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Hong Kong J. Dermatol. Venereol. (2012) 20, 77-81
Case Report
Lepromatous leprosy: a case simulating verrucouscarcinoma
MM Chang and PCL Choi
A 59-year-old immigrant from China presented with a
rapidly-enlarging exophytic growth on hisleft heel for 4 months. He
was also found to have peripheral neuropathy, Charcot's joint,
diffuseerythematous papules and acquired ichthyosis. Classical
histologic features of leprosy were noted,together with atypical
verrucoid proliferation. The diagnosis of lepromatous leprosy was
madeafter clinical-pathological correlation and confirmation by
polymerase chain reaction.
Keywords:Keywords:Keywords:Keywords:Keywords: Digitate papules,
lepromatous leprosy, leprosy, multibacillary leprosy,
verrucouscarcinoma
Division of Dermatology, Department of Medicine andDivision of
Dermatology, Department of Medicine andDivision of Dermatology,
Department of Medicine andDivision of Dermatology, Department of
Medicine andDivision of Dermatology, Department of Medicine
andTherapeutics, Prince of WTherapeutics, Prince of WTherapeutics,
Prince of WTherapeutics, Prince of WTherapeutics, Prince of Wales
Hospital, Hong Kongales Hospital, Hong Kongales Hospital, Hong
Kongales Hospital, Hong Kongales Hospital, Hong Kong
MM Chang, MBChB, MRCP
Department of Anatomical and Cellular PDepartment of Anatomical
and Cellular PDepartment of Anatomical and Cellular PDepartment of
Anatomical and Cellular PDepartment of Anatomical and Cellular
Pathologyathologyathologyathologyathology,,,,,Prince of WPrince of
WPrince of WPrince of WPrince of Wales Hospital, Hong Kongales
Hospital, Hong Kongales Hospital, Hong Kongales Hospital, Hong
Kongales Hospital, Hong Kong
PCL Choi, MBChB, FRCPA
Correspondence to: Dr. MM Chang
Department of Medicine and Therapeutics, 9/F ClinicalSciences
Building, Prince of Wales Hospital, 30-32 NganShing Street, Shatin,
New Territories
IntroductionIntroductionIntroductionIntroductionIntroduction
The cutaneous manifestations of leprosy are vast.We report a
case of lepromatous leprosy with
classical clinical and histological features, but
alsoco-existing with verrucoid growth, which is
seldomencountered.
Case reportCase reportCase reportCase reportCase report
A 59-year-old man was referred to the OrthopaedicTumour Clinic
for a fungating growth on his leftheel for four months. It was
asymptomatic butgrowing rapidly. There was no history of traumaor
contact with fish. He had a history of closedfracture on the same
ankle that was treatedconservatively in childhood, and carcinoma
ofrectum, now in remission, following surgery andchemo-radiation
therapy 12 years ago.
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MM Chang and PCL Choi78
Clinically, the mass was 45 cm, with verrucoussurface and
irregular border (Figure 1). There wasno satellite lesion or
regional lymphadenopathy.The provisional diagnosis by the
orthopaedic teamwas squamous cell carcinoma. MRI excluded
deepfascial invasion. Wide local excision followed bystaged skin
graft was performed.
The patient was referred to the dermatology teamwhen the
histology suggested otherwise. Onfurther enquiry, he complained of
insidious onsetof dry skin, sensation loss of the extremities
causingfrequent scald injuries after handling hot waterand
progressive painless deformity of left anklefrom which he reported
difficulty findingappropriate shoes. Social history revealed that
he
was born and immigrated from a village inGuangdong 40 years ago,
but he did not recallany history of similar condition among his
closefamily members. On examination, he had slightsaddle-nose
deformity, Charcot's joint on the leftankle (Figure 2), acquired
ichthyosis on the limbs,multiple non-descript ill-defined
erythematouspapules on the back (Figures 3 & 4) and
enlargedulnar nerves on palpation. Peripheral neuropathywas
confirmed on nerve conduction study.
Histologically, there was verrucoid epidermalhyperplasia without
invasion, atypia or increasedmitotic activities (Figure 5). There
were patchymixed inflammatory lymphohistiocytic infiltratesand some
plasma cells. Globi formation wasnoted with foamy histiocytes
(Figures 6 & 7). Ziehl-Neelsen stain and Wade-Fite stains
showedabundant acid-fast bacilli (Figure 8), while PAS,Grocott,
Warthin-Starry and Gram stains were allnegative. Polymerase chain
reaction (PCR) analysisof paraffin tissue was positive for
Mycobacteriumleprae DNA but not M. tuberculosis. A second skin
Figure 1.Figure 1.Figure 1.Figure 1.Figure 1. Verrucous growth
on heel.
Figure 2.Figure 2.Figure 2.Figure 2.Figure 2. Charcot's
joint.Figure 3.Figure 3.Figure 3.Figure 3.Figure 3. A close-up of
digitate-like ill-definedpapules on back.
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Lepromatous leprosy in a Chinese male 79
Figure 4.Figure 4.Figure 4.Figure 4.Figure 4. Multiple
erythematous papules on back.
Figure 5.Figure 5.Figure 5.Figure 5.Figure 5. Low magnification
showed verruoidepidermal hyperplasia with underlying fibrous
stroma(H&E, Original magnification 20).
Figure 6.Figure 6.Figure 6.Figure 6.Figure 6. Biopsy from the
back showed intradermalcollections of lymphocytes and histiocytes.
Note thepresence of globi (H&E, Original magnification 20).
Figure 7.Figure 7.Figure 7.Figure 7.Figure 7. Medium
magnification showed the presenceof globi admixed with lymphocytes
and histiocytes(H&E, Original magnification 200).
biopsy on a papule on the back also showedidentical histology
(Figure 6).
The diagnosis of lepromatous leprosy was madeand the patient was
started on rifampicin, dapsoneand minocycline in hospital. There
was noobservable reaction on treatment and the graftwas taking
well. Screening for HIV and diabeteswas negative. On discharge, he
was referred tothe Leprosy Skin Clinic for continuation of
multi-drug therapy and contact tracing.
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MM Chang and PCL Choi80
DiscussionDiscussionDiscussionDiscussionDiscussion
Leprosy is a chronic granulomatous infection ofthe skin and
peripheral nerves. It is transmittedby Mycobacteria leprae (M.
leprae), an obligateintracellular Gram-positive bacillus with
acid-fastproperty and affinity for macrophages andSchwann cells. It
is a global disease affecting allraces, highly endemic in Africa
and SouthAmerica, and prevalent in developing countriesin
South-East Asia and parts of China. With theintroduction of WHO
multi-drug therapy (MDT),the disease is now controlled with
decreasingprevalence. In fact, Hong Kong is declared byWHO to be
eliminated from leprosy1 with itsincidence of 0.088/10000
population. In the1950s and 60s, the majority of leprosy patientsin
Hong Kong (90%) were born in China,2 whilein recent years, imported
leprosy from South EastAsian countries accounted for the
majority.
The infection caused by M. leprae carries a longincubation
period, from months to 20 years,averaging three to five years. The
majority ofexposed individuals do not develop disease.Host genetic
and environmental factors influenceindividual susceptibil i ty to
infection anddisease progression. In lepromatous leprosy, a
Figure 8.Figure 8.Figure 8.Figure 8.Figure 8. Wade-Fite stain
demonstrated manyintracellular acid fast bacilli (red in stain)
(WF, Originalmagnification 40).
predominant-Th2 response suppressesmacrophage activity and cell
mediated immunitywhile a heightened Th1 response is found
intuberculoid leprosy. Several leprosy susceptibilityloci on
chromosome 6 and 10 are found; andthe presence of HLA allele DQ1
gears towardslepromatous expression. In a genome-wideassociation
study in Han Chinese, novelassociation with NOD2, TNFSF15, RIPK2
arefound, indicating heterogeneity of geneticsusceptibility between
ethnic groups.3 Transmissionresults from early childhood close
contact with asource with high infectivity (lepromatous
leprosy),and spreading by droplets of nasal or oralsecretions. The
household risk of acquiring diseasein an endemic area is 10%. There
are a few reportsof infections acquired through open wound
andfomites, and, recently, armadillos as a zoonotictransmission.4
Moreover, another species,Mycobacterium lepromatosis, has been
found tocause diffuse form of lepromatous leprosy inMexico and the
Caribbean.5
Our patient had classical clinical and histologicfeatures of
lepromatous leprosy, whichrepresented the more severe spectrum of
diseasewith multi-bacillary involvement and poor hostimmunity. What
was atypical would be the lateage of onset, very long incubation
and verrucoidpresentation. Most literature reported specialforms of
leprosy as histoid, dermatofibroma-likepapules, digitate or
shagreen-like patches.Verrucoid presentations are rare in
leprosyand were found in few anecdotal reportsof lepromatous
leprosy wi th coexis tentchromoblastomycosis,6 or in
HIV-positiveindividuals.7 A more logical association would
besquamous cell carcinoma arising from chronicunhealed leg ulcers,
and even with that, verrucouscarcinoma arising from such lesion was
rarelyreported.8 The underlying reason that ourimmunocompetent
patient developed verrucoidgrowth was obscure. Without other
supportingcutaneous suggestions of leprosy, the
differentialdiagnosis would have been malignant tumours(squamous
cell carcinoma, verrucous carcinoma,
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Lepromatous leprosy in a Chinese male 81
cutaneous metastasis, amelanotic melanoma), orinfection (giant
condyloma, deep fungal infection,atypical mycobacterial infection
or tuberculosisverrucosa cutis). A biopsy for histology and
culturewould be helpful to establish the diagnosis.
The diagnosis of leprosy is suggested by (1) thepresence of skin
lesions with definite sensory loss(except borderline lepromatous or
lepromatousleprosy), (2) thickened peripheral nerves, or (3)the
presence of acid-fast bacilli on skin smears ortissue biopsy.9 The
sensitivity and positivepredictive values based on the above
diagnosticfeatures are more than 95% in endemic countries.10
M. leprae cannot be cultured in artificial media.Newer
techniques like serology for M. lepraeantibody, phenolic
glycolipid-1 (PGL-1), or PCRfor M. leprae DNA detection are useful
inmultibacillary disease with high bacterial load. Thesensitivity
for serology ranges from 40%(paucibacillary) to 90%
(multibacillary) and it canbe used to monitor the effectiveness of
treatmentas its level declines with MDT. The sensitivity ofPCR
using M. leprae specific repetitive sequencein multibacillary
disease is 80% and can beperformed on skin or nasal biopsy specimen
andextracts of slit skin smear.11 These tests are usedmore as
identification tools rather than detectiontools for leprosy, as the
sensitivity for paucibacillarydisease is low overall.
Standard multi-drug therapy with monthlysupervised rifampicin
600 mg and clofazimine300 mg, and daily self-administered
dapsone100 mg and clofazimine 100 mg was prescribedto our patient.
He tolerated treatment well withcounseling, psychological support
and regularfollow-up. Podiatry assessment and footwearadjustment
was made. The relapse rate after twoyears of WHO-MDT is quoted to
be 0.77%globally1 to 3% locally.12 In our locality, a
moreconservative approach is adopted with prolongedMDT until all
skin lesions are inactive, followedby dapsone monotherapy for 10
years after theslit skin smear is negative.
In conclusion, although leprosy is a fading disease,clinicians
should remain vigilant in the screeningand diagnosis of leprosy in
Hong Kong. Earlyspecialist referral is recommended in view ofits
protean manifestations and infectiousimplications.
AcknowledgementsAcknowledgementsAcknowledgementsAcknowledgementsAcknowledgements
The author would like to thank Dr Lo Kuen Kongand Dr Luk Nai
Ming, for their supervision, andDr Tse Lung Fung, for supplying the
surgicalphoto.
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