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Orthopaedic complications of leprosy

Article  in  The Bone & Joint Journal · November 2005

DOI: 10.1302/0301-620X.87B10.16596 · Source: PubMed

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P. Moonot,
N. Ashwood,
D. Lockwood
From University
College London
Hospital NHS Trust,
London, England

P. Moonot, MRCS,
MS(Orth), Orthopaedic
Research Fellow
SWLEOC, Epsom & St.
Helier’s NHS Trust, Wrythe
Lane, Carshalton, Surrey
SM5 1AA, UK.

N. Ashwood, FRCS(Orth),
Orthopaedic Consultant
Queen’s Hospital, Belvedere
Road, Burton upon Trent,
Staffordshire DE13 0RB, UK.

D. Lockwood, MD, FRCP,
Consultant Physician &
Reader in Tropical Medicine
London School of Hygiene &
Tropical Medicine, Keppel
Street, London WC1E 7HT,
UK.
Correspondence should be
sent to Mr P. Moonot; e-mail:
drmoonot@yahoo.co.uk
©2005 British Editorial
Society of Bone and
Joint Surgery
doi:10.1302/0301-620X.87B10.
16596 $2.00
J Bone Joint Surg [Br]
2005;87-B:1328-32.
1328

ANNOTATION
Orthopaedic complications of leprosy
Worldwide leprosy remains a common prob-
lem with 750 000 new cases being diagnosed
each year. About 30% of patients have estab-
lished nerve damage at the time of diagnosis
and orthopaedic problems may therefore
develop even after successful antibiotic treat-
ment. Orthopaedic complications in leprosy
include altered bony architecture and stress
leading to repeated local trauma which, in
combination with nerve damage, causes plan-
tar ulcers.
Leprosy still causes considerable long-term
morbidity in both the developing and devel-
oped world. We discuss the orthopaedic com-
plications and management of leprosy.
Leprosy is an infectious disease caused by
Mycobacterium leprae. It is characterised by
skin lesions and peripheral nerve damage. It
may cause physical disability and disfigure-
ment. The global health costs cannot be easily
calculated.
It was Hansen1 in 1873 who discovered M.
leprae, the first bacterium to be identified as
causing disease in humans.
There are over 4 million individuals who
have or are disabled by leprosy worldwide and
about 120 cases in the United Kingdom under
regular observation at The Hospital of Tropi-
cal diseases, with from 15 to 20 new cases
diagnosed per year. With increasing migration,
it has become more important to understand
this disease since patients may present with the
disease or its complications outside endemic
areas. The diagnosis may be delayed in non-
endemic areas like the United Kingdom and
this may lead to permanent nerve damage and
disability.
M. leprae is an acid-fast rod and grows best
in cooler tissues (the skin, peripheral nerves,
anterior chamber of the eye, upper respiratory
tract, and testes).
Patients with untreated lepromatous leprosy
discharge bacilli from the nose.2 The principal
portal of entry into the human body is the
upper respiratory tract. M. leprae cannot
traverse through intact skin in either direction,
and the infection is not spread by touching.
The incubation period is two to five years in
tuberculoid disease and eight to 12 years in
lepromatous disease.2 In contrast to tuberculo-
sis, co-infection with HIV has no strong effect
on the development of leprosy.3
Pathology
M. leprae has a predilection for Schwann cells
and skin macrophages and is taken up by these
cells early in infection (Fig. 1).2
In many cases (indeterminate leprosy), early
lesions heal spontaneously with eradication of
bacilli. If the bacilli persist and multiply in the
Fig. 1
Mycobacterium leprae seen, on acid-fast
bacilli stain, as acid-fast rods with parallel
sides and rounded ends.
THE JOURNAL OF BONE AND JOINT SURGERY
 ORTHOPAEDIC COMPLICATIONS OF LEPROSY
Peripheral nerve lesion
Anaesthesia
Muscle imbalance
Ulceration
Excess trauma
Osteomyelitis
Deformity
skin and/or nerves, established leprosy develops.4 In tuber-
culoid leprosy there is involvement of skin and nerves. Lym-
phocytes breach the perineurium, and destruction of the
Schwann cells and axons may be evident, resulting in fibro-
sis of the epineurium, replacement of the endoneurium with
epithelial granulomas, and occasionally caseous necrosis.
Acid-fast bacilli are few or absent. Such invasion and
destruction of nerves in the dermis by T lymphocytes is
pathognomonic for leprosy.5 Sometimes a caseous abscess
may form inside the perineural sheath causing paralysis of
the nerve.
Lepromatous disease manifests with generalised involve-
ment of skin, nerves and mucous membranes. The damage
and hypertrophy of nerves resulting from bacillary invasion
tends to be symmetrical and is more insidious and extensive
than in tuberculoid disease. The granulomatous lesions
contain macrophages and abundant bacilli.
In skin lesions, the small dermal sensory and autonomic
nerve fibres are damaged. This can lead to glove and stock-
ing paraesthesiae and loss of sweating. Damage to peri-
pheral nerves leads to sensory loss and paralysis.2 Nerve
damage occurs across the leprosy spectrum.
Acute nerve pain with associated loss of function may
occur as part of a leprosy reaction, an acute immunological
reaction, which presents with inflamed skin lesions and
neuritis.
Immunology
The patient’s immune response to M. leprae determines the
features of the disease: the two poles are tuberculoid (pauci-
bacillary) and lepromatous (multibacillary) leprosy. At the
tuberculoid pole, well-expressed cell-mediated immunity
and delayed hypersensitivity control bacillary multiplica-
tion, which limits the disease to a few well-defined skin
lesions or nerve trunks. Few mycobacteria are found in the
lesions. In the lepromatous form, there is cellular anergy
towards M. leprae, resulting in abundant bacillary multi-
plication. There is uncontrolled proliferation of bacilli with
many lesions and extensive infiltration of the skin and
nerves.
VOL. 87-B, No. 10, OCTOBER 2005
1329
Fig. 2
Diagram of the pathophysiology of deformity.
Between these two poles lie different groups. These forms
are characterised by a progressive reduction in cell-medi-
ated immunity from borderline tuberculoid to borderline
lepromatous leprosy in cellular responses, associated with
an increasing bacillary load and increasing number of skin
and nerve lesions.
The intra-osseous lesions of leprosy are characterised by
granulomatous tissue reaction that lead to trabecular
destruction. The lesions are evident in the epiphysis and
metaphysis of tubular bones, and direct involvement of the
medullary canal can also occur.
Classification
Classification of patients according to the Ridley and
Jopling6 scale is clinically useful with a spectrum from
tuberculoid to lepromatous leprosy.
There is also a simpler classification determined by the
number of skin patches: single skin lesion (one patch),
paucibacillary (two to five patches) and multibacillary
(more than five patches). Patients with multibacillary lep-
rosy are more likely to have nerve damage.7
Clinical manifestations
The signs of leprosy are skin lesions, which are typically
anaesthetic at the tubercoloid end of the spectrum, thick-
ened peripheral nerves and eye involvement, which may
lead to blindness. In lepromatous leprosy, the skin lesions
are nodules, papules, or plaques with predilection for the
face, wrists, elbows, buttocks, and knees. Involvement of
major nerve trunks is common and there is also glove-and-
stocking anaesthesia in the extremities.
Patients with borderline disease have multiple skin and
nerve lesions. The pathological effects of leprosy on the
skeleton are primarily a consequence of neuropathy leading
to denervation, direct bony changes, secondary infection
and the sequelae of trophic ulcers (Fig. 2).
The following superficial peripheral nerve trunks may be
palpably hypertrophied: facial, great auricular (neck), ulnar
(elbow), median (wrist), radial cutaneous (wrist), lateral
popliteal (neck of fibula), and posterior tibial (medial mal-
1330 P. MOONOT, N. ASHWOOD, D. LOCKWOOD

Table I. Peripheral nerve involvement in leprosy

Nerve Motor loss Sensory loss

Ulnar nerve
Median nerve
Radial nerve (rare)
Common peroneal nerve
Posterior tibial
Clawing of ring and little fingers,
loss of intrinsic muscle function
Loss of thumb opposition and grasp
Wrist drop
Foot drop
Clawing of toes
Anaesthesia over the medial two fin-
gers of the hand
Anaesthesia over the thumb
Anaesthesia of first web space
Anaesthesia over the lateral malleolus
Anaesthesia of the sole of the foot

Inflammatory, symmetrical peripheral polyarthritis of

insidious onset with a pattern of exacerbation and remis-
sion, is occasionally seen.9 The wrist, metacarpals and
proximal interphalangeal joints of the hands, the knees and
the metatarsophalangeal joints are involved with morning
stiffness lasting up to one hour.
Patients with leprosy can present with skin lesions,
peripheral neuropathy, ulceration, soft-tissue infections,
and osteomyelitis, deformities of the hands and feet, and
joint involvement. They can present to a dermatologist,
neurologist, rheumatologist or an orthopaedic surgeon.
Diagnosis. The principal criteria for the diagnosis are4 a
hypopigmented patch of skin with sensory impairment,
thickening of the peripheral nerves, the identification of M.
leprae in slit-skin-smears, which are graded on the Ridley
Fig. 3
and Jopling bacteriological index as 1+ to 6+, and charac-
teristic histopathological changes in a biopsy specimen of a
Photograph showing clawing of the fingers and a trophic ulcer
skin lesion or a peripheral nerve.

Bone changes in leprosy

leolus). The most commonly-affected is the posterior tibial
nerve followed by the ulnar nerve at the elbow, whose
involvement results in clawing of the fourth and the fifth
fingers, and loss of dorsal interosseous musculature and
loss of feeling in the ulnar nerve distribution (Fig. 3).
Median nerve involvement impairs thumb opposition and
grasp, while radial nerve dysfunction, though rare in lep-
rosy, results in wristdrop.
Peroneal nerve palsies may result from leprosy itself or
from one of its reactional states. It leads to a partial or com-
plete foot drop, which causes an uneven distribution of
weight on the plantar surface and hence a predilection to
ulceration (Table I).
Repeated trauma in the absence of protective feeling may
cause trophic ulcers which may become secondarily
infected. In severe cases osteolysis of the terminal phalanges
can occur.
Plantar ulceration, particularly at the metatarsal heads,
is probably the most frequent complication of leprous neu-
ropathy,4 and secondary infection leads to cellulitis and
osteomyelitis.
In the face, hands and feet, there may be direct osseous
involvement due to extension of infection from overlying
dermal or mucosal areas. The periosteum is infected ini-
tially (leprosy periostitis) and subsequently the cortex and
medulla are infected (leprosy osteitis and osteomyelitis). At
the fingers and the toes it may appear as dactylitis.8
These can be divided into two groups, specific and second-
ary changes. Specific lesions are due to the direct involve-
ment of bone by the organism, whereas secondary lesions
are the result of trauma and infection (Fig. 4) imposed upon
denervated tissues. Secondary bony changes are commonly
observed and are therefore discussed first.
Secondary bony change. These are common in leprosy.
Localised osteoporotic changes result from immobilisation,
most frequently because of disuse associated with fixed
contractures of the fingers.
Motor denervation is sometimes associated with absorp-
tion of the cancellous bone and the development of a con-
centric type of bone atrophy. It affects the length, the width
or both. The most common changes in leprosy however are
those due to combined absorption of length and width of
bone. The result is a tapered appearance at the end of the
bone, termed ‘licked candy stick’.
Changes due to distal absorption affect the ends of insen-
sitive fingers and toes. When this process is complicated by
infection, it may be followed by progressive absorption
with loss of the digits and the development of the so-called
‘mitten hand’.10
Absorption secondary to trauma and infection has three
common sites of predilection in the insensitive foot. The
first is the distal type affecting the tips of the toes. In the sec-
ond type the metatarsophalangeal joint is at risk of damage
and the third involves the tarsus.

THE JOURNAL OF BONE AND JOINT SURGERY

 ORTHOPAEDIC COMPLICATIONS OF LEPROSY
Fig. 4
Radiograph showing osteomyelitis of the calcaneum secondary to plantar
ulceration.
The common paralytic deformities of the foot, which are
potentially damaging to the metatarsophalangeal joints, are
clawed toes and drop foot. Static deformities such as hallux
valgus, metatarsus primus varus and pes planus are also
associated with plantar ulceration, which may lead to bone
absorption.10 Tarsal disintegration is not infrequent and
may involve one or more tarsal bones. The most common
tarsal disintegration affects the medial arch. The lateral
arch is less commonly involved and occurs as a late compli-
cation of a rigid deformed foot.10
Specific bony changes. Specific bony lesions in leprosy are
rare with an incidence of between 3% and 5% among hos-
pitalised patients,11 and are mainly confined to the small
bones of the face, hands and feet. These lesions are charac-
terised by granulomatous tissue reactions, which are
destructive and manifest radiographically as focal areas of
increased rarefaction. The margins are thin, but may be
sclerotic. Obliteration of the cavity may result from its col-
lapse with flattening of the articular surface.
In the hands and feet, the disease mainly involves the
proximal and/or the middle phalanges. It may present as
thinning of the endosteum with corresponding widening of
the medullary canal localised to the area of the metaphysis.
Fusiform swelling of the soft tissues overlying the corre-
sponding part of the affected digit is more common.10 This
is occasionally associated with enlarged nutrient foramina.
Leprous osteitis in the hands commonly involves the dis-
tal ends of the proximal and middle phalanges, whereas it
usually affects the metatarsal heads in the feet. Because of
weight-bearing forces, there may be comminuted patho-
logical fractures of the metatarsal heads.
VOL. 87-B, No. 10, OCTOBER 2005
1331
Should the disease progress and the trabeculae be
destroyed, the radiographs reveal a ‘honeycomb and cystic’
appearance. With healing, radiographic changes become
sharply defined as cysts with sclerotic margins.
The articular surface can also be involved and the intrin-
sic forces in the hand may result in fracture, subluxations
and rigid clawing of the fingers.10
Prevention and treatment
It is vital to prevent the development of nerve palsies and
ulcers. Primary prevention is by early detection and the use
of antileprosy drugs. Secondary prevention is by health
education and self-awareness of the patient; hygiene of the
anaesthetic foot with attention to cracks and fissures, early
detection of tenderness and adequate rest; awareness of
potential injury mainly during work and cooking; appro-
priate foot wear.
Antibacterial. Multidrug therapy is the mainstay of treat-
ment. The current multidrug therapy recommended by the
World Health Organisation in adults with multibacillary
disease is rifampicin, 600 mg orally once a month, dapsone
100 mg orally daily, and clofazimine, 300 mg orally once
monthly and additionally 50 mg daily. The WHO recom-
mends that it be continued for 12 months but patients with
initial high bacterial loads may need longer treatment. For
paucibacillary disease (bacteriological index, 2+) the regi-
men is rifampicin, 600 mg orally once monthly, and dap-
sone, 100 mg orally daily for six months.
Steroid treatment for nerve damage. Any patient who devel-
ops peripheral nerve damage during the last six months of
treatment should receive a four- to six-month course of oral
steroids.
Physiotherapy. There is usually stiffness of the fingers.
Physiotherapy with active exercises, massage, passive
stretching and wax baths and splinting should be under-
taken before giving consideration to tendon transfer pro-
cedures.
Monitoring of nerve damage. Impairment of nerve function
can occur before diagnosis and during or after multidrug
therapy. Patients with multibacillary leprosy and pre-exist-
ing nerve damage are at the highest risk of impairment of
nerve function during and after treatment. These patients
should be under surveillance for two years from diagnosis.
Regular clinical evaluation should include nerve palpation,
motor testing of the small muscles of the hand and record-
ing of feeling in the hands and feet using the Semmes-Wein-
stein nylon monofilaments.12 If new nerve involvement is
detected steroid treatment should be started.
Surgical treatment. Surgery has an important role in the
management of motor imbalance, chronic ulceration,
chronic infection and the correction of soft-tissue and bony
deformities.
Decompression of nerves, such as the ulnar or common
popliteal in the early stages may prevent the development of
paralysis. Steroids may also be given to reduce inflamma-
tion.
1332 P. MOONOT, N. ASHWOOD, D. LOCKWOOD
Fig. 5
Photograph showing plantar ulcers with active infection.
Permanent claw-hand deformities can be treated by ten-
don transfers. These procedures should not, however, be
undertaken until six months after the start of antimicrobial
therapy and the conclusion of episodes of acute neuritis.5
The treatment of plantar ulceration includes debride-
ment of devitalised tissue, non-weight-bearing by means of
a total-contact cast or bed rest and the vigorous treatment
of secondary infection, which is most commonly caused by
Staphylococcus aureus (Figs 5 and 6). The ulcers should be
examined for infection and probed to detect any bony
involvement. If there is slough or necrotic material, it
should be thoroughly debrided. Bony involvement may
require multiple debridements and sometimes excision.
Leprous ulcers heal well if weight-bearing is prevented as
they have good vascular supply unlike those in diabetic
neuropathy. Once healing takes place, walking must be lim-
ited and only increased slowly. Extra-depth shoes or cus-
tom-made shoes with moulded inserts are required to
prevent recurrence. Areas of bony prominence which may
lead to ulceration should be removed surgically. Amputa-
tion may be indicated if there is gross destruction of bones
and joints.
Foot drop can be initially treated by simple splints. Sub-
sequent tendon transfers may be required. A neuropathic
foot may require more extensive surgery in the form of cor-
rective osteomy, arthrodesis or amputation.
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Fig. 6
Photograph showing plantar ulcers after thorough debri-
dement and use of a total-contact cast and rest
Conclusion
Leprosy is rare in the United Kingdom. Early diagnosis and
treatment is required in order to prevent nerve damage. A
history of travel from an endemic region, a skin rash and/or
neurological symptoms, and possibly a history of joint
symptoms should raise the suspicion of the diagnosis of lep-
rosy.
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THE JOURNAL OF BONE AND JOINT SURGERY