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Leprosy - case definition and examination
1. LEPROSY – case definition
and clinical examination
- Dr. Shivi Nijhawan
2. LEPROSY
At the seventh meeting of the WHO expert committee on
Leprosy in 1997,
A case of leprosy was defined as an indivisual who has not
completed the course of treatment and has one or more of the
three cardinal signs :-
Hypopigmented or erythematous skin lesions with definite
loss /impairment of sensations.
Involvement of the peripheral nerves as demonstrated by
definite thickening with sensory impairment.
Skin smear positive for AFB
3. CLASSIFICATION
Leprosy can present with predominantly neural symptoms or mainly
with cutaneous involvement.
In ancient India it had been called ‘Vat Rakta’ denoting the neural
component of the disease .
‘Arun kushta’ for cutaneous form of leprosy
First international classification –MANILA(1931) by Leonard Wood
Memorial, categorized the disease into cutaneous, neural and mixed
type.
MADRID classification (1953)- maculoanaesthetic macules ,
lepromatous macules and intermediate macules.
In the following years , many similar clasifications came up, where
the term cutaneous was replaced by lepromatous that repesented a
widespread involvement of skin , and the term neural got replaced by
tuberculoid representing raised lesions with neural deficit.
4. Lepromatous and tuberculoid were regarded as polar forms.
A widespread discontentment among leprologists about
maculoanaesthetic lesions and pure neuritic cases.
Indian leprologists regarded these two as separate entities
INDIAN classification,1953 (Indian Association of
Leprologists) proposed by Dharmendra and Chatterjee ,
similar to the Madrid classification. Accepted in 1955.
RIDLEY AND JOPLING classification (1962) – spectral
classification for research purposes. Since then globally
accepted.
1982, WHO classification- Paucibacillary (PB) and
Multibacillary (MB)
5. MADRID CLASSIFICATION
International Leprosy Congress held at Madrid in 1953 .
Two types –Lepromatous (L)and Tuberculoid (T)
Two groups- indeterminate (I) and borderline or Dimorphous
8. RIDLEY JOPLING
CLASSICATION
• William Jopling together with D.S. Ridley proposed this
classification.
• Referred as the immunological classification.
• Based on bacteriological, immunological, histopathological
and clinical features.
• Spectral and polar concept of leprosy.
• Two polar forms –Tuberculoid and Lepromatous –stable
• Immunologically unstable-Borderline group- BT,BB and BL
9.
10. BB –most unstable form
These forms transform into each other by uprading or
downgrading reactions.
Clinical , histopathological , bacteriological and immunological
features show slow continous change from one pole to another
ADVANTAGES-
1. Leprsoy is a disease of a wide range of clinical presentations
, course , prognosis and complications. This classification
helps to categorize different types of leprosy based on
correlationship of various parameters Widely used for
research purposes.
2. Strengthens the spectral and polar concept of leprosy.
3. Gives an idea about the prognosis.
11.
12. DRAWBACKS –
No specific place for indeterminate and pure neuritic leprosy in
the spectrum.
13. WHO CLASSIFICATION for
Leprosy control
Used for treatment purposes.
Segregation into two groups for treatment purposes - the less
bacillated patients could be treated with with lesser number of
drugs for shorter period , more bacillated patients treated with
more number of drugs for longer period.
Paucibacillary(PB) and Multibacillary leprosy(MB).
In 1988, based on whether the skin slit smears demonstrated
bacilli or not. Paucibacillary (no AFB in split skin smear),
multibacillary(AFB present in any skin lesion).
In 1998, based on the number of skin lesions.
1. Paucibacillary single lesion leprosy(SLPB)
2. Paucibacillary leprosy (2-5 lesions)
3. Multibacillary leprosy >6 lesions and also any split skin smear
positive
14.
15. Presently in India , number of nerves involved
is also taken into consideration while
categorizing the patients into paucibacillary
and multibacillary types as per the NLEP
(National leprosy Eradication programme ) of
Government of India.
17. TUBERCULOID LEPROSY(TT)
Benign and stable
Number – 1 to 3 lesions.
Morphology- well defined erythematous plaques with raised
and clear cut edges sloping inwards. Lesions may be flat in the
center with a raised well defined margin(annular)
Surface – dry ,hairless, anesthetic and usually scaly.
Nerve involvement occurs as a result of extension from or
through cutaneous nerve branches. Patchy and asymmetric.
Peripheral Nerve - sensory loss
Autonomic nerve -dry surface and loss of sweating. Rough
skin .
A solitary peripheral nerve trunk may be thickened in the
vicinity of a TT lesion (feeding nerve)
No AFB found on slit skin smear.
Lepromin test strongly +ve .
18.
19. BORDERLINE
TUBERCULOID(BT)
3 to 10 skin lesions
Lesions similar to TT , but there is evidence of the disease not
being contained. Small extension of the lesion at one
edge(pseudopodium) or there may be satellite lesions. Lesions
have less defined margins and the border may fade into the
normal skin.
Loss of sensations, dryness ,scaling ,erythema or
hypopigmentation less conspicuous than that in TT .
20.
21. BORDERLINE
BORDERLINE(BB)
Most unstable. BB state is short lived and rapidly shifts to other
poles, more often to BL.
Mostly downregulates towards lepromatous pole if untreated.
Presence of dimorphous type of lesions .
Multiple skin lesions with a tendency to symmetry. Lesions are
of all shapes and sizes including papules, plaques, circinate
lesions or rarely even nodules.
Characerstic skin lesions are the annular lesions where the
inner edge is well demarcated and the outer edge is ill defined
and slopes towards normal skin. ‘Punched out’ appearance
(inverted saucer shape).
Clinically , normal looking skin;within such plaques gives a
“swizz cheese” appearance.
Face may show inflitration with occasional nodules over ears
and chin.
24. BORDERLINE
LEPROMATOUS(BL)
Numerous skin lesions (>30)
Ill –defined showing tendency to bilateral symmetry.
Infiltrated macules with copper hue, round or oval about 2-3cm
in diameter. With disease progression , papules, nodules and
plaques may develop, with sloping margins which merge into the
normal skin.
Infiltration takes place within the itinial macules, creating a
plaque like appearance on face and ears. Surface of lesions
shiny.
Nerve trunks get damaged (not so symmetrically)
Eyebrows involvement absent or partial.
Systemic symptoms of involvement of oral cavity or eyes, testes
appear later
More prone to develop type 2 reactions (erythema nodosum
leprosum).
Many AFB’s seen on sss.
Lepromin test negative.
25.
26. LEPROMATOUS LEPROSY(LL)
Multiplication and universal spread of M.leprae
Early lesions –innumerable small infiltrated, shiny,
erythematous macules, with indistinct edges, widely
disseminated and distributed symmetrically.
Insidious onset and steady progression
LL with with infiltrated lesions presents as 3 distinct forms-
diffuse, infiltrated and nodular forms.
AFB positive in sss.
Lepromin reaction negative.
27. a) Diffuse LL
True subtype of LL
Results from gradual coalescing of various numerous
vague macules of macular LL.
Slight infiltration which is better appreciated by touch
rather than sight.
Thickness of skin due to diffuse infiltration. Thickness
most marked over the face especially forehead, earlobes,
eyebrows, nose and malar surfaces. Ear lobes are shiny
and thickened (buddha ears)
Thinning or loss of eyebrows.
28. b)Infiltrated LL
Areas of marked infiltration
Advanced stage of macular LL, easily visible infiltration
29. c)Nodular LL
Result of progressive deterioration of macular, diffuse or
infiltrative forms of LL.
Disease advances and the nodules appear on buttocks, face
,extremeties specially the elbows, fingers, over the joints and
genitals.
Infiltrated plaques accentuate the skin folds producing the
classical leonine facies.
Gradual appearance of sensory and autonomic nerve damage.
Clinical signs of nerve damage appears until the disease is well
advanced. Peripheral anesthesia is extensive due to systemic
dissemination of lepra bacilli. Sensory fibres damaged first.
Peripheral nerves first become firm, hard and then fibrosed at
the places where nerve trunks are close to cooler skin
surface(radial groove,behind medial epicondyle of ulna, neck of
fibula)
Anhidrosis with compensatory hyperhidrosis of the face ,trunk
and axillae.
Symmetrical sensory loss-over the extensors of forearms, legs,
hands and feet-typical glove and stocking anesthesia.
30. Nail changes- growth may be disturbed late in the disease
course.Nail plates become lustureless, ridged and curved
Hands and feet – swollen digits tending to taper towards the tips
(fusiform swelling)
Digits may shorten due to resorption of phalanges.
When significant anesthesia has developed, the hands and feet are
liable to trauma and pressure necrosis of soft tissue , leading to
ulceration and seconday infection with clinical signs of cellulitis &
osteomyelitis.
Involvement of upper respiratory tract mucosa -80 percent of LL
cases in form of a stuffy or blocked nose followed by epistaxis.
Anosmia , crusting or bleeding.
Nasal septal perforation –destruction of bony part-saddle nose
deformity
Oral mucosa- nodules and plaques over tongue and palate.
Larynx – hoarseness of voice and stridor
Eye – corneal anesthesia due to bacillary infiltration of cornel
nerves, lagopthalmos, uveitis, corneal opacity, perforation and
blindness.
31. Testicular atrophy
Prone to type 2 reactions.
Cause of Death – due to secondary infections (pneumonia and
TB) , amyloidosis and renal failure.
34. INDETERMINATE LEPROSY
Medium to large hypopigmented patches which are faintly
visible,often on the external aspect of the thigh, face,
extensor aspect of limbs.
Vague edges
Some loss of tactile and thermal sensations
Commonly misdiagnosed as P. alba
Excellent prognosis
Lepromin reaction is variable
AFB mostly not detectable.
Diagnosis can only be confirmed with a biopsy- typical
perineurovascular infiltrate.
38. PURE NEURITIC LEPROSY
Area of sensory loss in the absence of any skin patch
along the distribution of an involved nerve trunk with or
without motor deficit.
Neuritic manifestations- tingling,heaviness, numbness,
peresis, hypotonia, atrophy,claw hand and toes, wrist drop
and foot drop, neuropathic ulcers, bone resorption.
Most frequently in India and Nepal
Accounts for 5-10 percent
40. 1. GENERAL PHYSICAL EXAMINATION
2. CUTANEOUS AND MUCOSAL
INVOLVEMENT
3. OCULAR INVOLVEMENT
4. PALPATION OF PERIPHERAL NERVES
AND TESTING FOR SENSORY
IMPAIRMENT
5. EXAMINATION OF MUSCULOSKELETAL
SYSTEM
6. EXAMINATION OF EXTERNAL GENITALIA
7. OTHER SYSTEMIC EXAMINATION
41. GENERAL PHYSICAL
EXAMINATION
Thorough physical exmination should be performed.
Pallor, edema and lymphadenopathy may be a part of
lepromatous disease.
If a patient on dapsone therapy shows profound pallor of
sudden onset ,evidence of hemolysis should be looked for.
Pulse and blood pressure of all patients should be recorded at
presentation and followed up.
Hypertension in leprosy patients may result from chronic renal
impairment due to type 2 reactions.
Bilateral pedal edema also involving the dorsa of hands is seen
in lepromatous patients.
Generalized edema may result from autonomic neuropathy
affecting the small blood vessels or due to acute
glomerulonephritis associated with type 2 reactions.
Widespread tender lyphadenopathy – type 2 reaction
Fever, arthralgia and prostration in leprosy patients, possiblty
of type 1 or 2 reactions.
42. CUTANEOUS EXAMINATION
In adequate daylight, following proper exposure and ensuring the
privacy of the patient.
Widespread infiltration , shiny skin, sparse body hair, prominent
follicular openings and mild thickening appreciatble by gentle
pinching of the skin, is suggestive of LL.
Infilterated face with thick skin folds and nodularity (leonine
facies), depressed nose, enlarged ear lobules(buddha ears)
sparse beard and moustache indicate advanced LL.
Unilateral gynecomastia in males-impairment of testicularfunction
in LL
An overall brownish pigmentation involving skin, conjunctiva and
oral mucosa may indicate treatment with clofazimine,
43. Documentation of indivisual skin lesions
should be done on a printed proforma.
Allows proper follow up of the patients.
44.
45. Examination of indivisual skin
lesions
Total number of lesions- single, 1-5, >5(multiple) or numerable.
Fewer the number, lower the bacillary load.
Distribution – trunk, extremities, face; protected sites like, scalp,
axillae, groin, perineum, genitalia, palms and soles, genitalia or
buttocks. Asymmetric or bilaterally symmetrical ?
Shape- regular(round/oval), irregular/bizarre,annular
Size
Morphology :-
1. Patch/plaque,papule/nodule/vesicle/bulla
2. Color (hypo/hyperpigmented, skin-colored, erythematous,
coppery)
3. Surface (no change, dry/scaly/smooth and shiny/edematous or
ulcerated)
4. Border(well defined, raised/flat,sloping,punched out)
5. Presence, absence or sparseness of hair
6. Lesional tenderness
46. An ill-defined, hypopigmented, flat lesion without surface changes may
be an intermediate leprosy patch.
Thick ,elevated margin of a lesion suggestive of TT or BT. A feeding
nerve to the lesion can be detected by gentle rolling of a finger along
the borders in TT or BT.
Annular lesions- inner and outer margins –better defined inner margin
and outer margin sloping into normal skin giving a punched out
appearance (inverted saucer shaped) suggestive of a BB lesion.
Presence of pseudopodia and satellite lesions along the margins of
large lesion are suggestive of a BT lesion.
Nodules
1. Persistent, asymptomatic, erythematous, coppery normal skin colored
nodules, firm on palpation-LL
2. Well defined, succulent, hemispherical glistening nodules –histoid
leprosy
3. Recurent, erythematous, evanescent, tender nodules on the skin over
face, arms and thighs, healng with postinflammatory hyperpigmentaion
–ENL
Ulcerated lesions, Sudden onset of lesional erythema, edema and
tenderness suggests reactions
47. Testing sensations
Lesional skin should be tested for
1. Temperature(hold/cold water in test tubes)
2. Touch (wisp of cotton wool)
3. Pain(pin-prick)
while testing the sensation, the examiner should proceed
from uninvolved to involved skin.
All sensations shuld be tested gently ,only once at one site,
for a short while, not pressing the test object too hard or
brushing it on patient’s skin.
The patient may not be able to point the exact location of
the application of stimulus(misreference), which may be an
early sign of hypoesthesia. Permissible limits of mis-
refernce on hands are 1cm, on face 2cm and on back it is
upto 7cm.
Testing with Semmes-Weinstein monofilaments helps in
detecting early hypoesthesia.
48.
49. Distal extremeties should be examined for
‘gloves and stockings’ hypoesthesia in LL.
WHO recommended sensory testing sites on
palms and soles(10 on each side) for disability
grading .
*Hands – ulnar nerve :distal pulp and proximal
phalynx of little finger and hypothenar eminence.
median nerve:distal pulp of thumb and index
fingers and thenar eminence
*Feet – big toe, metatarsal heads( 1st,5th), mid-
lateral border of foot.
50.
51. Trophic changes: asymptomatic, deep, non-healing
fissures on heels and toes may be observed in people
who walk bare foot and agricultural/manual workers
suffering from leprosy.
Multiple, spontaneous and asymptomatic blisters on
hands and feet are indicative of anesthetic hands and
feet. Callosities may be present on both palms and
soles.
Vulnerable pressure points on palms, soles and bony
prominences like lateral malleoli, heel of the
hand(pisiform bone) and point of elbow should be
inspected for trophic ulcers.
Trophic ulcers should be examined carefully for
evidence of secondary infections( pus discharge, foul
smell and slough)
52. Icthyosis – widespread dryness indicates lack
of sweat and sebum secretion (autonomic
neuropathy)
53. MUCOSAL EXAMINATION
Lips and oral mucosa :
1. Diffuse engalrgement of lips may be a part of
infilteration in LL or type 1 reaction involving a BT
lesion overlying lips and surrounding areas.
2. Nodular infiltration of lip, tongue, palate and uvula may
be seen in patients of LL.
3. Fissuring of tongue –LL
4. Uvula- intact/partial or complete destruction, uvular
movements normal or restricted(fibrosis)
Nasal mucosa
1. Nasal examination done with artificial light and nasal
speculum
2. Mouth breathing, foul smell indicates nasal crusting.
3. Removal of crusts facilitates better visualization of
mucosa.
4. Nasal mucosa is insensitive, pale, thickened and
irregular with destrction of inferior turbinates.
54. OCCULAR EXAMINATION
Patient’s face should be inspected closely for :
1. Any skin lesion involving the peri-ocular area.
2. Frequency of blinking, blink interval (normally 6
per minute) is indicative of preserved corneal
sensation
3. Width of palpepral fissure
4. Photophobia, redness and watering of eyes.
Take the opinion of an opthalmologist in suspected
cases of eye involvement.
60. Schwann cells play a host to M.leprae.
Not all nerves get affected in leprosy.
Only the peripheral nerves bear the brunt of damage , that too limited
to certain segments of the nerve.
Sites of predilection of nerve damage -where the nerves are most
superficial, without the cover of muscle mass and hence cooler.
Localisation of M.leprae in the segments having low temperature.
At these sites, nerves pass through tough fibro osseous tunnels. Any
inflammation here presses upon the nerve supply of the fibres to result
in ischemia and damage. Local inflammation results in nerve
thickening.
Sudden increase in inflammation and inflammatory edema in nerve
gives rise to pain or nerve tenderness
Therefore, thickening of nerves with or without tenderness on palpation
–very important.
61. Done with the pulp of fingers and not tip of digits .
Both sides to be compared. While palpating the nerve , following
points are noted:
1. Enlarged or not ?
2. Is the nerve compressible? Lack of compressibility indicates a
fibrosed nerve .
3. Unilateral /bilateral? (asymmetric nerve enlargement –
borderline)
4. Extent of nerve palpable in its course.
5. Regularity –smooth or regular. If swelling present – its extent,
nature-uniform, sausage shaped or beaded, whether the
swelling is solid or fluctuant.
6. Nerve tenderness – indicative of neuritis.
78. Sensory testing
Semmes–Weinstein monofilaments (SWM) to measure light-
pressure thresholds
Standard clinical sensory tests employing cotton wool,(tactile),
pinpricks(pain), and tubes containing hot and cold water(thermal).
Semmes-weinstein (SW) monofilaments – nylon monofilaments
made of polyhexamethylene dodecanediamine (nylon 612). Set of
6 monofilaments depending on the force they produce.
Green -0.05 gm- normal sensation
Blue- 0.2 gm- diminished light touch
Purple- 2gm- diminished protective sensation
Red- 4gm- loss of protective sensation for hands
Orange-10gm - “ “ “ “ for feet
Light red- 300gm- deep pressure
79. Applied perpendicularly with mild pressure until
there is a bent-c curve .
Start with the finest touch (green) and built up to
orange.
Reference values to which normal indivisuals to
respond are: 0.05gm (green) for face ,0.2gm
(blue) for hand and 2gm (purple) for the foot.
80. Temperature testing
2 Test tubes
Cold sensation- containing water around 5 to
10 °C
Hot sensation- water around 37 to 47°C
Alterations in thermosensation - warm
hypoaesthesia, or cold hypoaesthesia.
86. MOTOR SYSTEM
EXAMINATION
Inspection of patient as a whole , then detailed
examination
1. Face(signs of facial pasly, leonine facies etc)
2. Upper extremities (obvious deformities like
claw hand, wrist drop, wasting of muscles,
shortening of digits etc)
3. Lower extremeties ( abnormal gait- “high
stepping gait”, foot drop, claw toes, collapsed
arches, shortening of toes,wasting of muscles
etc)
87. VOLUNTARY MUSCLE TESTING(VMT)
Assessment of functions of muscles of forearms,
hands, legs and feet (strength or power)
6 grades( medical research council scale):
Grade 5- normal power (with full resistance)
Grade 4- muscle contraction against slight
resistance but power subnormal
Grade 3- movement possible without resistance
Grade 2- active movement when gravity is
eliminated
Grade 1- flicker of movement
Grade 0- no movement
90. RADIAL NERVE
The radial nerve (and its deep branch) provides motor innervation to the muscles in
theposterior compartment of the arm and forearm, which are mostly extensors.
radial nerve proper
triceps
anconeus
ECRL
ECRB
brachioradialis
PIN
ED
supinator
EDM
ECU
APL
EPL
EPB
EI
91.
92.
93.
94. MEDIAN NERVE
Motor branches
Arm -Pronator Teres
Forearm -Supf Flexors –muscles of superficial
compartment- Pronator Teres / FCR / PL /
FDS) ; Ant Interosseus N -deep
compartment-FDP / FPL / Pronator Quadratus
Hand - Thenar Eminence / Lateral Lumbricals
(02)
95.
96. 1. FPL : Flexion of the Terminal Phalanx of 1 digit ;
Pt asked to flex terminal phalanx of thumb while base
is steadied by the examiner.
106. 2.INTEROSSEI
DORSAL (ABDUCTION OF FINGERS)
EGAWA TEST - Pt is asked to abduct fingers against
Examiner’s resistance; individually tested ; Palm facing
downwards
107. PALMAR
CARD TEST - With palm facing upwards, patient is asked to
hold card between 2 fingers tightly against the pull exerted by
Examiner
108. 3. 1st Palmar Interossei + Adductor
Pollicis – BOOK TEST
Pt is asked to hold a book between extended thumb and fingers
with both hands against pulll by the Examiner ; FROMENT’S
SIGN + when pt pinches grasp by flexing
thumb, using FPL.
113. COMMON PERONEAL NERVE-
lateral and anterior compartment
Superficial peroneal Deep peroneal
• Anterior
compartment of leg
• Tibialis anterior
• EHL
• EDL
• Dorsiflexion &
extension of toes
• Lateral
compartment of leg
• Peroneus longus
• Peroneus brevis
• Eversion and
plantar flexion
114. CRANIAL NERVES
12 cranial nerves
But in leprosy , only involvement of :
olfactory nerve
peripheral branches of trigeminal nerve
(supratrochlear, supraorbital)
Peripheral branches of facial nerve
(zygomaticotemporal, buccal, mandibular,
cervical)
Most common facial>olfactory>trigeminal
115. Olfactory nerve
Tested by having bottles containing
characteristic substances such as
peppermint, coffee or lavender and asking the
patient to identify each in turn. If you do not
have such tools available, ask the patient to
close his/her eyes and then hold a bar of soap
under the patient's nose for them to smell.
116. Trigeminal nerve
Opthalmic (purely sensory)
Maxillary(“ “)
Mandibular (sensory and motor)
Sensory supply- one half of the face
Motor supply: temporalis
tensor tympani
masseter
tensor veli palitini
lateral /medial pteregoids
117.
118. Sensory part -
1. One half of the face
2. Corneal reflex -
• Touch the cornea with
cotton wisp from lateral
aspect.
• Affarent – opthalmic
division of trigeminal nerve
• Efferent – branch of facial
nerve supplying orbicularis
oculi.
119. Absence of corneal reflex – lesions of Vth
Or VII th CN, and loss of connection btw these
nerves.
120.
121. Motor part-
Testing of temporalis and masseter –
Ask the patient to clench the teeth, and palpate
the muscles above and below zygomatic arch.
Compare the strength of both sides.
Lateral pterygoids –
lateral movement of jaw against resistance.
125. Testing the muscles of facial
expressions
1. Frontal belly of occipitofrontalis
Ask the patient to look upwards.
Wrinkling of forehead
2. Orbicularis oculi
ask patient to closes his eyes tightly.
Unable to do so , in case of palsy.
3. Buccinator
ask patient to blow the cheek .
126. 4. Platysma
Ask pt to show the teeth with mouth open
Prominence of platysma
5. Loss of nasolabial fold
6. Deviation of angle of mouth to normal side
– drooling of saliva
7. Labial dsyarthria – slurring of speech
127.
128.
129. External genitalia examination
In males, testes palpated to see for –
1. Size and consistency
2. Testicular sensation
3. Tenderness