007 2008 2009 2010 2011

722 720 884 1706 1901 2100 2394 706

Sudan (North & South) pre-peace agreement

Sudan (North & South) / post-peace agreement

Sudan (North) / post separation

ELIMINATION OF LEPROSY

In 1991 WHO's governing body, the World Health Assembly (WHA) passed a resolution to eliminate leprosy by the year 2000.

Elimination of leprosy is defined as a prevalence rate of less than 1 case per 10 000 persons.

The target was achieved on time and the widespread use of MDT reduced the disease burden dramatically.

12/16/2012 9:55 AMDr. Alteib

Con..

Over the past 20 years, more than 14 million leprosy patients have been cured, about 4 million since 2000.

The prevalence rate of the disease has dropped by 90% – from 21.1 per 10 000 inhabitants to less than 1 per 10 000 inhabitants in 2000.

12/16/2012 9:55 AMDr. Alteib

Con..

Dramatic decrease in the global disease burden: from 5.2 million in 1985 to 805 000 in 1995 to 753 000 at the end of 1999 to 181 941 cases at the end of 2011.

Leprosy has been eliminated from 119 countries out of 122 countries where the disease was considered as a public health problem in 1985.

12/16/2012 9:55 AMDr. Alteib

Con..

So far, there has been no resistance to antileprosy treatment when used as MDT.

Efforts currently focus on eliminating leprosy at a national level in the remaining endemic countries and at a sub-national level from the others.

Early diagnosis and treatment with multidrug therapy (MDT) remain the key elements in eliminating the disease as a public health concern.

12/16/2012 9:55 AMDr. Alteib

Con...

WHO STRATEGY FOR LEPROSY ELIMINATION

Components:

ensuring accessible and uninterrupted MDT services available to all patients through flexible and patient-friendly drug delivery systems

ensuring the sustainability of MDT services by integrating leprosy services into the general health services and building the ability of general health workers to treat leprosy

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Con..

Encouraging self-reporting and early treatment by promoting community awareness and changing the image of leprosy

Monitoring the performance of MDT services, the quality of patients’ care and the progress being made towards elimination through national disease surveillance systems.

12/16/2012 9:55 AMDr. Alteib

Con..

Sustained and committed efforts by the national programs along with the continued support from national and international partners have led to a decline in the global burden of leprosy.

Increased empowerment of people affected by the disease, together with their greater involvement in services and community, will bring us closer to a world without leprosy.

12/16/2012 9:55 AMDr. Alteib

Con..

ACTIONS AND RESOURCES REQUIRED:

In order to reach all patients, leprosy treatment needs to be fully integrated into general health services.

Moreover, political commitment needs to be sustained in countries where leprosy remains a public health problem.

Partners in leprosy elimination also need to continue to ensure that human and financial resources are available.

12/16/2012 9:55 AMDr. Alteib

Con..

The age-old stigma associated with the disease remains an obstacle to self-reporting and early treatment.

The image of leprosy has to be changed at the global, national and local levels.

A new environment, in which patients will not hesitate to come forward for diagnosis and treatment at any health facility, must be created.

12/16/2012 9:55 AMDr. Alteib

REFERENCES

www.who.int/mediacentre/factsheets/fs101/en/index.html

WHO; Global leprosy situation, 2012

WHO; Weekly epidemiological record

OXFORD HAND BOOK OF CLINICA MEDICINE

Dermatology Online Journal – Volume 9 - Number 2 / Department of Bioregulation, Leprosy Research Center, National Institute of Infectious Diseases, Higashimurayama, Tokyo, JAPAN, norishii@nih.go.jp

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THANKS

AND

ALL THE BEST

12/16/2012 9:55 AMDr. Alteib