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TOPICS
Introduction
Historical perspective Risk Factors Etiology Pathology Classification Clinical features Differential Diagnosis Management
Diagnosis Treatment
General Medical Surgical
INTRODUCTION
Chronic infectious disease caused by
HISTORICAL PERSPECTIVE
Armauer Hansen in 1873
(Norway). First Bacterium to be associated with exclusive human chronic disese. India is considered the starting point with Skeletal evidence dating back to 2000 B.C. Leprosy Act of 1898 in Colonial India to institutionalize and prevent spread
RISK FACTORS
10 20 % of patients develop significant disability.
1.
PATIENT FACTORS
Sex Male > Female in severity and frequency Age Elder > Young in risk of disability Race White > Dark Skinned Puberty, Pregnancy and parturition Type Lepromatous > Tuberculoid Duration - Longer Number of nerve trunks involved - > 2 > 0-2 Reactions Increase the risk of nerve damage five fold
2.
DISEASE FACTORS
3.
ENVIRONMENTAL FACTORS
ETIOLOGY
Mycobacterium Leprae. Extra and intra
cellular
Gram Positive, acid and alcohol fast organism Man is the Sole reservoir Host Not possible to cultivate bacteria in In vitro medium
Highly infections. Low pathogenic Attack rate- 4.4 to 12% symptomatic in 5 yrs
Transmission :Lepromatous leprosy (>1011 Bacteria) Nasal discharge Broken Skin, Nodules, rarely through sweat and sebaceous glands Intrauterine ?? Breast Milk Very Rarely bites of arthropods and tattoo needles
PATHOLOGY
Chronic Granulomatous disease. Incubation Period : 2- 10 yrs Types:
1. Indeterminate/ Early Leprosy 2. Established forms
a) Tuberculoid b) Lepromatous c) Borderline
3. Borderline reactions
EARLY/INDETERMINATE LEPROSY
Lymphocytic infiltration without granuloma formation Epidermis, Sweat Glands, arector pili muscle, hair follicles and neurovascular bundles with few scanty perivascular lymphocytes with or without demonstrable bacilli
TUBERCULOID LEPROSY
Good CMI and DTH to bacilli and its antigens Epitheloid cell granuloma Epithelial cells and langerhans giant cells (also in BT undergoing upgrading reaction)
LEPROMATOUS LEPROSY
Absence of CMI and DTH Diffuse and spreading Macrophage Granuloma Cut onion dermal nerves (perineural) Foamy macrophages (fat vacuoles) Few clusters of lymphocytes LARGE number of AFB in macrophages, nerve bundles, endothelial cells and arector pili Band like infiltration in superficial dermis Flattening and atrophy of rete
BORDERLINE LEPROSY
Indeterminate CMI and DTH Borderline tuberculoid (BT) Midborderline(BB) Borderline lepromatous (BL)
Transition Epitheloid granuloma (BT) to Macrophage granuloma (BL) with progressive increase in number of bacilli and macrophages with decrease in lymphpcytic infiltraion
BORDERLINE REACTIONS
Upgrading reaction/Type I
Increase in CMI-DTH status Well formed compact granuloma approximating epidermis with free sub epidermal zone Increase in epitheloid cells and lymphocytes with decrease in bacilli
o
Decrease in CMI-DTH Loosening of granuloma pushed down to dermis with free sub epidermal zone Increasing number of macrophages and AFB Dilatation of upper dermal blood vessels, with perivascular neutrophilic infiltration of lower dermal and subcutaneous vessels Extravastation of RBCs, scanty granular AFBs and MICROABSCESS
Tuberculoid
Borderline
4 or more lesions others same as above. Usually progresses to lepromatous. Margins well defines BT, Partially defined BB, ill defined BL. Nerves enlarged tender firm to soft
anaesthetic
Lepromatous
Diffuse infiltration, b/l symm. Hypo pigmented or coppery red. Ill defined.. Nerves thickened, soft, tender
Pure neuritic
negative
WHOclassification (treatment)
Paucibacillary All smear -veTT, BT and indeterminate Multibacillary Smear +ve TT, BT, indeterminate. ALL BB, BL and LL
RIDLEY JOPLING ( immuno histological) Tuberculoid TL Borderline Tuberculoid BT Borderline BB Borderline Lepromatous BL Lepromatous LL
CLINICAL FEATURES
SYMPTOMS Painless skin patch accompanied by loss of sensation but not itchiness (Loss of sensation is a feature of tuberculoid leprosy, unlike lepromatous leprosy, in which sensation is preserved.) . Loss of sensation or paresthesias where the affected peripheral nerves are distributed Wasting and muscle weakness Foot drop or clawed hands (may result from neuritic pain and rapid peripheral nerve damage;) Characteristic clawed hand deformity caused by ulnar involvement in leprosy.
o Ulcerations on hands
or feet (ulcer at the metatarsal head is seen in the image below) Lagophthalmos,
iridocyclitis, corneal ulceration, and/or secondary cataract due to nerve damage and direct bacillary skin or eye invasion
`
Symptoms in reactions
Type 2 (erythema nodosum leprosum [ENL]; as seen in the image below) Many skin nodules, fever, redness of eyes, muscle pain, and joint pain
Patient with erythema nodosum leprosum type 2 reaction several weeks after initiation of drug therapy. Travel: Leprosy should be
considered in anyone who has lived in the tropics or who has traveled for prolonged periods to endemic areas.
Exposure: The incubation period of leprosy is long, ranging from a few months to 20-50 years. The mean incubation time is estimated to be 10 years for lepromatous leprosy and 4 years for tuberculoid leprosy. The organism's slow dividing time (once every 2 wk) contributes to the challenge of epidemiologically linking exposures to the development of disease. Because of immunologic reasons, only around 5-10% of the population is estimated to be susceptible to infection
CLINICAL FEATURES
Physical examination:The first physical signs of leprosy are usually cutaneous.
Tuberculoid leprosy
The initial lesion is often a sharply demarcated hypopigmented macule that is ovoid, circular, or serpiginous. The lesions may be somewhat elevated with a dry scaly center and erythematous borders. Common lesion sites include the buttocks, face, and extensor surfaces of limbs. The perineum, scalp, and axilla are not normally involved because of the temperature differential in these zones, as predilection is toward cooler zones. Destruction of normal skin organs such as sweat glands and hair follicles. Superficial nerves enlarge and are sometimes palpable. Sometimes severe neuropathic pain. Nerve involvement can also lead to trauma and muscle
Lepromatous leprosy Extensive bilaterally symmetric cutaneous involvement, which can include macules, nodules, plaques, or papules. Poorly defined borders and raised and indurated centers.Lepromatous lesions are worst on cooler parts of the body. Common areas of involvement include the face, ears, wrists, elbows, buttocks, and knees. Hoarseness, loss of eyebrows and eyelashes, and nasal collapse secondary to septa perforation may occur in advanced disease. The leonine facies associated with leprosy Axillary and inguinal adenopathy may develop, in addition to scarring of the testes and subsequent gynecomastia and sterility. Nerve involvement in lepromatous leprosy is not as severe as in tuberculoid leprosy, since nerves, although visibly thickened and highly
-Multiple flat hypopigmented lesions on shoulder and neck, suggestive of multibacillary leprosy. Note ulceration of hypothenar area of hand, indicative of ulnar neuropathy
Involvement of the eye may include keratitis, glaucoma, or iridocyclitis as seen in the image below. Man with advanced deformities caused by unmanaged leprosy. Keratitis, loss of eyebrow, thickened skin, and typical hand impairments.
MANAGEMENT
DIAGNOSIS The WHO case definition of leprosy is M leprae infection in an individual who has not completed a course of treatment and has one or more of the following:
Hypopigmented or reddish skin lesions with loss of sensation Involvement of the peripheral nerves as demonstrated by their thickening and associated loss of sensation Skin smear positive for acid-fast bacilli
Lab Investigations
Skin biopsy, nasal smears, or both are used to assess for acid-fast bacilli using Fite stain. Biopsies should be full dermal thickness taken from an edge of the lesion that appears most active.
Serologic assays can be used to detect phenolic glycolipid-1 (specific for M leprae) and lipoarabinomannan (commonly seen in mycobacteria).
Molecular probes detect 40-50% of cases missed on prior histologic evaluation. Since probes require a minimum amount of genetic material (ie, 104 DNA copies), they can fail to identify paucibacillary leprosy. Laboratory tests related to drug treatment follow-up include the following:
CBC count Creatinine level Liver function tests
Bacterial Index number of bacilli in oil immersion fields (OIF) in an average microscope Help in monitoring
0 1+ 2+ 3+ No bacilli 100 OIF 1 or<1 in each mic field Bacilli found in all the fields Many bacilli in all the fields
Bacterial Index= total of indices / number of sites examined Total number of + in 7 sites - 4 skin lesions, 1 nasal swab, both ear lobes --- Pauci bacillary 2 --- Multibacillary>2
Phenolic glycolipid-1:
detection of antibodies to phenolic glycolipid-1. sensitivity of 95% for the detection of lepromatous leprosy but only 30% for tuberculoid leprosy.
Treatment
GOALS
1. 2. 3.
To interrupt transmission and reduce incidence To treat patients.. cure.. rehab To prevent formation of associated deformities
Medical treatment
Paucibacillary leprosy should be treated for 6-12 months. followed by treatment with dapsone as monotherapy for 3 years in patients with tuberculoid leprosy or 5 years in patients with borderline lepromatous leprosy. Multibacillary leprosy should be treated for 24 months Prednisolone is believed to minimize pain and acute inflammation. The recommended initial dose is prednisolone 40 mg daily. Newer drugs1. 2. 3. 4. 5.
Fluoroquinolones oflox, perflox Minocycline Macrolides clarithro, roxithro Ansamycins other than rifampicin Dihydrofolate reductase inhibitors - Brodimoprim
Surgical Management
Depending upon the complications
COMPLICATIONS
Reactions Neuritis Eye complications Ulcers
Hand deformities
Foot deformities
Systemic Complications
Lepra reaction
TYPE 1 Reversal reaction TYPE 2 ENL Relapse
Time interval
Pathology
Ulceration
Nerve involvement Gen condn Response to steroids
May
Multiple+ painful and tender.Loss of nerve function. Silent neuritis quite nerve paralysis May have fever jt pains. malaise Rapid
possible
Single nerve. Painful and enlarged Fever, conjunctivitis, keratitis Nil. Use Clofazamine
Lepra reaction
LUCIO PHENOMENON:Latin America Pure diffuse polar lepromatous leprosy Extensive painful bizzare necrosis of skin with constitutional symptoms Variant of ENL Severe vasculitis. May be fatal
Treatment:Steroids Analgesics and Anti inflammatory like aspirin ANTILEPROSY MEDICATIONS ARE NOT STOPPED Thalidomide in retractable cases (males and postmenopausal women Cyclophosphamide Nerve:
Rest, Diathermy, Decompressing surgeries Steroids NOT proven
NEURITIS
ACUTE NEURITIS ANTI-REACTION
PART
PHYSICAL THERAPY WAX BATH,
FACTOR
DIVIDE EPINEURIUM RELIEVE THE FASCICLES FROM BEING
NERVE ABSCESS
COLD ABSCESS SURGICAL INTERVENTION
DRAINING THE ABSCESS MOPPING NECROTIC CONTENT REMOVING ADHERENT SLOUGH EXCISING THE ABSCESS
HOT ABSCESS SURGERY RARE
Hand in leprosy
Most frequently involved Primary impairments
Loss of sensibility is the most common primary impairment Ulnar paralysis Median nerve paralysis Rarely radial nerve paralysis
Secondary impairments
Ulceration Scar contracture Shortening of digits
Hand in leprosy
Deformities:Specific deformities:Neglected Lepromatour leprosy Banana Fingers or Sausage fingers Reactional episodes 1. Skin over the dorsum becomes atrophic, leathery and adherent to deeper structures 2. Non Paralytic Claw Hand d/t Contraction of dermal and subdermal collagen 3. INTRINSIC PLUS DEFORMITY d/t fibrosis of intrinsic muscles 4. Contractures 5. Pathological fractures d/t demineralisation in acute stage
Hand in leprosy
Deformities:Paralytic deformities: 1. 2. 3. 4. 5.
Observations:Ulnar paralysis most common and radial nerve paralysis is least common When median nerve is paralyzed it is almost always at distal level. Hence only hands are mostly involved High median nerve palsy very rare Median nerve palsy always associated with ulnar nerve palsy Radial nerve palsy associated with Ulnar and median nerve palsy
Hand in leprosy
Deformities:Ulnar palsy:- Partial clawing of ring and little finger. Complete clawing in Martin gruber anastomosis Wasting of hypothenar and interossei muscles and in few cases flattening of distal part of thenar eminence Z-Deformity of thumb Loss of adduction of thumb Combined palsy :- Complete clawing with thumb in hyperextended and supinated at the CMC jt Thenar flattening Triple palsy:- a/w Wrist drop
Hand in leprosy
Anesthetic Deformities:Leprosy Damage Injury Infection Healing with deformity
Anesthesis
Neglect
Anesthesia over medial two fingers Contractures Shortening Mutilation Disorganization Wrist disorganisation from base of hypothenar eminence usually from a pressure sore over pissiform bone
Hand in leprosy
Clinical features:Claw deformity is sever ONLY when both interossei and lumbricals are paralyzed BOUVIER BEEVOR PHENOMENON:- Long extensors can extend ip joints only when MCP stabilized IN ANY POSITION EXCEPT HYPEREXTENSION GUTTERING:- Extemsor tendon sliding off the knuckle on making a fist
o
o o
o
o
Flattening and wasting of hypothenar and thenar eminences Hollowing of intermetacarpal spaces most evident in thumb web Deviation of fingers towards ulnar side BEAKING of nail FLATTENING OF DISTAL METACARPAL ARCH
Hand in leprosy
Hypertrophic PIP knuckle pads, scarring over knuckle with BOUTONNIERE (HOODING), PSEUDOBOUTONNEIRE deformity. Rx:- Release of contracted lig + Z-plasty of extensor expansion + repair of central tendon Claw correction by volar capsulotomy + tendon transfer with pulleys Sever Fusion in 300 flexion
Hand in leprosy
Management
TIMING MDT for atleast 6months with good response. Without any episode of attack or exacerbations. Muscle paralysis >/= 1yr Operations for restoration of intrinsic function of fingers:1. 2. 3. 4. 5. 6. 7.
LASSO or Pulley insertion by FDS (mild uncomplicated) Riordan FCR (habitual flexing the wrist or flexion contracture) Modified Bunnell FDS ring Brand ECRL/ECRB (extensors > flexors) Fowler EIP or EDQP (FDS or Wrist flexor/extensor not available) Zancolli Capsulodesis (no muscle available) Fowler tenodesis
RIORDAN
MODIFIED BUNNELL
BRAND
FOWLERS
FOWLERS TENODESIS
ZANCOLLIS CAPSULODESIS
Hand in leprosy
Thumb in leprosy:Thumb in extension at basal joint (CMC) and flexion at MCP and IP joints When basal (CMC) joint stabilised by opponens, MCP extended or hyperextended and IP flexed When MCP joint also stabilised (by FPB) extension on distal phalanx improves Ulnar paralysis:1. Grip weakens (AdP) 2. Power pinch weak (FPB In
Hand in leprosy
Thumb in leprosy:Combined palsy:Only KEY GRIP SPARED
Adduction extension contracture (First web space) Capsular contracture of CMC
Hand in leprosy
Management:Restoration of Thumb Opposition
5.
6.
Riordan (Sublimis) Brand (Sublimis) Burkhalter(Extensor indicis Proprius) Groves and Goldner (Sublimis + FCU) Camitz (PL) Littler and Cooley (AbDQ)
o Restoration of Adduction 1. Boyes (Brachioradialis or Radial Wrist extensor) 2. Smith (ECRB) 3. Modified Royle thompson
RIORDAN BRAND
Hand in leprosy
BURKHALTER
BOYES
SMITH
Hand in leprosy
RADIAL NERVE PALSY:Wrist Drop and finger drop Not a common problem in leprosy (<1%) Almost always associated with ulnar and low median N. palsy ---- TRIPLE NERVE PARALYSIS Three types:1. 2.
3.
Complete High triple nerve paralysis Incomplete High triple nerve paralysis Classic triple nerve paralysis
Hand in leprosy
RADIAL NERVE PALSY:Wrist Drop and finger drop Not a common problem in leprosy (<1%) Almost always associated with ulnar and low median N. palsy ---- TRIPLE NERVE PARALYSIS Three types:1. 2. 3.
Complete High triple nerve paralysis Incomplete High triple nerve paralysis Classic triple nerve paralysis
Management:Two stage reconstruction I stage :- Stability of wrist finger and thumb in extension II Stage :- Claw correction and opponensplasty
Hand in leprosy
First Stage:-
Hand in leprosy
FIRST STAGE:1. 2. 3.
PL to EPL Release of 3rd extensor compartment - EPL translocates radially and brings extension and abduction as PL contracts In PL absence FCR to EPL and EDC Arthrodesis of wrist (not advised)
Only when forearm muscles are weak or paralyzed Wrist joint has fixed deformity with instability of wrist
SECOND STAGE:1. 2.
Claw finger:- FDS (middle finger) to dorsal extensor expansion Opponensplasty:- FDS(ring) by dual insertion technique
Volar capsulodesis MCP joint or dermadesis with pulley advancement for claw Extra articular bone block between first and second metacarpal bases Intraarticular trapeziometacarpal arthrodesis
Foot
oParalytic deformities:1. Lateral popliteal N. at the
level of neck of fibula leads to foot drop 2. Posterior tibial N. paralysis at the level of ankle jt leads to Claw toes
Foot
Foot Drop Management:EARLY cases:Chances for recovery high if <1yr Medical management is prime stay 1. Anti leprosy medication 2. Steroids 60mg/day of prednisolone tapered fornightly by 40mg, 30mg, 20mg 3. A foot raising strap or spring 4. Daily electric galvanic stimulation o Surgical management:o Indications: Worsening of symptoms No improvement by 2-3 wks of conservative rx o Surgical decompression of
Foot
Foot Drop Management:ESTABLISHED cases:Goals:
1. Stepping gait abolished 2. Prevent destruction of outer part of foot
o Surgical Correction 1. Tendon transfers in Supple foot 2. Corrective triple arthrodesis in equinovarus deformity
o Medial popliteal nerve is almost always never involved.
Hence Tibialis posterior is the most common tendon to be transferred o Peronei muscles secondary
Foot
Tibialis posterior tendon transfers:Obers Barr Srinivasan Carayon Dr. Grace Warren SRINIVASAN
Obers
Subcutaneous circumtibial transfer Attachment to third metatarsal base. Note:- Attachment to bone in leprosy is discarded because of precipitation of neuropathic tarsal disorganisation
Biomechanical advantage
Srinivasan
Subcutaneous circumtibial transfer Attachment to EDL and EHL. Peroneus tertius Foot in 700 dorsiflexion
Neglected drop foot Acquired equinovarus Maybe followed by tibialis posterior transfer
Foot
o
Claw toes:o o o o
Paralysis of posterior tibial and plantar nerves Plantar anesthesia leads to blisters and ulcer Paralysis of lumbricals and interossei Deformity:o o
o o
Less disability Grades :Mild - No contractures and supple joints II. Moderate - Flexion contracture at IP joints III. Severe - Flexion contracture and stiffness in hyperextension of MTP Subluxation and dislocation of MTP
I.
Foot
o
Claw toes:o
Mangement:o Surgical Correction o Grade I: Tendon transfer 1. Taylor (Short and long flexor to extensor expansion over proximal phalanx) 2. Forrester Brown a. FHL to EHL b. IP fusion c. EDL looped around metatarsal for remaining 3. Dixon and Diveley (EHL to FHL and IP fusion) 4. Parrish(FDL to midportion of proximal Phalanx) 5. Hibbs (Long extensor of lateral 4 toes to Lateral cuneiform) 6. STANDARD :- FDL to Extensor expansion of proximal Phalanx Garceau DWYER
HIBBS
DWYER
Foot
o
Claw toes:o
Mangement:o o o
PIP arthrodesis with k-wire Soft tissue release with MP capsuloplasty Resection of MT head Open reduction Proximal shift of long extensor tendon to MT neck IP arthrodesis Condylectomy of MT head Surgical Syndactylia
Grade III:ii.
iii. iv. v. vi. vii.
Systemic complications
Nose and para nasal air sinuses
Maggots
Ear
External, Middle and inner ear disturbances
Smooth muscle Dormant in scrotum and areola of breast Leprous osteitis Osteoporosis, Arthritis, Disorganization of bones Autonomic nervous system Cardiovascular (Myocarditis, endocardial elastosis etc.) Renal (Nephritis and amyloidosis) Adrenals (life threatening) Testes Mammary Glands
Physical Therapy:
For increasing/regaining ROM: ROM can be increased by soaking the skin or part in warm water and then performing passive movement to the part affected.
To improve strength specially in tendon transfer: Active
soap water, rubbing off thick skin, oiling, self massage and protecting the part from infection.
Home care: teaching skin, hand, foot and eye care to
groups and individuals and teaching the patients actual home care.
Protect tissue during healing: Rest, body position and POP cast.
HAND
LOSS OF FEELING MAKES PREVENTION MORE
DIFFICULT
EXERCISES TO PREVENT FIXED CLAWING OF
SPLINTAGE
NERVE INJURED
RADIAL NERVE PALSY ULNAR NERVE PALSY
SPLINT USED
COCK-UP SPLINT KNUCKLE BENDER SPLINT
COCK - UP SPLINT
GOOD EXERCISE TO PREVENT TIPTOE CONTRACTURES WITH FOOT DROP IS TO STRETCH THE HEEL CORDS BY LEANING FORWARD AGAINST A WALL OR BY SQUATTING WITH HEELS ON THE GROUND
THEIR FOOT - FOOT SUPPORT HERE MAY HELP TAKE PRESSURE OFF THE BALL OF THE FOOT AND PREVENT NEW SORES.
Appropriate economic rehabilitation is provided e.g. sewing machines, handcrafts, carpentry etc.
Sc