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From the Faculty of Medicine (Boggild, Correia, Keystone, Kain) and the Global Health Program, McLaughlin Center for Molecular Medicine (Kain), University of Toronto, and the Tropical Disease Unit, University Health Network Toronto General Hospital (Keystone, Kain), Toronto, Ont.
| Abstract |
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Methods: We reviewed the clinical records of all 184 leprosy patients who were referred to the Tropical Disease Unit at Toronto General Hospital between 1979 and 2002 and abstracted demographic and clinical information.
Results: Patients were more likely to be male (122 or 66.3%) and of Indian (44 or 23.9%), Filipino (49 or 26.6%) or Vietnamese (37 or 20.1%) origin. Patients experienced symptoms for a mean of 4.8 years before referral to the Tropical Disease Unit. Most had no family history of leprosy (152/172 or 88.4%). Most patients presented with either borderline tuberculoid (80 or 43.5%) or borderline lepromatous (37 or 20.1%) disease. On average, patients presented with 5.8 skin lesions. Upper- and lower-extremity nerve dysfunction was common at presentation, with up to one-third of patients demonstrating either sensory or motor loss. A significantly greater lag time to presentation was observed in patients who emigrated from low-prevalence regions (p < 0.001).
Interpretation: Leprosy is a chronic infectious disease that is associated with serious morbidity if left untreated. Leprosy is uncommon in developed countries, but it is important for physicians to have a high index of suspicion when a foreign-born patient presents with chronic dermatitis and peripheral nerve involvement.
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We report here on a number of imported cases of leprosy in Canada. The objective was to review the demographic characteristics, presentation and outcome of patients presenting in an urban centre. This report underscores the importance of accurate diagnosis and appropriate management of patients with leprosy.
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2 analysis for comparing proportions of categorical variables. For multiple comparisons of proportions of categorical variables, the
2 distribution via contingency tables was employed. Means for continuous variables were compared with Student's t test. For subsamples with unequal variance the KruskalWallis one-way analysis of variance was applied. This study was approved by the Research Ethics Board of the Toronto General Hospital.
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Patients experienced symptoms for a mean of 4.8 years before presentation to the Tropical Disease Unit (Table 2). Greater lag time to presentation was predicted by emigration from a low-prevalence region rather than a region where leprosy is endemic (p < 0.001). Patients from low-prevalence countries (fewer than 0.5 cases per 10 000 population) such as Sri Lanka, Guyana and Trinidad had a mean 4.7-year lag time to diagnosis, whereas those from higher-prevalence countries such as India, Vietnam and the Philippines had a mean lag time of 1.5 years. Patients with lepromatous disease (lepromatous leprosy [LL] or borderline lepromatous leprosy [BL]) were older at presentation than those with tuberculoid disease (borderline tuberculoid leprosy [BL] or tuberculoid leprosy [TT]) (p = 0.048). Mean age at presentation was 32.8 years for TT patients and 44.4 years for LL patients (Table 2). At presentation, patients were most likely to be classified as having BT (80 patients or 43.5%), BL (37 or 20.1%) or LL (29 or 15.8%) disease. Of note, patients originally from the Philippines were more likely to have lepromatous leprosy (LL or BL) at presentation (23/49 patients or 47%) than those from Vietnam (7/37 or 19%) or India (11/44 or 25%) (
2 contingency table = 8.49, p < 0.025).
One-third of patients exhibited upper- or lower-extremity sensory nerve dysfunction, and one-quarter had motor dysfunction on presentation (Table 1). Of those with upper-extremity nerve dysfunction, generalized sensory loss was most common (31/180 or 17.2% of the main sample), whereas motor involvement was most likely in the ulnar distribution (15/180 or 8.3% of the main sample), followed by the combination of median and ulnar distribution (12/180 or 6.7% of the main sample). Similarly, lower-extremity sensory loss, present in 33.1% of patients, was most likely to be generalized (42/181 or 23.2% of the main sample), whereas motor dysfunction, present in 22.3% of patients, was most likely to be in the distribution of the common peroneal nerve (29/179 or 16.2% of the main sample). Table 3 highlights that lag time to presentation was greater among those with nerve dysfunction at the lepromatous pole.
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On the basis of World Health Organization recommendations,1,4 most patients with paucibacillary leprosy (indeterminate leprosy, TT or BT) were treated with dapsone plus rifampin (67/103 or 65.0%); for a minority (12/103 or 11.7%) clofazimine was added. The majority of patients with multibacillary disease (borderline leprosy [BB], BL or LL) were treated with dapsone, rifampin and clofazimine triple therapy (42/73 or 57.5%). The median duration of treatment for paucibacillary and multibacillary patients was 6 and 24 months respectively.
At presentation, 48 patients (25.5%) were undergoing a leprosy reaction, with most suffering a type 1 reversal reaction (Table 1). During treatment, 42 patients experienced a reaction, and following treatment cessation an additional 19 patients experienced a reaction; in both situations, the majority were type 1 or reversal reactions (Table 1). Mean duration of follow-up for the sample was 47.9 (median 44, range 1 to 132) months. Of patients completing therapy at the Tropical Disease Unit, true relapse was documented clinically and histologically in only 2 individuals, but 17 patients were lost to follow-up before the recommended 5 years.
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The positive correlation in this study between age and shift toward the lepromatous end of the disease spectrum is interesting. A possible explanation is that debilitating nerve involvement occurs later in lepromatous disease than in tuberculoid illness, and patients may thus delay seeking care because they perceive their symptoms as benign. This possibility is supported by the shorter lag time to presentation among patients with paucibacillary (tuberculoid) disease than among those with multibacillary (lepromatous) disease (in particular, the median lag time to diagnosis was 8 years among those with LL disease but just 3 years among those with TT disease). In addition, those born outside high-prevalence areas had a greater lag time from onset of symptoms to presentation and might therefore have had more encounters or more opportunities for encounters with the health care system before accurate diagnosis. This greater lag time suggests that a lack of disease awareness and recognition may contribute to delay in diagnosis and treatment. However, additional prospective studies are needed to establish whether this is in fact the case.
At the time of presentation, sensory loss was apparent in one-third of patients and motor dysfunction in one- quarter. As Table 3 illustrates, those at the lepromatous end of the spectrum tended to have symptoms, including motor and sensory neuropathy, for a longer period before diagnosis. Clearly, this type of nerve involvement is an important cause of disability and lost productivity. However, these deficits are often ameliorable with multidrug therapy and corticosteroids. That a minority of patients in this study were managed differently from the current treatment guidelines emphasizes the changes in recommendations over time, the complexity of the disease and the occasional need for individualized, case-based therapy.
Leprosy reactions are common among patients undergoing multidrug therapy, but care is needed in distinguishing reactions from relapse, because their management is different.2
In summary, the diagnosis of leprosy should be considered in cases of chronic dermatitis with peripheral nerve involvement in foreign-born individuals, as well as those who have undertaken protracted travel abroad. Leprosy can be a complicated and challenging disease to manage. If suspected, referral to a tropical disease expert or a dermatologist with expertise in leprosy is warranted.
ß See related article page 71
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Contributors: Jason D. Correia and Jay S. Keystone contributed to the study design, data acquisition and analysis, and were involved in revising the manuscript. Andrea K. Boggild and Kevin C. Kain contributed to the study design, data acquisition, analysis and interpretation, and were primarily responsible for writing the manuscript.
Acknowledgement: We thank Stephen Retchford for his invaluable contribution to database design and implementation throughout this project.
Competing interests: None declared.
Correspondence to: Dr. Kevin C. Kain, Toronto General Hospital, 200 Elizabeth St., ES-9-412, Toronto ON M5G 2C4; fax 416 595-5826; Kevin.Kain{at}uhn.on.ca
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