leprosy

Submitted by:-
Amit Kochhar
B.O.T. 2nd year
Pt. D.D.U. I.P.H.
 Introduction
• Leprosy is a chronic bacterial infection with
Mycobacterium leprae.
• It primarily affects the skin, mucous membranes
(e.g. nose), peripheral nervous system (nerve
function), eyes and testes.
• The form the disease takes depends on the
person's immune response to the infection.
• Leprosy is also known as Hansen disease and is
one of the oldest known diseases of mankind.
• It is curable but if untreated can lead to severe
deformities.
Mycobacterium
leprae
 History
• Leprosy has tormented humans
throughout recorded history.
• The earliest possible account of a disease
that many scholars believe is leprosy
appears in an Egyptian Papyrus document
written around 1550 B.C.
• Around 600 B.C.
• Indian writings describe a disease that
resembles leprosy.
• In Europe, leprosy first appeared in the records of
ancient Greece after the army of Alexander the
Great came back from India and then in Rome in
62 B.C. coinciding with the return of Pompeii's
troops from Asia Minor.
• Throughout its history, leprosy has been feared
and misunderstood.
• For a long time leprosy was thought to be a
hereditary disease, a curse, or a punishment from
God.
• Before and even after the discovery of its
biological cause, leprosy patients were
stigmatized and shunned.
• For example, in Europe during the Middle Ages,
leprosy sufferers had to wear special clothing,
ring bells to warn others that they were close,
and even walk on a particular side of the road,
depending on the direction of the wind.
• Even in modern times, leprosy treatment has
often occurred in separate hospitals and live-in
colonies called leprosarium because of the
stigma of the disease.
• Leprosy has been so prevalent in various areas
as certain times throughout history that is has
inspired art work and influenced other cultural
practices
Nigerian Mask: A representation of leprosy
disfigurement
• In 1873:
Dr. Gerhard Henrik Armauer Hansen of
Norway was the first person to identify the
germ that causes leprosy under a
microscope.
Hansen's discovery of Mycobacterium leprae
proved that leprosy was caused by a germ,
and was thus not hereditary, from a curse, or
from a sin.
 classification
• Depending on clinical features, leprosy is
classified as:
Indeterminate leprosy (IL)
Tuberculoid leprosy (TT)
Borderline tuberculoid leprosy (BT)
Borderline borderline leprosy (BB)
Borderline lepromatous leprosy (BL)
Lepromatous leprosy (LL)
 Intermediate Leprosy (IL)
• This is the earliest and mildest form of the
disease.
• Few numbers of hypopigmented macules
(cutaneous lesions) may occur.
• Loss of sensation is rare.
• Most cases progress into a later form, although
patients with strong immunity may either clear
the infection on their own or persist in this form
without progressing.
Intermediate leprosy :
hypoaesthesia and
hyposudation investigations
must be systematic in endemic
area.
• Patients with indeterminate leprosy, a very
early form of leprosy, may either be cured
or progress to one of the other forms of
leprosy depending on their immune status.
 Tuberculoid leprosy (TT)
• Tuberculoid (TT) leprosy appears on the
extreme left of the Immunological Spectrum as
that form of the disease which is most resistant.
• Those who have this form have a good Cell-
Mediated Immunity (CMI).
• The key word in relation to this disease in
making a diagnosis is “LOCALISED”. Because
of the body’s strong CMI defenses, the disease
is being contained or localized either to a skin
patch - generally ONE patch in the early stages,
or to a nerve trunk.
• When the affected tissue of a Tuberculoid
patient is examined pathologically, such a
skin biopsy shows collections of these
epithelioid cells surrounded by many
groups of lymphocytes and, occasionally
“Giant Cells” can be seen in such a
grouping.
• Nerves in TT leprosy lesions can be
seriously affected and even destroyed if
the disease is not treated
• CLINICAL FEATURES IN TUBERCULOID
LEPROSY :-
(1) Lesions generally are on the face,
lateral aspect of extremities and the
buttocks
(2) Skin lesions may be single of few
(3) Lesions are asymmetrical
• DESCRIPTION OF SKIN LESIONS:-

(1) Lesions are slightly or well infiltrated

(2) Patches may be hypopigmented or
erythematous
(3) Margins of the patch are well defined
(4) Margins of the patch may be raised
(5) Patch may indicate a central area which is
healing, but dry and rough
(6) Patch has a sensory deficit - often totally anaesthetic
(7) Patch may be small or large
(8) Patch is warm due to loss of sweating
(9) Hair may be absent or spare in lesions.
• DESCRIPTION OF NERVE LESIONS:-
(1) The cutaneous nerve supplying the lesion is
usually palpable
(2) Trunk nerves (one or two) supplying the
lesion may be tender and enlarged
(3) The related main peripheral nerve trunks
may show thickening, tenderness, abscess
formation and occasionally calcification.
• DESCRIPTION OD SMEAR
EXAMINATION:-
(1) Skin smears taken by the regular “slit and
scrape” method are usually negative
(2) Smears taken from the margin (active part)
of the lesion occasionally show a few M.leprae

• 50% + of TT patients have bacilli in striated

muscle and this is often far more than can be
found in skin lesions.
• LEPROMIN TEST :-
Strongly positive.
 Borderline tuberculoid leprosy
(BT)
• Lesions in this form are similar to those in
the tuberculoid form, but they are smaller
and more numerous. The nerves are less
enlarged, and is less alopecia is present.
• Disease can remain in this stage, convert
back to the tuberculoid form, or progress.
 Borderline borderline leprosy
(BB)
• Cutaneous lesions consist of numerous, red,
irregularly shaped plaques that are less well defined
than those in the tuberculoid type.
• Their distribution may mimic those of the
lepromatous type, but they are more asymmetric.
• Anesthesia is only moderate.
• Regional adenopathy may be present.
• Disease may remain in this stage, improve or
worsen.
• Sensory loss is moderate
Tuberculoid- like lesion of mid-borderline
leprosy with hypopigmentation and
anesthesia.
The raised border is prominent in this
case.
• MA are activated in epitheliod cells but do
not form distinct granulomas
• Edema is prominent
• Scanty lymphocytes
• No Langhan‘s giant cells
• Nerves not enlarged and fairly normal
Borderline lepromatous leprosy
(BL)
• Lesions are numerous and consist of
macules, papules, plaques, and nodules.
• Annular punched-out-appearing lesions
that look like inverted saucers are
common.
• Anesthesia is often absent.
• As with the other forms of borderline
leprosy, the disease may remain in this
stage, improve, or regress.
• Peripheral nerves are often enlarged
symmetrically, but cutaneous nerves are
not.
• Poorly to moderately defined granulomas
(MA granuloma) with prominent
lymphocytes
– No giant cells
– Dense lymphocyte cuff around nerves bundle
– Nerves are nor swollen but the perineum is
laminated (perineural fibroblast proliferation -
„onion structures“)
– Foamy cells are not prominent
 Lepromatous leprosy (LL)
• Early nerve involvement may go unnoticed
• Numerous lesions of all kinds, plaques,
macules, papules and nodules
• Early symptoms include nasal stuffiness,
discharge and bleeding, and swelling of
the legs and ankles
• Left untreated, the following problems may
occur:
– Skin thickens over forehead (leonine facies),
eyebrows and eyelashes are lost, nose
becomes misshapen or collapses, ear lobes
thicken, upper incisor teeth fall out
– Eye involvement causing photophobia (light
sensitivity), glaucoma and blindness
– Skin on legs thickens and forms ulcers when
nodules break down
– Testicles shrivel causing sterility and enlarged
breasts (males)
 – Internal organ infection causing enlarged liver
and lymph nodes
– Voice becomes hoarse due to involvement of
the larynx
– Slow scarring of peripheral nerves resulting in
nerve thickening and sensory loss. Fingers
and toes become deformed due to painless
repeated trauma.
• Lepromatous leprosy is of two type:
– Macular
– Nodular
• Clinical Manifestations of Macular
Lepromatous leprosy :-
1) Generally present on Face, Buttocks,
Trunk and the extensor aspect of the
extremities
(2) Patches are multiple, or vaguely
erythematous / hypopigmented.
Distribution is symmetrical
(3) Texture of skin - waxy appearance,
smooth and erythematous
(4) Margin of patch NOT well defined (as in TT)
but merges into surrounding area
(5) Initially, patches do NOT have sensory deficit

(6) Sweating may be lost in lesions but later than

in TT.
(7) To compensate, the body’s cooling system
makes the sweatable areas sweat
excessively
(8) Nerves are not thickened.
(9) Skin smears and nasal scrapings
Bacteriologically POSITIVE.
• Clinical Manifestations of Nodular
Lepromatous leprosy :-
(1) “Nodular” is the most advanced form of
lepromatous leprosy
(2) The Nodules are rounded and
circumscribed
(3) Nodules are of varying size and shape
(4) LL Nodules are not tender
(5) LL Nodules progress very slowly,
taking several months to develop
 (6) The Nodules are found in mucous
membrane and in the skin
(7) They are most common on the face and
ear-lobes
(8) When LL nodules ulcerate, they
discharge large numbers of M.leprae
(9) These are the TRUE “leprous” or “
lepromatous” ulcers, unlike those on hands
and feet which are secondary and due to
injuries such as burns etc.
Lepromatous Leprosy
(Early/Late Stages)
Lepromatous Leprosy Pre- and
Post-Treatment
 Tuberculoid vs. Lepromatous Leprosy
Clinical Manifestations and Immunogenicity
Lepromatous vs. Tuberculoid
Leprosy
How the Human Body is
Affected by leprosy
Nerve is Large bumps (legions)
damaged and on the skin that do not
broken by heel and cannot feel
Leprosy infection leprosy
pain.
Nerve infection.
 The Immune cells attempt
to seal off the infection,

Human Body Diagram

which can destroy sweat
glands and hair follicles and
the skin’s sensitivity.

Mycobacterium Leprae
enters the body

On of these things
can happen

Immune cells unable to destroy M.

Leprae and the bacteria multiplies
freely in the skin.
 Treatment
• Chemotherapy:
All patient should receive multi-drug therapy (MDT).
First line drugs: Rifampicin, Clofazimine, and Dapsone.
 Treatment
Second line therapy: Minocycline, clarithromycin, and
ofloxacin, are highly effective against M. leprae.

Reversal reaction:
Peak time: during the first 2 month of therapy, even up 12
months, and after (MDT) is completed.
Corticosteroids 40-60mg daily, taper 5 mg every 2-4
weeks, duration of therapy 3-4 months.
Recovery rate for nerve function 60-70%, less with pre-
existing nerve damage or recurrent reaction.
MDT
 Treatment
Type 2 reaction (ENL):

Develop during 1st or 2nd year of MDT, and can relapse

over several years.

Anti- inflammatory: Clofazimine 300mg daily. Or

Drug that target overproduction of TNF-α,Thalidomide

400mg daily, or pentoxifylline. Or

Neutralization of TNF-α with monoclonal antibodies, or

soluble inhibitors.
 Leprosy and orthopeadics
• Leprosy is known in the society as a
disease producing ugly deformities and
mutilations.
• Deformities are seen in all types of
leprosy, but are more common in
tuberculoid and borderline types.
 Mechanism causing disability
• Nerve involvement, leading to
anaesthesia,adryness of the skin and
paralysis, is primarily responsible for
deformity and disability of the hand and
feet.
• These factors predispose the affected
limb to misuse, resulting in ulceration,
scar formation and secondary infection
• These in turn, add to disability and create
a vicious cycle whereby loss of deep
tissue results.
• The clinical manifestation of leprosy
relevant from viewpoint of orthopaedics
are:
– Deformities
– Motor weakness and muscle atrophy
– Trophic ulcers
– Mutilations
– Neuritis
 Motor weakness and muscle
atrophy
• As a result of nerve involvement,
commonly seen motor weakness are claw
hand and wrist drop in the hand and foot
drop in the leg.
• Conservative treatment is by splints.
• The following reconstructive procedures
may be performed in some cases
– For claw hand:- Paul Brand’s multi tail tendon
transfer
– For opponens weakness:- opponensplasty
– For wrist drop:- Jone’s transfer
– For foot drop:- transfer of tebialis posterior
tendon on the dorsum of the foot
 Trophic ulcers
• These are found at anaesthetic sites, and
are precipitated and perpetuated by
recurrent injury or abnormal areas of
pressure developing on paralysed hand
and feet.
• The cause of injury could be mechanical,
thermal etc.
• Common sites of trophic ulcers are:-
– Head of first and fifth metatarsals
– Heels
– Terminal phalanges of fingers
• Early manifestations may be spontaneous
blister, a nodule or an injury at the
anaesthetic site.
• This leads to ulcers formation, which may
get secondarily infected
• The ulcer may extend deep and affect
soft-tissue and bones, and become
chronic and progressive.
• Cause responsible for chronicity of an
ulcer are impeded vascular supply and
repeated trauma to the ulcer, and
superadded infection
• Treatment:- prevention of ulcer is most
important because once it occurs,
healing takes a long time.
• Treatment of an established ulcer
consists of the following:-
– Elimination of stress
– Eradication of infection
• Debridement
• Sequestrectomy
• Antibiotics
• Occlusive dressing
– Other:-
• Plastic surgery procedure
• Amputation
– Prevention of recurrences:-Protect the scar
from further injuries by:-
• Good care of the part
• Proper footwear or splints
• Careful use of the part (e.g., avoiding jumping,
walking in cases with foot ulcers)
 Mutilations
• Mutilations result from recurrent trophic
ulcers, sequestration of bone and
decalcification of bones.
• These are as a results of ultimate neglect
of a fairly treatable condition
 Neuritis
• Leprosy may result in acute or chronic
neuritis.
• The patient complains of pain along the
course of the nerve, and later neurological
symptoms.
• In acute stage , rest of the part and anti-
leprosy chemotherapy is given .
• Some surgeon prefer local or systemic
corticosteroid in acute stage.
• In chronic cases there is diffused
thickening of the nerves.
• Occasionally, a nerve abscess can be
palpated.
• The indications for surgical intervention
are:
 – Abscess inside the nerve- in which case it is
drained;
– Intractable pain in a person in whom paralysis
in the distribution of the nerve is already
present.
• Neurolysis has been attempted in these
cases as no further harm can be done.
 Deformities
• The primary factor responsible for
deformities in leprosy is involvement of
peripheral nerves, but secondary factors
contribute to a large percentage of
deformities.
• The latter are totally preventable, hence
important.
• Some such factors are:
– Malpositioning of paralysed limbs
– Scarring and ulceration
– Self inflicted injuries to an anaesthetic part
etc.
• Nerves commonly affected in leprosy are
those in superficial locations.
• In order to frequency these are:-
– Ulnar nerve at the elbow
– Median nerve above the wrist
– And common peroneal nerve at the knee
• Common deformities:-
– Hand:-
• Claw (partial or total)
• Ape thumb
• Wrist drop
– Foot:-
• Foot drop
 Treatment
• A great deal of deformities in leprosy are
preventable,
• Firstly by early detection of leprosy and
adequate drug therapy;
• Secondly by health education about
– Hygienic care of anaesthetic foot,
– Prevention of insect bite,
 – Proper splintage of paralysed part, and
– Prompt and adequate care of trophic ulcers.
• Conservative treatment using splints,
exercises and other occupational and
physiotherapeutic measures are used in
most cases
• In some cases surgical correction of the
deformity is required.