Leprosy

History of the disease

Leprosy is known since Antiquity. The first descriptions go back to 600 years before J. - C. One finds it in ancient civilizations in China, in Egypt, in India. One believed a long time besides in an Asian origin; it was thought that it would have then spread by the warriors of Alexandre Large the then by the Phénicie NS and the Romains. Work on the Génome of the bacterium to the Institut Pasteur (Marc Monnot, Stewart Sticks, published in Science on May 13rd 2005) would rather indicate an East-African origin or of the Middle East before arriving to Asia and Europe. It would have arrived to West Africa with the explorers north-Europeans, then slavery would have disseminated it in the the Caribbean and the South America.

The Bible contains passages referring to “leprosy”, at the same time in the Old Testament and the Nouveau. One cannot know if it is about the same disease: this term was indeed used for many skin diseases of very variable origin and gravity. A Metzora , is a person reached of Tzara' At (" lèpre") in the book of the Lévitique. The law Jew made obligation with the Prêtre S to know to recognize leprosy ().

In the New Testament, Jesus cured the leprous ones.

The oldest texts testify some, leprosy always represented a threat, and the leprous ones put at the round of applause of the company, rejected by their community and their family. It is still often the case nowadays.

Leprosy gave place to measurements of segregation and social Exclusion, sometimes hereditary, like in the case of the Cagots of the South-west of France. The decrease of leprosy in Europe began as of the 16th century without a satisfactory explanation being had.

In 1873, the Norwegian Armauer Hansen discovers the bacillus responsible for this disease.

These 20 last years more than 12 million individuals were cured leprosy.
Its prevalence decreased by 90% and leprosy was éradiquée in 108 of the 122 touched countries.
leprosy is not any more one public health problems world since its world prevalence is currently lower than 1 case for 100.000 inhabitants.
It remains public health problems in 14 country of Africa and Asia (of which India).
the medical care to look after leprosy was discovered by a researcher of the Venezuela.

Epidemiology

One estimates at 2 million the population mutilated by leprosy in the world (2,8 million in 2005 according to).

Leprosy nowadays touches even more 700 000 people per annum in the world (France counts 250 cases declared, all originating in DOM-TOM or the zones of Endémie).

In 2000,738 284 new cases were thus identified (for 640 000 in 1999).
In 2001,755 000 cases of leprosy were diagnosed.
In 2002,763 917 new cases were detected.

The the World Health Organization (WHO) gave a report on 91 touched countries:

90% of the cases is with the Brésil, Myanmar, Mozambique, Madagascar, Ethiopia, India and Nepal (prevalence going from 2 to 4,6 for 10 000 inhabitants according to the countries). But the world prevalence remains stable, in the neighborhoods of 1%.
650 000 patients are under treatment.
70% of the cases recorded at the beginning of 2002 lived in India.
There were 34 new cases in Europe in 2002.
the most touched areas are by decreasing order: Southeast Asia, South America, Africa.
the sex-ratio is of 1.

Until recently, the man was the only known natural basin of Mycobacterium leprae , but 15% of the Tatou S savages of Louisiana and of the Texas were found carrying the disease. Mycobacterium leprae can be also present in the ground.

The transmission of Mycobacterium leprae is badly known. It often goes back to childhood by inhalation of “postilions” of leprous contagious. It is also done by mucosities of leprous settings in contact with ulcerations or wounds cutaneous, finally via soiled objects: linen, plait, pillows… All these modes imply the close contacts and durable of a promiscuity of the family type. The hereditary transmission does not exist but a congenital transmission is possible. Moreover, the infected ground and the insects vectors (bugs, mosquitos) could play a part in the transmission of the disease.

The untreated patients reached lepromatous type lodge a great number of Mycobacterium leprae in their nasal mucous membrane, nasal secretions, saliva, the cutaneous lesions. Leprosy tuberculoïde, the least severe form, is generally regarded as noncontagious. Incubation, exceptionally long (several years), explains why the disease develops only in the young adults.

Diagnosis

There exist various types of leprosy. Schematically, 2 clinical forms are distinguished: the leprosy tuberculoïde and the lepromatous leprosy , themselves connected by forms known as intermediate.

Since the years 1960, in order to better standardize and regulate the therapeutic one, WHO classified the clinical forms of leprosy in:

forms multibacillaires, corresponding to the forms lepromatous and intermediaries, having more than five cutaneous lesions.

forms paucibacillaires, corresponding primarily to the form tuberculoïde.

Leprosy tuberculoïde

This form of leprosy is most frequent. It associates:

of large spots dépigmentées on the skin, which became insensitive with the touch, edges Nets, single or of small number, containing little or not bacilli. The cutaneous eruptions, as in all the forms of leprosy, are nonpruriginous.
of the nervous disorders concerning the members, with disorders of the sensitivity and cutaneous anomalies: Ulcer perforating S, evils, mutilations, paralyzes.
and of the circulating Lymphocyte S which recognize Mycobacterium leprae .

These patients are not contagious.

Lepromatous leprosy

It is a general disease.

It is a form where the cutaneous and mucous lesions prevail:

the cutaneous attack prevails, with Macule S hypochromic S (with or without Anesthésie) discrete, with vague contours. Then the typical lesions of this form appear, the Léprome S, which are Papule S (infiltrated nodules) luisantes nodular of normal sensitivity, sitting on all the body, but prevailing with the face (with épistaxis and nasal Congestion).
the attack of the Nerf S is less severe in this form.
It does not have there immunity with respect to Mycobacterium leprae .

These patients are contagious.

Environ half of the patients presenting a lepromatous leprosy develop a leprous knotty erythema (ENL) during the very first years of effective Antibiothérapie. This reaction can occur spontaneously before the treatment, facilitating the diagnosis, or it can occur up to 10 years after the treatment.

Attacks join it:

Otho-rhino-laryngologiques (bloody Rhinitis, perforation, nasal Mutilation ),
ophthalmologic (very variable, being able to touch the Conjunctive , the Eyelid, the lachrymal Apparatus, the oculomotricity but with a preference for the former segment Uvéite, Sclérite and épisclérite, cataract, Glaucome)
nervous
visceral (dumb except complication): invasion Ganglion naire, hepatosplenic, testiculaires (90% of the men reached of lepromatous leprosy , with risk of sterility)

Leprosy borderline

Between these two well characterized forms, a true spectrum of forms known as intermediate is located, for which the defense reactions are unstable and variable. This “spectrum” is still badly known medical environment.

Differential diagnoses to evoke in front of hypochromic lesions

Seborrheic eczema S,
Pityriasis versicolor
rosy Pityriasis of Gibert
Dermite seborrheic
Vitiligo (but it there forever of achromy in leprosy)
Lupus
Herpes circiné
Hypomélanose idiopathic of the old subject (in drops)
creole Dyschromie (physiological in the mongrels)
Dépigmentation by dermocorticoïdes (to think of it at the African ones)

Differential diagnosis to evoke in front of papulous lesions

cutaneous Sarcoidosis (biopsy),
Recklinghausen,
Leishmaniose,
Disease of Kaposi.

Bacteriological diagnosis

It allows the diagnostic confirmation by description of the Mycobacterium leprae or Bacille of Hansen.

Its negativity does not eliminate the diagnosis, but its research is important for the forms borderline, to adapt the treatment (patient pauci- or multibacillaires), and to diagnose the relapses.

the bacilloscopy consists of 3 taking away: juice dermic of the 2 lobes of ear + 1 taking away on the level of a lesion.
the IDR of Mitsuda is abandoned.
the Nasal Smear and PCR are carried out according to the laboratories.

NB: public health clinics on Paris:

CHU Bichat- Claude Bernard, Pity,
Pasteur Institute.

A Coloration of Ziehl-Neelsen makes it possible to visualize the Mycobacterium leprae or Bacille of Hansen starting from products of scraping of the mucous membrane of the nose (leprous rhinitis) or starting from the cutaneous cells of eruption (lépromes).

One appreciates:

bacteriological numeration = Index IB = Bacillar Load with dimensions from 0 to 6
morphology = morphological Index = Viability of the bacilli (in %)

One distinguished the patients paucibacillaires PB (not of visible bacilli) and multibacillaires MB (bacillar Index not no one).
Actuellement the description of the bacillus is not necessary any more, and one distinguishes forms PB and MB according to the number of lesions.

Neurological attack

It determines the forecast of the disease.

Leprosy touches mainly the peripheral nerves. The Mycobacterium leprae has a neurological Tropisme. The bacillus multiplies in the Cellule of Schwann.

It begin (in the 1st year of evolution of the disease) with a hypertrophy from the nervous trunks to seek on the level of the cubital one, median, Sciatique external poplity (SPE), tibial posterior (TP), surface cervical plexus.

Then with the passing of years, appearance of a multiple mononévrite painful overdrawn.

The deficit touches initially the thermo-algic sensitivity, then driving conduction with driving deficit (Parésie then Paralysie), trophic Amyotrophie, and disorders…

The neurological clinical expression indicates that 30% of nervous fibers are reached by the bacillus of Hansen.

The most touched nerves are:

nerve V (corneal anesthesia),
nerve VII (attack of orbicular of the eyelids or the lips involving of the difficulties for the food, the elocution and thus of the difficulties of a psychosocial nature)
cubital nerve +++ (cubital claw, amyotrophie Hypothénar, cutaneous hypoesthesy thus burns…)
median nerve (amyotrophie thénar, cutaneous hypoesthesy….)
one can see mixed paralyzes cubitomédianes (“hand of monkey”), no possible gripping
the radial one, more rarely (falling Main)
the posterior tibial (TP) +++, Orteil S out of claw, badly perforating plantar
the SPE +++, falling foot (steppage), equine foot in varus
reached mixed SPE + TP

In general the chronology of the central nervous system disorders arises as follows:

Anesthesia, disorders trophic
cracks, Wound S, Burn S
infection ostéoarticulaire,
reduction in the bearing surface,
Scar S adherent
Amputation, Mutilation, loss of substance…

Disability

They are the ultimate complications of all the forms of leprosy.

These complications can be invisible (psychosocial consequences, disease taboo), and visible (mutilations, deformations, paralysis)

WHO is based especially on the ocular and neurological attacks to establish a score of disability.

Treatment

Medical care

The Medical care of leprosy was discovered by an Enquiring of the Venezuela.

Although not mortal, leprosy exposes to severe disabilities and permanent handicaps if it is not treated in time. The treatment comprises several antibiotics, in order to avoid selecting resistant stocks of the germ. The the World Health Organization (WHO) recommends since 1981 a polychimiothérapie (PCT) including/understanding three drugs, because Mycobacterium leprae develops finally resistance S in the event of monothérapie

the Dapsone (DDS)
the Rifampicine (RMP)
, the Clofazimine (CLO)

These three antibiotics constitute the treatment of reference of WHO. This medicamentous association destroys the disease-causing agent and cures the patient. The duration of the treatment oscillates between 6 and 24 months, according to the gravity of the Maladie.

In the event of resistance and/or allergy, one uses:

(S) = supervised treatment

New therapeutic diagram in the course of evaluation

rifampicine + ofloxacine + minocycline in 1 monthly catch supervised during 3 to 6 months for the patients paucibacillaires and 12 to 24 months for the multibacillaires.

Other treatments

When the lesions are already made up, the treatment rests in more on orthopedic Prothèse S, interventions , special shoes, etc

Relapses

It is the resumption of the disease after a well followed PCT. Sometimes a resistance to the ATB is in question.
They can be late, up to 9 years (6 for PB) after the PCT.
They are rare, 0,77% to 9 years (1,07% for PB at 6 years).

Immunity between tuberculosis and leprosy

There is a certain immunity crossed between the Tuberculose and leprosy.

The countries where tuberculosis prevailed in the past since are longest removed from leprosy.

See too

Canting hypocrites

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