Leprosy - an overview of clinical features, diagnosis, and treatment.

Leprosy is a chronic infectious disease caused by Mycobacterium (M.) leprae. Worldwide, 210,758 new cases were diagnosed in 2015. The highest incidence is found in India, Brazil, and Indonesia. While the exact route of transmission remains unknown, nasal droplet infection is thought to be most likely. The pathogen primarily affects the skin and peripheral nervous system. The disease course is determined by individual host immunity. Clinically, multibacillary lepromatous variants are distinguished from paucibacillary tuberculoid forms. Apart from the various characteristic skin lesions, the condition is marked by damage to the peripheral nervous system. Advanced disease is characterized by disfiguring mutilations. Current treatment options are based on WHO recommendations. Early treatment frequently results in complete remission without sequelae. While paucibacillary forms are treated with rifampicin and dapsone for at least six months, multibacillary leprosy is treated for at least twelve months, additionally requiring clofazimine. Leprosy reactions during therapy may considerably aggravate the disease course. Besides individual treatment, WHO-supported preventive measures and strategies play a key role in endemic areas.

Leprosy in Puerto Rico: insight into the new millennia.

BACKGROUND: Leprosy, or Hansen's disease, is a chronic infectious disease caused by the bacillus Mycobacterium leprae. In 2000, the World Health Organization (WHO) defined the elimination of the disease as a global prevalence of less than one case per 10,000 population. However, disease transmission is an ongoing worldwide public health concern, as evidenced by the more than 220,000 new cases diagnosed each year. METHODS: This study is an update of the incidence and prevalence of leprosy in Puerto Rico for the period of 2000-2014. A retrospective analysis of data was obtained from the Tropical Disease Clinic (TDC) of the University of Puerto Rico School of Medicine. RESULTS: Sixty-three new cases of leprosy are detailed in this study. Disease incidence and prevalence were 1.65 and 5.26 per 100,000 inhabitants (of the island of Puerto Rico), respectively, and an average of 4.2 new cases per year. Most of the male patients in the study suffered from lepromatous leprosy (P = 0.026). In all, 47 (74.6%) patients had been born in Puerto Rico, and 29 (46%) had an affected family member or were in close contact with someone with leprosy. CONCLUSIONS: Compared to those of previous studies, these results demonstrate a decrease in both the incidence and prevalence of leprosy in Puerto Rico over the past 15 years. The relatively high prevalence of leprosy in Puerto Rico means that it remains endemic on the island. Concerted efforts must be undertaken to achieve the goal of the elimination of this old and stigmatized disease.

Autochthonous borderline tuberculoid leprosy in a man from Florida.

Leprosy (Hansen's disease) is a chronic contagious granulomatous disease principally affecting the skin and peripheral nervous system, caused by Mycobacterium leprae. In this report, we present a case of autochthonous leprosy in a man from Florida as the first human case reported from this region. Authors believe dermatologists need to be aware of the possibility of autochthonous transmission of leprosy in the Eastern-Southern United States, and should consider leprosy in any patient with atypical skin lesions, even when a history of contact with armadillo is missing.

Estudio epidemiológico en los enfermos de Lepra residentes en el sanatorio de Fontilles (Alicante, España): La última leprosería de Europa / No disponible

La lepra es una enfermedad infecciosa crónica causada por M. leprae con un importante componente genético y socioambiental. Estudiamos factores ambientales, geográficos, hereditarios y de contagio que puedan favorecer el desarrollo de la enfermedad y valoramos su evolución en una población muy concreta que vivió una larga etapa desprovista de tratamiento efectivo. 30 españoles adultos, que fueron afectados por la lepra y que residen hoy en el Sanatorio de Fontilles, contestaron de forma voluntaria y anónima a un cuestionario epidemiológico que dio lugar a 70 variables. Se analizó la significación estadística de las variables. El 70% de los pacientes provienen de Andalucía y el 85% de un ambiente pobre. El 80% fueron afectados de lepra lepromatosa. 56,7% de los pacientes refieren un contacto cercano con un enfermo de lepra, en su mayoría de tipo intradomiciliario, con un tiempo medio de convivencia de 16 años. Ningún paciente tuvo un hijo afecto. 20 de 27 pacientes valorados físicamente presentan algún grado de discapacidad de manos y pies. El 74,1% no puede garantizar haber bebido siempre agua limpia. Es necesaria una mayor investigación de los factores etiopatogénicos implicados en la enfermedad para controlar así su transmisión y conseguir su erradicación a nivel mundial

Leprosy is an infectious disease caused by M. Leprae, which has an important influence of genetic and socialenvironmental factors. We study environmental, geographic, hereditary and transmission factors which could contribute to the development of the illness and we evaluate its course and consequences in a very specific population sample that suffered from leprosy without treatment. 30 Spanish adults affected by leprosy who live today in the Sanatorio Fontilles, answered voluntarily and anonymously an epidemiological survey which provided us with 70 variables. We analysed the statistical significance of the variables. 70% of the patients were born in Andalusia and 85% of them come from a poor socioeconomic background. 80% where affected by lepromatous leprosy. 56,7% of the patients declared a close contact with a leprosy patient. In general, the contact took place in the household, with an average of 16 years living in the same household. No patient had an affected child. 20 out of the 27 physically evaluated patients present certain degree of disability. 74,1% of the patients cannot guarantee having had always access to a clean source of water. Further investigation of the etiology factors involved in leprosy is necessary to achieve the control of transmission and world eradication

Examining ERBB2 as a candidate gene for susceptibility to leprosy (Hansen’s disease) in Brazil

Leprosy remains prevalent in Brazil. ErbB2 is a receptor for leprosy bacilli entering Schwann cells, which mediates Mycobacterium leprae-induced demyelination and the ERBB2 gene lies within a leprosy susceptibility locus on chromosome 17q11-q21. To determine whether polymorphisms at the ERBB2 locus contribute to this linkage peak, three haplotype tagging single nucleotide polymorphisms (tag-SNPs) (rs2517956, rs2952156, rs1058808) were genotyped in 72 families (208 cases; 372 individuals) from the state of Pará (PA). All three tag-SNPs were associated with leprosy per se [best SNP rs2517959 odds ratio (OR) = 2.22; 95% confidence interval (CI) 1.37-3.59; p = 0.001]. Lepromatous (LL) (OR = 3.25; 95% CI 1.37-7.70; p = 0.007) and tuberculoid (TT) (OR = 1.79; 95% CI 1.04-3.05; p = 0.034) leprosy both contributed to the association, which is consistent with the previous linkage to chromosome 17q11-q21 in the population from PA and supports the functional role of ErbB2 in disease pathogenesis. To attempt to replicate these findings, six SNPs (rs2517955, rs2517956, rs1810132, rs2952156, rs1801200, rs1058808) were genotyped in a population-based sample of 570 leprosy cases and 370 controls from the state of Rio Grande do Norte (RN) and the results were analysed using logistic regression analysis. However, none of the associations were replicated in the RN sample, whether analysed for leprosy per se, LL leprosy, TT leprosy, erythema nodosum leprosum or reversal reaction conditions. The role of polymorphisms at ERBB2 in controlling susceptibility to leprosy in Brazil therefore remains unclear.

Examining ERBB2 as a candidate gene for susceptibility to leprosy (Hansen's disease) in Brazil.

Leprosy remains prevalent in Brazil. ErbB2 is a receptor for leprosy bacilli entering Schwann cells, which mediates Mycobacterium leprae-induced demyelination and the ERBB2 gene lies within a leprosy susceptibility locus on chromosome 17q11-q21. To determine whether polymorphisms at the ERBB2 locus contribute to this linkage peak, three haplotype tagging single nucleotide polymorphisms (tag-SNPs) (rs2517956, rs2952156, rs1058808) were genotyped in 72 families (208 cases; 372 individuals) from the state of Pará (PA). All three tag-SNPs were associated with leprosy per se [best SNP rs2517959 odds ratio (OR) = 2.22; 95% confidence interval (CI) 1.37-3.59; p = 0.001]. Lepromatous (LL) (OR = 3.25; 95% CI 1.37-7.70; p = 0.007) and tuberculoid (TT) (OR = 1.79; 95% CI 1.04-3.05; p = 0.034) leprosy both contributed to the association, which is consistent with the previous linkage to chromosome 17q11-q21 in the population from PA and supports the functional role of ErbB2 in disease pathogenesis. To attempt to replicate these findings, six SNPs (rs2517955, rs2517956, rs1810132, rs2952156, rs1801200, rs1058808) were genotyped in a population-based sample of 570 leprosy cases and 370 controls from the state of Rio Grande do Norte (RN) and the results were analysed using logistic regression analysis. However, none of the associations were replicated in the RN sample, whether analysed for leprosy per se, LL leprosy, TT leprosy, erythema nodosum leprosum or reversal reaction conditions. The role of polymorphisms at ERBB2 in controlling susceptibility to leprosy in Brazil therefore remains unclear.

Report of three new leprosy cases in children under fifteen in the municipality of Itaguai, Rio de Janeiro: event alert for epidemiological investigation.

Leprosy is an infectious disease with an average incubation period of two to five years. It is caused by Mycobacterium leprae, mainly affecting skin, mucous membranes and peripheral nerves. When it occurs in children under fifteen, it reflects an intense and long contact period, with a high bacillary load. Therefore, it is considered an important alert sign that points to the challenge of controlling the disease. The authors report three cases of leprosy in children under fifteen that occurred in the Itaguaí district, Rio de Janeiro. The epidemiologic implications of new cases detected at this age and the fundamental role of examining the patients' close contacts and the possible identification of source cases as an effort for leprosy control are discussed.

Relato de três casos novos de hanseníase em menores de quinze anos no município de Itaguaí, Rio de Janeiro: evento de alerta para investigação epidemiológica / Report of three new leprosy cases in children under fifteen in the municipality of Itaguai, Rio de Janeiro: event alert for epidemiological investigation

A hanseníase é uma doença infectocontagiosa, com período de incubação médio de dois a cinco anos, causada pelo Mycobacterium leprae, o qual possui tropismo para a pele, as mucosas e os nervos periféricos. Quando manifestada em crianças abaixo de quinze anos, reflete a intensidade e longo período de exposição à grande carga bacilar. Representa, então, um importante evento de alerta que aponta para uma dificuldade no controle da doença. Os autores relatam três casos de hanseníase, em menores de quinze anos, provenientes do Município de Itaguaí, Rio de Janeiro. Discutem-se as implicações epidemiológicas da detecção de novos casos nessa faixa etária e o papel fundamental do exame de contatos e da busca do caso fonte no controle da Hanseníase.

Leprosy is an infectious disease with an average incubation period of two to five years. It is caused by Mycobacterium leprae, mainly affecting skin, mucous membranes and peripheral nerves. When it occurs in children under fifteen, it reflects an intense and long contact period, with a high bacillary load. Therefore, it is considered an important alert sign that points to the challenge of controlling the disease. The authors report three cases of leprosy in children under fifteen that occurred in the Itaguaí district, Rio de Janeiro. The epidemiologic implications of new cases detected at this age and the fundamental role of examining the patients' close contacts and the possible identification of source cases as an effort for leprosy control are discussed.

In vitro and skin lesion cytokine profile in Brazilian patients with borderline tuberculoid and borderline lepromatous leprosy.

OBJECTIVE: We investigated the in vitro and skin lesions production of cytokines in non-treated borderline tuberculoid (BT) and borderline lepromatous (BL) patients. PATIENTS AND METHODS: Seven untreated, non-reactional BT patients and 12 untreated, non-reactional BL patients were studied. Levels of the cytokines IFN-gamma, IL-10, TGF-beta1 and TNF-alpha were measured in supernantant of peripheral blood mononuclear cells (PBMC) cultures, stimulated with specific M. leprae antigen (sonicated and whole). The cytokines iNOS, IL-10 and TGF-beta1 were detected by immunohistochemistry in skin biopsies. RESULTS: BT patients produced higher levels of IFN-gamma than BL patients; iNOS expression in skin lesions was also higher in BT patients. TGF-beta1 was detected in more cells in BL patients; IL-10 expression was similar in both groups. There was a negative correlation between iNOS and TGF-beta1 expression in skin biopsies, positive correlation between TGF-beta1 in skin lesions and bacillary index, as well as positive correlation between iNOS detected in skin biopsies and PBMC IFN-gamma production. CONCLUSIONS: The BT patients had a mainly a Th1-profile of cytokines in their skin lesions and BL patients had a Th2 profile.

Reactividad serológica y celular frente a proteínas micobacterianas en la enfermedad de Hansen / No disponible

Se diseñó un estudio para evaluar la reactividad inmunológica frente a diferentes preparaciones proteicas micobacterianas utilizando pruebas serológicas y de inmunidad celular. Para el estudio fueron incluidos pacientes con manifestaciones clínicas de lepra predominantemente de la forma multibacilar. El 58% correspondía a la forma clínica de Lepra Lepromatosa (LL) n=81, el 29% a la forma Borderline Lepromatosa (BL) n=41 y 10% a Borderline Bordeline (BB) n=14. Sólo el 3% fueron pacientes Borderline Tuberculoide (BT): 74% masculino y 26% femenino. El fenómeno reaccional más frecuente fue de tipo eritema nodoso leproso (ENL). Las proteínas micobacterianas ensayadas fueron: antígenos proteicos crudos totales de Mycobacterium leprae (MISA), Mycobacterium bovis (MbSA y MbSA de excreción), antígeno proteico de excreción parcialmente purificado con una movilidad relativa de 30 kDa (MI30) y proteínas recombinantes de Mycobacterium (Mt70, Mb 65, MI 36, 28, 18 y 10 KDa) encontrándose que las proteínas recombinantes (MI 10KDa, MI36 kDa) a mayor carga bacilar presentaban una mayor reactividad serológica estadísticamente significativa (p=0.0051 y 0.050 respectivamente). La proteína de 30 kDa) a mayor carga bacilar presentaban una mayor reactividad serológica estadísticamente significativa (p=0.0051 y 0.0050 respectivamente). La proteína de 30 kDa fue predominantemente reconocida por anticuerpos de los pacientes multibacilares. Los resultados demuestran que el promedio de los valores de anticuerpos en pacientes no reaccionales fueron superiores en presencia de proteínas completas (MbSA y MbSA de exreción) en comparación con el grupo de pacientes que presentaron fenómenos reaccionales (p=0.000567 y 0.000061 respectivamente). Este mismo comportamiento se observó frente a las proteínas micobacterianas individuales (30kDA, 10kDa y 36 kDa). La respuesta proliferativa de los linfocitos T en los pacientes multibacilares reaccionales y no reaccionales frente a las proteínas micobacterianas (MISA, ML 10kDa, MbSA, MbSA de excreción) fue negativa en ambos grupos (AU)

The study was designed for evaluating immunological reactivity to various mycobacterial protein preparations using serological and cell-mediated immunological test in patients with clinical leprosy signs, predominantly, with the multibacillary forms. Al patients wer adults with ages between 20 and 39 years. Fifty eight (n=81) percent corresponded to Lepromatous Leprsy (LL), 29% (n=41) to Borderline Lepromatuous Leprosy (BL) and 10% (n=14) to Borderline Borderline Leprosy (BB); only 3% were Bordeline Tuberculoid (BT) patients: 74% males and 26% females. The most frequent reactional phenomenon was of the Erythema Nodosum (ENL) type. The mycobacterial proteins tested were: total crude Mycobacterium leprae antigens (MISA); Mycobacterium bovis (MbSA and excretion MbSA); partially purified excretion protein antigen, with a 30KDa relative movility (Ml30); and recombinant M. leprae protein (Mt70, Mb 65, Ml36, 28, 18 and 10kDa). Two of the recombinant proteints (M110 and Ml36 kDA) presented a statiseally significant higher serological reactivity, directly related with a larger bacillary load (p=0.0051 and 0.050 respectively). The 30kDa protein was predominantly recognized by antibodies from multibacillary patients. Results show that mean antibody values were higher in non reactional patients when tested against complete proteins (MbSA and ex MbSA) when compared with the group of patients who presented reactional phenomena (p=0.000567 and 0.000061, respectively). Comparing reactional with non reactional patients, it was seen that mean antibody values against complete proteins (MbSA and ex MbSA) were higher in non reactional individual (p=0.000567 and 0.000061, respectively). This same behavior occurred towards individual mycobacyterial proteins (30, 10 and 36 kDa). The T lymphocyte prolyphetative response in reactional and non reactional patients towards mycobacterial proteins (MISA, Ml 10 kDA, MbSA, ex MbSA) was negative (AU)

Caracterización del comportamiento de casos de lepra en la ciudad de La Habana. 2004-2008 / Characterization of the behavior of cases of lepros in Havana city. 2004-2008

Estudio descriptivo, retrospectivo, de corte transversal, para determinar el comportamiento de la lepra en ciudad de la Habana entre los años 2004 y 2008. De un universo de 97 casos notificados en este quinquenio, se estudiaron 94 pacientes que tenían encuesta epidemiológica. Se analizaron variables sociodemográficas y clínicas para caracterizar los casos notificados. Se determinó que más de la mitad de los casos estudiados estuvo en el grupo de edad de 45 y más años (64.9%) y fue más frecuente en el sexo masculino (65.9%). El mayor número de los casos diagnosticados (79.7%) clasificaron con lepra multibacilar, correspondiendo un 54.2% de ellos a lepromatosa. El síntoma más frecuente corresponde a la presencia de máculas con un 49.2%. Considerando el resultado de la investigación realizada, se presentará una propuesta al Departamento Provincial de este programa, para capacitar al personal que trabaja en las diferentes áreas de salud.

Descriptive, retrospective, cross-sectional study, to determine the behavior of leprosy in Havana City between 2004 and 2008. In a universe of 97 cases reported in the five years, 94 patients who had an epidemiological survey were studied. Sociodemographic and clinical variables were analyzed to characterize the reported cases. It was determined that more than half of the cases studied were in the age group 45 and over (64.9%), and was more frequent in males (65.9%). The highest number of diagnosed cases (79.7%) classified as multibacillary leprosy, corresponding a 54.2% of them to Lepromatous. The most common symptom is the presence of macules in 49.2%, Considering the outcomne of the investigation, a proposal will be presented to the Provincial Department of this program to train staff working in different health areas. .

Clinical and immunological assessment of periodontal disease in Japanese leprosy patients.

Periodontitis is a chronic inflammatory disease caused by the infection of periodontopathic bacteria in dental plaque. However, an individual's susceptibility to this disease appears to be associated with multiple genetic factors, as seen in the case of leprosy. In order to gain a better understanding of the pathophysiology of periodontal disease in subjects with leprosy, we investigated the clinical features of periodontitis and the immunological responses against periodontopathic bacteria in 382 subjects with a history of leprosy and 451 age-matched control subjects. The prevalence of periodontitis and the degree of periodontal pocket depth were found to be significantly higher in leprosy patients than in age-matched controls. Furthermore, a comparison of the clinical parameters of lepromatous leprosy (L-lep) and tuberculoid leprosy (T-lep) patients showed that the probing pocket depth of L-lep patients with periodontal disease was significantly higher than that for T-lep patients. In contrast, serum IgG titers against Porphyromonas gingivalis in L-lep patients were significantly lower than in T-lep patients. These results imply that L-lep patients show more severe periodontal disease than T-lep patients or age-matched control subjects, and that low humoral immunity against P. gingivalis might be one of the genetic factors determining periodontal disease susceptibility in leprosy patients.

Seropositivity to anti-phenolic glycolipid-I in leprosy cases, contacts and no known contacts of leprosy in an endemic and a non-endemic area in northeast Brazil.

The seroprevalence rates of IgM anti-phenolic glycolipid-I (PGL-I) antibodies in four study groups with differing exposure to Mycobacterium leprae in Ceará, Brazil were investigated between March 2005 and August 2006. The first three groups in a high prevalence area included 144 cases of leprosy, their 380 contacts and 317 participants with no known leprosy contact. The fourth group in a low prevalence area consisted of 87 participants with no known leprosy contact living in an area in which no cases of leprosy had been reported in the previous 6 months. Seropositivity and levels of IgM antibodies to PGL-I were investigated using ELISA. The seropositivity levels of anti-PGL-I among the different clinical forms of leprosy cases were 61% for lepromatous, 25% for tuberculoid and 27% indeterminate. The levels of anti-PGL-I antibodies in the endemic area differentiated leprosy cases from non-cases. However, the seropositivity was similar among contact cases (15.8%) and no known leprosy contact cases from high (15.1%) and low (13.8%) prevalence areas. The seropositivity of both contacts and no known contacts was much higher than previously reported among no known contacts in other endemic areas. The study indicates that anti-PGL-I antibodies are not useful as immunological markers of household leprosy contacts and no known leprosy contacts in endemic areas.

Presence of intestinal helminths decreases T helper type 1 responses in tuberculoid leprosy patients and may increase the risk for multi-bacillary leprosy.

Resistance to intracellular pathogens such as Mycobacterium leprae is dependent upon an effective T helper type 1 (Th1)-type immune response. On the other hand, intestinal helminths are known to subvert the host's immune response towards to either a Th2-type immune response or a regulatory T cell up-regulation, which may affect the host's ability to mount an effective response to mycobacteria. Here, we report a significant association between intestinal helminth infections and lepromatous leprosy [odds ratio (OR), 10.88; confidence interval (CI) 95%: 4.02-29.4; P<0.001]. We also observed that the frequency of intestinal helminths correlated strongly with the mycobacterial index (r=0.982, P<0.01). Corroborating with our hypothesis, intracellular levels of interferon-gamma were decreased significantly in leprosy patients co-infected with intestinal helminths when compared to leprosy patients without worms. Conversely, lepromatous leprosy patients with intestinal worms produced higher levels of both interleukin (IL)-4 and IL-10. Our results suggest that a pre-existing infection by intestinal helminths may facilitate the establishment of M. leprae infection or its progression to more severe forms of leprosy.

Occurrence and management of leprosy reaction in China in 2005.

BACKGROUND: Leprosy reactions are a major cause of disability before, during and after anti-bacterial treatment. Prompt diagnosis and correct management of reaction is a crucial matter for improving the quality of leprosy health services. OBJECTIVES: To describe the pattern of leprosy reaction and its management in China during 2005. METHODS: A retrospective survey using a questionnaire was carried out in all the provinces of China at the beginning of 2006. Patients included were those presenting with leprosy reaction between 1 January and 31 December 2005. RESULTS: 452 questionnaires from 25 provinces were analysed. There were 313 male and 139 female patients who had 159 Type I reactions, 273 Type II reactions and 20 Type I and II mixed reaction. 72.4% of reactions occurred in the first year of MDT and 27.6% of patients during the second year of MDT. The highest frequency of reaction was during the first 6 months of MDT; 57.3% of patients developed new nerve impairment during and after MDT. CONCLUSIONS: New nerve function impairment and disability still occurs among patients during and after MDT. The early detection and management of leprosy reaction remains important.

Manifestaçoes fonoaudiologicas em um grupo de doentes de Hanseniase / Manifestations fonoaudiologicas in a group of patients from leprosy

A Hansenise, doença cronica infecto-contagiosa e de nofificaçao compulsoria, e causada pelo bacilo de Hansen (Mycobacterium leprae), e pode apresentar multiplas lesoes em qualquer local do corpo...

Primary neuritic leprosy: a reappraisal at a tertiary care hospital.

AIM: To study the clinico-epidemiological profile of primary neuritic leprosy. METHODS: Retrospective analysis of the leprosy records of clinically diagnosed patients of PNL who attended the Leprosy Clinic from 2000 to 2004 was carried out for details of presenting complaints, age and sex distribution, duration, number of nerves involved and pattern of nerve enlargement, BI (skin), nerve abscess, and deformities. RESULTS: There were 32 (4.6%) patients of PNL out of a total of 686. Majority (56.2%) had complaints for less than a year. There were 29 (90.6%) males, and 3 (9.4%) females, with 15-30 years as the commonest age-group (65.6%) involved. Paraesthesia and numbness were the presenting complaints in 20 (62.5%), and motor deficit (paresis) in 11 (34.4%); deformities (claw hand, foot drop, trophic changes) were seen in 16 (50%) cases. Polyneuritic pattern was noted in 21 (63.56%) patients and mononeuritic in 11 (16.5%) with ulnar nerve as the most commonly enlarged nerve (63.6%). Nerve abscess was noted in 4 (12.5%) cases. Slit-skin smear was positive in 2 (6.2%) cases only. DISCUSSION: PNL continues to be common in India. Sensory complaints are early and more common. The disease is more common in males. Polyneuritic pattern was predominant, and the ulnar nerve was the most commonly involved nerve. Majority of the cases belong to the tuberculoid spectrum. Early diagnosis depends on complete neurological examination in order to reduce the sequelae of the disease.

A recessive major gene controls the mitsuda reaction in a region endemic for leprosy.

BACKGROUND: Leprosy is a chronic infectious disease caused by Mycobacterium leprae. The Mitsuda reaction is a delayed granulomatous skin reaction elicited by intradermal injection of heat-killed M. leprae. Interestingly, results of the Mitsuda test are positive in the majority of individuals, even in areas not endemic for M. leprae. Like leprosy, the Mitsuda reaction is thought to be genetically controlled, but its mode of inheritance is unknown, although the role of the NRAMP1 gene has previously been reported. METHODS: We conducted a segregation analysis of quantitative Mitsuda reactivity in 168 Vietnamese nuclear families ascertained through patients with leprosy. RESULTS: We found strong evidence (P<10-9) for a major gene controlling the Mitsuda reaction independently of leprosy clinical status. Subsequent linkage analysis showed that this major gene was distinct from NRAMP1. Under the major-gene model, approximately 12% of individuals are homozygous for the recessive predisposing allele and are predicted to display high levels of Mitsuda reactivity (mean, approximately 10 mm, versus 5 mm in other individuals). CONCLUSION: We provide evidence that the Mitsuda reaction is controlled by a major gene. Our study paves the way for the identification of this gene and should provide novel insight into the mechanisms involved in granuloma formation, especially in M. leprae infection.

Clinico-pathological study of 12 cases of patients with leprosy admitted to Sina Hospital, Hamadan, Iran, from 1991 to 2000.

BACKGROUND: Leprosy is considered a chronic disabling condition. Many clinical and immunological aspects of the disease remain ill defined. AIM: The study of clinico-pathological and laboratory findings of patients with leprosy admitted to Sina Hospital, Hamadan, Iran, from 1991 to 2000. METHODS AND PATIENTS: This is a descriptive retrospective cross-sectional study. The statistical community comprised all patients diagnosed leprosy. This diagnosis was clinical and confirmed through pathology (skin-biopsy) and laboratory (peripheral smear) measures. RESULTS: In this study, the disease was more common in males than females with a mean age of 48.5 +/- 16.2 years. Most of the patients were more than 40 years old. Among 12 patients in this study, six cases were urban and six cases were rural. Six cases were living in Hamadan province and two cases migrated to Hamadan province (one of them from Afghanistan and the other from Kurdestan). Clinical diagnosis was confirmed by pathology in 11 cases, but in one case the clinical diagnosis did not match the pathology. In four cases the clinical diagnosis did not match the peripheral smear. Eight cases were admitted just once. Four cases had a history of recurrence and readmission (two patients had one time recurrence and the other two patients had two recurrences). There was no difference in the clinical findings between first presentation and recurrence. From the point of complication and disability, extremity disability was more common than eye disability. Increased severity of complications was found in patients with a delayed diagnosis and incomplete treatment. CONCLUSION: This study showed that a rapid and correct diagnosis and complete treatment was necessary for prevention of complication and disability in patients with leprosy. Also the accuracy of pathology (skin biopsy) in the diagnosis exceeded the peripheral smear. Skin biopsy is recommended to confirm the diagnosis in all cases of leprosy. In the absence of pathology, patients must be considered as multibacillary patients and treated as such.

Comportamiento clínico-epidemiológico de la lepra en el municipio Morón en el quinquenio 1998-2002 / Clinico-epidemiological behaviour of leprosy in Moron municipality during 1998-2002 period

Se realizó un estudio longitudinal retrospectivo para conocer el comportamiento clínico-epidemiológico de la lepra en el municipio de Morón durante el periodo de 1998 hasta el 2002. El universo de estudio estuvo representado por 30 casos de lepra. La lepra constituye un problema de salud en el municipio porque la tasa de prevalencia es superior a 1 caso x 10 a la 5 Habitantes. El año que mayor tasa presentó fue el 2000. La forma clínica más frecuente es la Lepra Tuberculoide. Más de la tercera parte de los casos de lepra se diagnosticaron sin incapacidad. No se han notificado ningún caso de lepra con incapacidad grado II. Más de la mitad de los pacientes son detectados a través del examen dermatoneurológico que realiza el médico de la familia a su población de riesgo(AU)