CHAPTER I

INTRODUCTION

Leprosy or also known as leprosy and Morbus Hansen is a disease that

has infected humans since more than 4000 years ago. The word leprosy a
translation of the Hebrew language, zaraath, which actually includes several
other skin diseases. Leprosy is also known as leprosy derived from the Indian
language, kushtha. The name Morbus Hansen is in accordance with the name
that finds germs, namely Dr. Armauwer Gerhard Hansen in 1874 2.
Leprosy is a chronic granulomatous disease that primarily affects the
skin, the channel upper respiratory and peripheral nervous system. cause of
leprosy is Mycobacterium leprae is intracellular obligate, and in the year 2009
has found a new cause that is Mycobacterium lepromatous. Leprosy formerly
known as disease that can not heal and treated, but since 1980, where the Multi
Drug Treatment (MDT) start introduced, leprosy can be diagnosed and treated
adequately, but unfortunately though has done adequate MDT therapy, the risk
for sensory and motor damage i.e. disability and deformity can still occur so
that the symptoms of limp hands, finger mutilation. The circumstances that
make the emergence of stigma against leprosy3 Despite the past 25 years much
has been developed about leprosy, knowledge of pathogenesis, causes,
treatment, and prevention of leprosy continues investigated.
 CHAPTER 2
LITERATURE REVIEW
2.1 Definitions
Leprosy is a chronic granulomatous infection disease caused by
Mycobacterium leprae is an obligate intracellular. The peripheral nerves as the
first affinity, but may also occur in the skin and mucosa of the upper respiratory
tract, and other organs except the central nervous system. (1.) (3)

2.2 Etiology
Mycobacterium leprae is a bacterium. It is intracellular, aerobic, cannot
be cultured in vitro, Gram-positive bacillus with size 3 8 m x 0.5 m, is
resistant to acid and alcohol (2). This bacterium has affinity to macrophages and
Schwann cells, replication is slow in Schwann cells to stimulate cell-mediated
immune response, which causes chronic inflammatory reaction, causing
swelling in the perineurium, can also result in ischemia, fibrosis, and death
axons. (3)
Mycobacterium leprae can reproduce at maximum temperature of 27
C-30C, it cannot be cultured in vitro, infecting the skin and cutaneous nervous
system. It has the ability to survive in a dry environment (skin, peripheral
nervous system, nose, earlobes, anterior chamber of the eye, upper respiratory
tract, legs, and testis), and not on a warm area (axillary, inguinal, heads, backs
the center line) (1)

2.3 Epidemiology
The prevalence of leprosy in the world is reported to be just <1 per
10,000 populations (as targeted WHO resolution on leprosy elimination). Most
prevalent in the tropical and Subtropical regions. 86% of cases were reported
in 11 countries, Bangladesh, Brazil, China, Congo, Ethiopia, India, Indonesia,
Nepal, Nigeria, Philippines, Tanzania. However, the prevalence of leprosy has
decreased since start of MDT (multi drug therapy) in 1982. In mid-2000, the
number of leprosy patients registered in Indonesia as many as 207,042 people,
mostly found in East Java, West Java, South Sulawesi, and Irian Jaya. The
spread of leprosy may be caused by displacement of population is infected with
the disease (2). (3)
Leprosy is more prevalent in males than females, by comparison 2:1,
with a peak incidence of 10-20 years and 30-50 years, is rare in infants. The
predisposing factor is the population in the area that is endemic, has leprosy
susceptibility in blood, and poverty (malnutrition). (1)

2.4 Classification
Ridley and Jopling introduced the term spectrum determinate on leprosy which
consists of various types, namely:
TT: polar tuberculoid, stable form
Ti: Tuberculoid indefinite
BT: borderline tuberculoid
BB: mid borderline unstable shape
BL: borderline lepromatous
Li: lepromatous indefinite
LL: polar lepromatous, stable form
TT is a type of polar tuberculoid leprosy, i.e. 100% tuberculoid, stable
type. may change type. LL is also a type of polar lepromatous, i.e. 100%
lepromatous. While the type between Ti and Li is called borderline or mixed
type, it means mix between tuberculoid and lepromatous. BB is a mixed type
50% tuberculoid and 50% lepromatous. BT and Ti more tuberculoid, while BL
and Li more lepromatous. This type of mixture is a labile type, meaning it can
switch type, either toward TT or LL (2).
According to WHO (1981), leprosy is divided into multibacillary (MB)
and paucibacillary (PB). Multibacillary means to contain many bacosi index
(IB), found bacteria more than +2, i.e. type LL, BL, and BB on Ridley-Joping
classification. Paucibacillary containing less bacillus with IB less than +2, i.e.
TT type, BT, and I classification Ridley- Joping (3).

2.5 Pathogenesis
The mode of transmission is not known exactly, assuming only the
classical assumption namely through direct contact between skin to skin
contact. The second assumption is by inhalation, because M. Leprae can live
for several days in the droplet. The incubation period is between 40 days to 40
years, averaging 3.5 years. Actually, M. Leprae has low pathogenicity and
invasion power, because patients who contain more germs may not necessarily
give more symptoms weight, even vice versa. Imbalances of degree of
infection and degree of disease, another cause is by no immune response, which
triggers the onset of granulomas local or comprehensive self-limiting or self-
progressive. Therefore, leprosy can be referred to as an immunologic disease.
The tissue damage depends on the cellular system of the cell, the type of bakery
spread, the presence of complications of leprosy reactions, and nerve
damage. Affinity in Schwann cells, mycobacteria bind to Domain G chains of
alpha laminin 2 found in peripheral nerves in the lamina basalt. Replication
inside these cells leads to a response of the cellular immune system causing an
inflammatory reaction, which causes swelling of the perineum, ischemia,
fibrosis, and axon death.
In LL type leprosy, there is paralysis of the cellular immune system,
thus macrophages are unable to destroy the bacterium and it multiplies freely
and damage the network. In leprosy type TT occurs otherwise, the ability of
cellular immunity is high, so macrophages are capable of destroying
germs. But after the germs are phagocytosed, macrophages transformed into
epithelioid cells and sometimes united to form the Langatans datia cells. Mass
epithelioid can cause nerve damage and surrounding tissue. (3)
 The appearance of leprosy symptoms occurs due to the development of
granuloma, and the patient may experience reactions, which can occur at
about> 50% of a particular patient. Spectrum leprosy granuloma consists of, a
high-resistance tuberculoid response (TT), a low- or absent-resistance pole
lepromatous (LL), a dimorphic or borderline region (BB), borderline
lepromatous (BL), borderline tuberculoid (BT). Based on the highest
resistance to a low resistance, namely TT, BT, BB, BL, LL (1).
The immune response to M. leprae can produce several types of
reactions related to clinical status. Leprosy reaction type 1 (downgrading and
reversal reactions), or non-nodular reactions occur in borderline types (Li, BL,
BB, BT, B), inflammation occurs among existing skin lesions. Clinical
symptoms of reversal reactions are generally partial or all existing lesions are
active and / or new lesions arise in relatively short time. The symptoms of acute
neuritis are most important to note, because it is very decisive by corticosteroid
drug, because without neuritis, administered facultative
corticosteroids. Reaction type 2(Erythema Nodosum Leprosum, ENL) occurs
in some individuals with LL, usually arise after anti-lepra therapy. Clinical
symptoms include erythema nodes, and pain with place of predilection in arms
and legs. Other organs can cause symptoms such as idiocyclitis, acute neuritis,
lymphadenitis, arthritis, orchitis, and acute nephritis with proteinuria. It can be
accompanied by constitutional symptoms from rug to weight depending on
status immunologic.
There is also a very severe leprosy reaction that occurs in lepromatous
type leprosy non-nodular diffuse, called the Lucio
phenomenon. Lucio reaction is a reaction that occurs in individuals with
widespread LL. Clinical features may include diffuse plaque or infiltrates,
pink, irregularly shaped and painful. Lesions especially in the extremities, then
extends throughout the body. Severe lesions appear more erythematous, with
purpura, and the bullous, then quickly occurs necrosis and painful ulceration.
lesions heal slowly and ultimately result in forming of scar tissue. (1). (3)

2.6 Diagnosis
The diagnosis of leprosy is based on clinical features, bacteriopia,
histopathological, and serological. Among the three, clinical diagnosis is
paramount. Between clinical and histopathologic diagnosis, there is the
possibility of equality and different types. Therefore, the clinical diagnosis
should be based on the results of the examination and clinical abnormalities of
the person's body. The form of clinical diagnosis is with inspection, palpation,
then checking using a simple tool such as needle, cotton, test tube containing
hot and cold water, and so forth.

2.6.1 Clinical Symptoms and Physical Examination

In leprosy, there are 3 cardinal signs, where one of them exists, its already
enough to establish a diagnosis of leprosy, namely: skin lesions anesthesia,
peripheral nerve thickening, and the discovery of M. leprae as bacteriological
positive. The incubation period is 2 - 40 years (average 5 - 7 years). Onset of
occurrence is slow and painless. The first time about the peripheral nervous
system with persistent or recurrent paresthesia and numbness without any
clinical symptoms. Involvement of the nervous system causes muscle
weakness, muscle atrophy, neurotic pain, and contraction of the hands and
feet. 90% of patients usually have a complaint at first time is the sense of
numbness, loss of sensory temperature so cannot distinguish between hot with
cold. Furthermore, the sensation of touch and pain, especially experienced on
the hands and feet, resulting in ulcer or numbness. Other parts of the body that
can be affected by leprosy is cold areas, i.e. the areas of the eyes, testes, chin,
nostrils, ears, and knee. Skin disorders in uncomplicated leprosy can only be
macular, infiltrates alone, or both. Also referred to as the greatest imitator of
many diseases other similar skins. (3)
To know the existence of malfunction of the autonomic nerves, note
there whether or not dehydration in the lesion area by scratching the lesion with
ink (Signs Gunawan), by scratching it from the center of the lesion toward the
normal skin, will be visible scratches on normal skin thicker. Alopecia may
also be observed in the region lesions. Impaired motoric functions can be
assessed by the Voluntary Muscle Test (VMT).
To find out the grip on the peripheral nerves, see if it has enlargement,
consistency, presence or absence of spontaneous and / or tender pain. There is
some superficial nerves that need to be checked, namely:
a. N. Ulnaris
Numbness on the fingertips of the anterior pinkie and ring finger.
Clawing on the little finger and ring finger.
Hypothenary atrophy and interosseous muscle and both medial
lumbrical muscles.
b. N. Medianus
Numbness at the fingertips of the anterior part of the thumb, forefinger,
and middle finger.
Not able to adduct the fingers.
Clawing your thumb, forefinger, and middle finger.
The thumb to contract.
Atrophy of fame and both lateral lumbrical muscles.
c. N. Radialis
Numbness of dorsum manus, as well as the proximal end of the index
finger.
Hanging hands (wrist drop).
Unable to extension fingers or wrist.
d. N. Poplitea Lateralis
Lower leg numbness, lateral part and dorsum pedis.
Foot drop (foot drop).
Peroneus muscle weakness.
e. N. Tibialis posterior
Sensory loss of the foot.
Claw toes.
Parinic intrinsic muscle paralysis and collapse of the pedis arch.
f. N. Facialis
Branches temporal and zygomatic: langophthalus.
Buccal, mandibular, and cervical branches: Loss of facial expressions
and failure to close lips.
g. N. Trigeminus
Numbness of facial skin, cornea, and eye conjunctiva.
Mushroom atrophy and both lateral lumblical muscles.

2.6.2 Physical examination

1. Tuberculoid Leprosy (TT, BT)
In TT, immunity is still good, can be cured spontaneously and still able
localize so that obtained image is clear. Regarding the skin as well as
nerves. The skin lesions may be one or several, may be macules or plaques,
and on the middle part can be found in a regression or central clearing
lesion. Surface lesions may be scaly, with edges rising. May be accompanied
by peripheral nerve thickening which is usually palpable. Smear negative BTA
is a sign of a response immune to leprosy. At BT, it cannot recover
spontaneously, Lesions resemble TT types but can be accompanied by satellite
lesions at the edges. Number of lesions can be one or several, but a picture of
hypopigmentation, skin dryness or squama not as clear as TT. Neurological
disorders are not as severe and asymmetrical.

2. Borderline Leprosy
In type BL is borderline, is the most unstable type, also known as Dimorphic
shape. Skin lesions are between tuberculoid and lepromatous. Consists from
macula infiltrative, glossy, the limit of the lesion is less assertive, the quantity
exceeds type BT and tend to be symmetrical. Lesions vary, can form punch
outs typical. In this type of anesthesia occurs and decreased sweat.

3. Lepromatous Leprosy
Type BL, classically the lesion begins with the macula, initially slightly
with quickly spread throughout the body. Macula is more varied in
shape. Distribution of lesions almost asymmetrical. Lesions infiltrates, and
plaques are like punched out. Signs of damage nerves in the form of loss of
sensation, hypopigmentation, decreased sweat and the disappearance of hair
comes faster. Thickening of the peripheral nerve palpable on the spot
predilection. Type LL, the number of lesions is very large, nodules reach the
size of 2 cm, symmetrical, smooth surface, more erythematous, shiny, bounded
not firm and on Early stages are not found in anesthesia and anhydrosis. Also
found lesions Dermatofibroma-like multiple, firm limits, erythematous
nodules. Distribution of typical lesions on face, about the forehead, temples,
chin, ear lobe. At an advanced stage it appears progressive skin thickening
forms facies leonine. Nerve damage cause symptoms stocking and glove
anesthesia. (1)
4. Histoid leprosy
This is the first type of lepromatous lesion variation. Clinical features include
nodes that are bounded firmly, can form plaque. Bacterioscopic positive high.
Generally, arises as cases of sensitive relapse or resistant relapse.

5. Neural type leprosy

Pure neural leprosy types have the following signs:
No or no skin lesions.
There is one or more neural enlargements.
There is anesthesia and / or paralysis, as well as muscular atrophy in
the region its nerve.
Bacterioscopic negative.
Test positive.
Perform a nerve histopathologic examination to determine the type
(usually type tuberculoid, borderline, or nonspecific).

2.6.2 Investigations
Bacterioscopic examination, preparation of skin tissue scrapings or
swabs nasal mucosa colored by staining of ZIEHL NEELSEN. Bacteriocopic
negative does not mean the person does not contain M. bacteria Leprae. First
of all, the skin lesions expected to be the most densely packed bacillus after
first determining the exact amount taken. For research can checked 10 places
and for routine preferably at least 4-6 places i.e. the second lobe lower ears and
the other 2 -4 most active lesions are the most erythematous and most
infiltrative. Selection of ear lobe regardless of whether or not it exists lesions
at the site because the ear lobe usually found plenty of M. leprae 3. M. Leprae
classified as smear, will appear red on the dosage. Differentiated the shape of
the stems intact (solid), stem broken (fragmented), and granular. Solid form is
a living germ, while fragmented and granular germs die. BTA density without
differentiating solid and nonsolid on a dosage expressed with a bacterial index
(IB) with a value of 0 to 6+ according to Ridley
0 if there is no smear in 100 field of view (LP).
1 + When 1 - 10 BTA in 100 LP
2 + When 1 - 10 BTA in 10 LP
3 + When 1 - 10 average smear in 1 LP
4 + When 11 - 100 average BTA in 1 LP
5 + When 101 - 1000BTA averaged in 1 LP6 + When> 1000 BTA averaged in
1 LP
Histopathological examination, histopathologic picture of tuberculoid
type is tubercles and more apparent nerve damage, no bacillus or only a few
and non-solid. Some lepromatous type seam silent sub epidermal (sub
epidermal clear zone) that is an area directly below the epidermis that the
network does not pathologic. Viral can be found with many bacilli. There are
borderline types a mixture of these elements. Virchow cells are histiocytes
were used as M. leprae as a breeding ground and as a means of transporting
dissemination. Serologic examination, based on the formation of antibodies in
a person's body infected by M.leprae. Serology is MLPA (Mycobacterium
Particle Aglutination leprae), and ML dipstick ELISA test, PCR. The lepromin
test is a non-specific test for leprosy classification and prognosis but not for
diagnosis. This test is useful to show the patient's immune system to M.
leprae. 0.1 ml of lepromin prepared from the bacillus extract of the organism,
injected intradermal. Then read after 48 hours/2days (Fernandez's reaction) or
3-4 weeks (Mitsuda reaction). Fernandez's reaction is positive when there is
induration and erythema showed that patients react to M. leprae, which is a
response. This slow immune types like Monteux test (PPD) on tuberculosis (3)
2.7 Differential Diagnosis
In macular lesions, the differential diagnosis is vitiligo, pityriasis
versicolor, pityriasis alba, Tinea corporis. In papule lesions, granuloma
annular, lichen planus. On plaque lesions, corpus tinea, pityriasis rosea,
psoriasis. In nodular lesions, acne vulgaris, neurofibromatosis. In nerve
lesions, amyloidosis, diabetes, trachoma (3).

2.7 Management
The main goal is to break the transmission chain to reduce the incidence
illness, treat and cure the patient, prevent disability, to achieve that goal, the
principal strategy undertaken is based on early detection and treatment of
patients with (4)
To prevent resistance, the use of multi-drug treatment of
leprosy treatment (MDT). The existence of this MDT is an attempt to prevent
and treat resistance, shorten the duration of treatment, and speed up the
breaking of the chain transmission.
Leprosy treatment regimen tailored and recommended by WHO/DEPKES RI
(1981). For that the classification of leprosy is simplified to be:
1. Pauci Bacillery (PB)
2. Multi Bacillery (MB)
PB with a single lesion given ROM (Rifampicin Ofloxacin
Minocyclin). Giving medication only once directly RFT = Release From
Treatment. Drugs taken at front officer. Children Pregnant women are not
given ROM. If the drug ROM has not available in Puskesmas treated with PB
treatment regimen of lesions (2-5). When a single lesion with nerve
enlargement administered: regimen of PB treatment lesions (2-5).
Treatment of Leprosy Reactions
1. ENL
Prednisone tablets 15-30 mg / day.
When the reaction is mild, it does not need to be given. In accordance with the
improvement of the reaction, dose lowered gradually.
Talidomide
The first choice drug. Contra indication in pregnant or fertile period, because
it is teratogenic. This medicine is not available in Indonesia.
Clofazimine
Used with higher doses, 300 mg / day. Given for 2-3 month. If there is
improvement, it will be reduced to 200 mg / day for 2-3 months. If there is
improvement, it is lowered to 100 mg / d for 2-3 months, and further back to
the original chlofazimine dosage, 50mg / day. At that moment similarly, the
dose of prednisone is gradually reduced.
2. Reversal reaction
- No neuritis: no additional treatment required.
- With acute neuritis: Prednisone tablets 40mg / day, lowered slowly.
Treatment should be started immediately to avoid nerve damage
suddenly. Members of motion affected by neuritis should be rested.

2.9 Prognosis
Depending on the extent of the lesion and the stage of the
disease. Recovery depending also on patient compliance with
treatment. Sometimes the patient can suffered paralysis and even death, as well
as the quality of life of patients dropped (2).
 CHAPTER III
CONCLUSION

Leprosy is a chronic granulomatous infection disease caused by

Mycobacterium leprae. The peak incidence is at the age of 10-20 years and 30-
50 years. Based on Ridley and Jopling leprosy is divided into TT, Ti, BT, BB,
BI, Li, LL, and according to WHO divided into Multibacillary and
Paucibacillary. Leprosy Diagnosis is performed based on clinical,
bacteriological, and histopathologic examination. Treatment leprosy
with Multi Drug Treatment regimen therapy began to be applied to prevent
possible rise of resistance.