Lepr Rev (2014) 85, 170 176 Leprosy An imported disease ZHENLI KWAN*, JAYALAKSHMI PAILOOR**, LENG LENG TAN*, SUGANTHY ROBINSON*, SU-MING WONG* & ROKIAH ISMAIL* *Dermatology Unit, Department of Medicine, University Malaya Medical Centre, Kuala Lumpur, Malaysia **Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia Accepted for publication 22 June 2014 Summary Objectives: Leprosy remains a public health concern in Malaysia and globally. We aim to review the characteristics of leprosy patients in a tertiary institution in urban Malaysia. Design: This is a case series of 27 leprosy patients who presented between 2008 and 2013. Results: The majority of our patients consisted of male (74 1%), Malaysian (63 0%), blue collar workers (51 9%) and married (59 3%) patients; 48 1% had lepromatous leprosy. All except one of the patients presented with skin lesions, 25 9% had nerve involvement and 33 3% developed lepra reactions. Forty-four point four percent (44 4%) of the cases seen initially in the primary care setup were misdiagnosed. Conclusions: Doctors need to have a high index of suspicion for leprosy when patients present with suggestive skin, nerve or musculoskeletal lesions. Immigrants accounted for 37% of cases and these patients may become a reservoir of infection, thus accounting for the rise in incidence. An increasing trend in multibacillary cases may be attributed to the spread from migrants from countries with a high burden of leprosy. Introduction Leprosy is a chronic granulomatous bacterial infection caused by Mycobacterium leprae transmitted via droplets, which mainly involves the skin and the peripheral nerves. Table 1 shows the prevalence of leprosy, the number of new cases and number of new multibacillary cases in Malaysia and neighbouring countries 1 while Figure 1 shows the incidence and prevalence of leprosy in Malaysia from 1984 till 2012. 2 18 Correspondence to: Zhenli Kwan, University Malaya Medical Centre, Lembah Pantai 59100, Kuala Lumpur, Malaysia (Tel: þ60379494422; Fax: þ60379562253; e-mail: kzhen@dr.com) 170 0305-7518/14/064053+07 $1.00 q Lepra
Table 1. The leprosy situation in Malaysia and neighbouring countries 1 Leprosy An imported disease 171 Country Registered prevalence at the end of first quarter of 2012 Number of new cases detected (2011) Number of new cases of multibacillary leprosy Bangladesh 3300 3970 1798 India 83187 127295 63562 Indonesia 23169 20023 16099 Myanmar 2735 3082 2165 Malaysia 219 216 173 Nepal 2410 3184 1683 Sri Lanka 1565 2178 1045 Thailand 678 280 193 Vietnam 644 748 269 Prevalence of leprosy in Malaysia showed an initial downward trend with the advent of multidrug therapy, with elimination achieved in 1994; 5 however the incidence in the recent few years has shown a resurgence although prevalence remains static. There has also been an increasing trend in the proportion of multibacillary cases in Malaysia as shown 1,19 22 in Figure 2. 6 5 6 5 3 5 4 4 3 9 Incidence (per 100000) Prevalence (per 10000) 3 38 3 2 9 2 4 2 1 0 2 1 2 1 2 1 1 9 1 9 1 7 1984 1985 1986 1987 1988 1 9 1 7 1 8 1 3 1 8 1 4 1 6 1989 1990 1991 1992 1993 1994 1995 1 5 1 3 1 3 1 1 0 9 1 0 9 0 87 0 97 1 1 11 0 89 0 8 0 84 0 76 0 810 73 0 75 0 6 0 66 0 68 0 5 0 5 0 5 0 4 0 4 0 4 0 4 0 3 0 3 0 3 0 3 0 3 0 2 0 2 0 2 1996 1997 1998 1999 2000 2001 2002 Figure 1. Prevalence and incidence of leprosy in Malaysia, 1984 to 2012. 2 18 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012
172 Z. Kwan et al. 100 Proportion of multibacillary and paucibacillary leprosy cases (%) 90 80 70 74 73 69 6 67 9 73 2 62 3 76 9 72 1 67 3 74 3 73 8 87 6 80 1 60 50 55 52 Proportion of multibacillary cases Proportion of paucibacillary cases 40 45 48 30 20 10 0 1992 1993 26 27 30 4 32 1 26 8 1994 1995 1996 1997 1998 1999 37 7 23 1 2000 2001 2002 2003 2004 32 7 27 9 25 7 26 2 2005 2006 2007 2008 2009 2010 19 9 12 4 Figure 2. Proportion of multibacillary and paucibacillary cases among new leprosy cases in Malaysia, 1992 2011. 1,5,19,20 We aim to study the demographics and clinical presentation of leprosy patients between 2008 and 2013 in a tertiary centre which provides medical care to patients in Kuala Lumpur, the surrounding Klang Valley and other states in Malaysia. Malaysia is a South-East Asian country with a multi-ethnic population in which the main ethnic groups are the Malays and other native groups, the Chinese and the Indians. 2011 Materials and methods The list of leprosy patients seen between 2008 and 2013 was obtained using the Dermatology Clinic census and by comparison with the list of patients who had undergone a slit-skin smear (SSS) test and the list according to the International Classification of Diseases (ICD) diagnosis from Medical Records. Histopathological findings were reviewed with the pathologist involved in this study. The case notes were retrieved and reviewed to obtain the clinical findings and information regarding treatment. The results were analysed using IBM SPSS Statistics version 21 0. Ethical approval and funding were not required. Results Twenty-seven adult leprosy cases seen between 2008 and 2013 were reviewed as shown in Table 2. The median age for Malaysian patients was 49 years while the median age for non- Malaysian patients was 27 5 years. Confirmation of diagnosis was done either using a positive
Leprosy An imported disease 173 Table 2. Demographics and clinical presentations of leprosy patients Features Number of patients (%) Gender Male 20 (74.1) Female 7 (25 9) Ethnicity Malay 4 (14 8) Chinese 8 (29 6) Indian 7 (25 9) Others 8 (29 6) Nationality Malaysian 17 (63 0) Non-Malaysian 10 (37 0) Occupation Blue collar 14 (51 9) White collar 1 (3 7) Retired or unemployed 5 (18 5) Unknown 7 (25 9) Marital status Single 4 (14 8) Married 16 (59 3) Unknown 6 (22 2) Widowed 1 (3 7) Classification Tuberculoid leprosy 6 (22 2) Borderline tuberculoid leprosy 3 (11 1) Borderline leprosy 1 (3 7) Borderline lepromatous leprosy 1 (3 7) Lepromatous leprosy 13 (48 1) Multibacillary leprosy (unspecified Ridley-Jopling classification) 3 (11 1) Skin lesions 26 (96 3) Face 20 (74 1) Trunk 15 (55 6) Upper limbs 15 (55 6) Lower limbs 15 (55 6) Hypoaesthesia/anaesthesia 11 (40 7) Nerve involvement 7 (25 9) Greater auricular 3 (11 1) Ulnar 6 (22 2) Peroneal 2 (7 4) Effects Motor 1 (3 7) Sensory 2 (7 4) Sensory and motor 2 (7 4) Complications Nerve 7 (25 9) Musculoskeletal 7 (25 9) Eye 2 (7 4) Others 3 (11 1) Lepra reactions 9 (33 3) Type 1 3 (11 1) Type 2 4 (14 8) Type 1 and 2 2 (7 4) Lucio s phenomenon 0 (0 0)
174 Z. Kwan et al. slit-skin smear result [18 cases (66 7%); of which four cases were diagnosed based on SSS alone (14 8%)] or on histopathological examination. Of note, 22 cases (81 5%) were diagnosed based on skin biopsies while another one (3 7%) was diagnosed based on nerve biopsy. The mean initial Bacillary Index (BI) was 2 6 and the mean initial Morphological Index (MI) was 1 0. For non-malaysian patients, two (7 4%) were of ethnic Indian descent. Of the remaining eight patients, three (11 1%) were from Indonesia, two (7 4%) from Nepal, two (7 4%) from Myanmar and one (3 7%) from Sri Lanka. One Myanmarese gentleman had skin lesions since his arrival in Malaysia three years ago but his condition was worsening for the past year prior to presentation. Another Myanmarese gentleman had previously defaulted treatment for leprosy in his home country and presented with new lesions of a year s duration. For the others, one Indian lady had been in Malaysia for 9 months before presenting with leprosy while two Indonesian gentlemen had been in Malaysia for 1 year and approximately 2 years respectively before developing skin lesions. The timeframe between duration of lesions and arrival in Malaysia was unknown for four patients (14 8%). Only one Indian patient (3 7%) had been a long-time resident in the country, having emigrated from India as a child. There was a missed diagnosis of leprosy in 12 patients (44 4%) of patients. The initial diagnoses given by primary care physicians included skin infection, fungal infection (n ¼ 2), tuberculosis, sarcoidosis or connective tissue disease, xanthoma, nerve tumors, viral warts, allergic reaction (with a differential diagnosis of erythema marginatum) and keloids. Only one patient (3 7%) had a positive contact history. Five (18 5%) had a history of immunodeficiency. Of these five cases, one was a renal transplant patient on immunosuppressants, another patient had idiopathic primary immunodeficiency with low CD4 counts, and the other three were diabetic. Most of the patients (n ¼ 12, 44 4%) were followed-up in our centre. Four (14 8%) were foreigners, and subsequently returned to their home countries for further follow-up while three (11 1%) of the patients died due to other causes. One patient (3 7%) transferred care to a private centre and another three (11 3%) chose to continue follow-up at Ministry of Health hospitals due to logistic reasons. The fates of four patients (14 8%) were unknown. Discussion Foreigners account for 37% of the leprosy cases in our institution, which is consistent with the national average of 40% of new cases. 5 In the late 1980 s, when Malaysia was undergoing a transition from an agro-based country to a rapidly industrialised nation, there was an influx of migrant workers from neighbouring countries with a heavier burden of leprosy especially in terms of multibacillary cases since the mid-1990 s. 1 3 The top six countries of origin for migrants in Malaysia in 2006 were Indonesia (65 7%), Nepal (10 8%), India (7 6%), Myanmar (5 0%), Vietnam (4 6%) and Bangladesh (3 2%). 24 In our review, the countries of origin of migrants included Indonesia, Sri Lanka, Myanmar and Nepal. This influx led to the spread of leprosy and contributed to the rising trend in the proportion of multibacillary cases among Malaysians and the migrant community due to crowded living conditions of migrant workers especially in urban areas; such as in longhouses or hostels at construction sites. The percentage of foreigners among new cases detected in Malaysia showed a significant upward trend from 28 3% in 1992 to 46 4% in 2010. 4,25
Leprosy An imported disease 175 The average age of non-malaysian patients in our review is lower than Malaysian patients. We postulate that this is due to the majority of migrants entering the country when they are young enough to migrate and perform manual labour. However this may lead to spread among the working age population in Malaysia and among families, especially when intermarriage occurs. There is also a male preponderance, which has been postulated to be due to the reluctance of females to present to health facilities with skin lesions. Another possible reason could be a higher number of male compared to female migrants. In 2004, 51% of Indonesian migrant workers in Malaysia were male and the majority of migrants in Malaysia are from Indonesia. 24,26 Our finding of 44 4% initially missed diagnoses indicates a public health problem, especially since patients in a tertiary referral centre like ours are referred here by primary care providers. Health professionals need education regarding leprosy, as the diagnosis may be overlooked; especially when many feel that leprosy has to present with hypoesthesia. A high index of suspicion needs to be maintained when patients present to health facilities. Delay in commencing treatment can lead to severe deformities and disabilities. Notification of cases and contact screening also plays an important role in the early diagnosis of leprosy. Conclusions Leprosy remains a major public health concern in this part of the world, especially in view of the roles of immigration and globalization; and the increase in the proportion of multibacillary cases. Most patients present with lepromatous leprosy. It is important to be vigilant and have a high index of suspicion for leprosy to reduce morbidity secondary to the disease and its complications. Contributorship Dr. Zhenli Kwan (guarantor) Planning, data collection and analysis, writing and reporting the work. Professor Dr. Jayalakshmi Pailoor Histopathological analysis and input. Dr. Leng Leng Tan Data collection and analysis. Dr. Suganthy Robinson Data collection and analysis. Dr. Su-Ming Wong Writing and reporting the work, editing. Professor Dr. Rokiah Ismail Planning, writing and reporting the work, editing. References 1 World Health Organization. Global leprosy situation, 2012. Weekly Epidemiol Rec. 2012; 87: 317 328. 2 Ministry of Health Malaysia. Annual report Ministry of Health Malaysia 2003. Ministry of Health Malaysia, Kuala Lumpur, 2003; p. 106. 3 Ministry of Health Malaysia. Annual report Ministry of Health Malaysia 2009. Ministry of Health Malaysia, Putrajaya, 2009; p. 61. 4 Ministry of Health Malaysia. Annual report Ministry of Health Malaysia 2010. Ministry of Health Malaysia, Putrajaya, 2010; pp. 53 54. 5 Ministry of Health Malaysia. Annual report Ministry of Health Malaysia 2011. Ministry of Health Malaysia, Putrajaya, 2011; pp. 62 63.
176 Z. Kwan et al. 6 Ministry of Health Malaysia. Health facts 2000. Ministry of Health Malaysia, Kuala Lumpur, 2000; p. 5. 7 Ministry of Health Malaysia. Health facts 2001. Ministry of Health Malaysia, Kuala Lumpur, 2003; p. 9. 8 Ministry of Health Malaysia. Health facts 2002. Ministry of Health Malaysia, Kuala Lumpur, 2004; p. 9. 9 Ministry of Health Malaysia. Health facts 2003. Ministry of Health Malaysia, Kuala Lumpur, 2004; p. 9. 10 Ministry of Health Malaysia. Health facts 2004. Ministry of Health Malaysia, Putrajaya, 2005; p. 10. 11 Ministry of Health Malaysia. Health facts 2005. Ministry of Health Malaysia, Putrajaya, 2006; p. 7. 12 Ministry of Health Malaysia. Health facts 2006. Ministry of Health Malaysia, Putrajaya, 2007. 13 Ministry of Health Malaysia. Health facts 2007. Ministry of Health Malaysia, Putrajaya, 2008. 14 Ministry of Health Malaysia. Health facts 2008. Ministry of Health Malaysia, Putrajaya, 2009. 15 Ministry of Health Malaysia. Health facts 2009. Ministry of Health Malaysia, Putrajaya, 2010. 16 Ministry of Health Malaysia. Health facts 2010. Ministry of Health Malaysia, Putrajaya, 2011; p. 6. 17 Ministry of Health Malaysia. Health facts 2012. Ministry of Health Malaysia, Putrajaya, 2012; p. 6. 18 Ministry of Health Malaysia. Health facts 2013. Ministry of Health Malaysia, Putrajaya, 2013; p. 19. 19 Leprosy Elimination Unit World Health Organization Western Pacific Region. Overview and epidemiological review of leprosy in the WHO Western Pacific Region 1991 2001. World Health Organization Regional Office for the Western Pacific, Manila, 2003; p. 42. 20 World Health Organization. Western Pacific region: leprosy situation at the end of 2006. Regional Office for the Western Pacific, Manila, 2006. URL http://www.who.int/lep/situation/wprostatsend2006.pdf [Accessed July 29, 2013]. 21 World Health Organization. Western Pacific region: leprosy situation at the end of 2005. Regional Office for the Western Pacific, Manila. 2006. URL http://www.who.int/lep/situation/wprostatsmay06.pdf [Accessed July 29, 2013]. 22 Sagabiel D, Cunanan AC Jr, van Weezenbeek C et al. Epidemiological review of leprosy in the Western Pacific Region 2008 2010. World Health Organization Western Pacific Region, Manila, 2011; pp. 14 16. 23 Department of Statistics Malaysia. Table 5: Percentage distribution of labour force by ethnic group Malaysia, 1982 2011. URL http://www.statistics.gov.my/portal/download_economics/files/data_series/survey10/ PDF/TABLE5.pdf [Accessed July 29, 2013]. 24 Nair P, Jantan N. Malaysia. International migration in Malaysia. Accepted for presentation at the Expert Group Meeting on ESCAP Regional Census Programme, November 2006, Bangkok, Thailand. 25 Ministry of Health Malaysia. Annual report Ministry of Health Malaysia 1992. Ministry of Health Malaysia, Kuala Lumpur, 1992; pp. 65 66. 26 Female Migrant Workers Research Team. Fact sheet: migration, remittance and female migrant workers. World Bank, Washington, 2006. URL http://siteresources.worldbank.org/intindonesia/resources/fact_ sheet-migrant_workers_en_jan06.pdf [Accessed August 4, 2013].