Palatal perforation in a patient with borderline lepromatous leprosy: leprosy still not eradicated.

Leprosy was eliminated globally in 2000, but it continues to be endemic in developing countries like India, Brazil and Indonesia, with a prevalence of 0.57/10 000 persons in India (2020). At the end of the year 2020, the prevalence was 129 389, and oral manifestation of the leprosy is luncommon. We hereby report a case of a female patient in her late 30s who presented with palatal perforation. Following a thorough history taking and full body clinical examination, we arrived at a diagnosis of leprosy, and prompt treatment was initiated. Knowledge of cases like this becomes important as the oral lesion is said to form an essential source of leprosy dissemination in the community, and awareness about them becomes crucial, demanding immediate attention.

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.

Sociodemographic and epidemiological profile of leprosy patients in an endemic region in Brazil

Abstract: INTRODUCTION: The incidence of Hansen's disease is high in overlooked populations. METHODS: Data of Hansen's disease cases reported in the information system of the Department of Informatics, Brazilian Unified Health System, from 2013 to 2014 were analyzed. RESULTS: Among 434 studied cases of Hansen's disease, the female sex (52.5%), adult age (73.7%), low educational level (61.8%), and multibacillary form were associated with higher prevalence rates. CONCLUSIONS: Hansen's disease is more frequent among female adults with a low educational level, and the prevalence of multibacillary leprosy reflects disease detection at late stages.

A study of the prevalence of smear-positive leprosy cases in a tertiary care center in the post-elimination phase of leprosy.

BACKGROUND: India has declared leprosy to be eliminated in the country, according to the WHO criteria of attaining a prevalence of less than 1 in 10,000. However, smear-positive leprosy cases are frequently being encountered. METHODS: This is an 18-year retrospective study done in the Department of Dermatology and Venereology, Government Medical College, Trivandrum. Data were collected from the records of all cases in the study period, and the prevalence of smear-positive cases was determined in the post-elimination phase and this was compared to the pre-elimination phase. RESULTS: A total of 901 cases were analyzed, which comprised 538 cases in the pre- and 363 cases in the post-elimination phase. The male/female ratio in the pre and post phases was 2.61 : 1 and 2.45 : 1, respectively. Borderline tuberculoid accounted for the commonest type in both the pre and post phases comprising 45.54 and 50.69%, respectively. Type 2 lepra reactions were seen in 40.21% of the reaction cases in the post-elimination phase. The smear-positive cases in the post-elimination phase accounted for 34.99% compared to 14.68% in the pre-elimination phase. Lepromatous leprosy (LL) accounted for 67.71% of the smear-positive cases in the post phase compared to 67.08% in the pre phase. CONCLUSIONS: There was an increase of 20.30% of smear-positive cases in the post-elimination phase, which was statistically significant (P < 0.001), and the majority of them were LL (P < 0.001).

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

Household costs of leprosy reactions (ENL) in rural India.

BACKGROUND: Erythema nodosum leprosum (ENL) is a common immune-mediated complication of lepromatous (LL) and borderline lepromatous (BL) leprosy. Most patients experience chronic or multiple acute ENL over many years during an economically active period of their lives. Understanding the economic burden of ENL is essential to provide effective patient support, yet this area has not been investigated. METHODS: Ninety-one patients with LL or BL leprosy attending a leprosy hospital in Purulia district of West Bengal, India, were interviewed using a structured questionnaire. Cases (n = 53) were identified as those who had one or more episodes of ENL within the last 3 years. Controls (n = 38) had LL or BL leprosy but no history of ENL. Data were collected on household income, direct and indirect costs, and coping strategies. FINDINGS: The total household cost was Rs 1543 per month or 27.9% (IQR 13.2-52.6) of monthly household income for cases, and Rs 237 per month or 4.9% (IQR 1.7-13.4) of monthly household income for controls. Indirect costs accounted for 65% of total household costs for cases. Direct costs accounted for the remaining 35% of household costs, and resulted almost entirely from treatment-seeking in the private sector. Total household costs exceeded 40% of household income for 37.7% of cases (n = 20) and 2.6% of controls (n = 1) [1 USD = 59 INR]. INTERPRETATION: Households affected by ENL face significant economic burden and are at risk of being pushed further into poverty. Health policy should acknowledge the importance of private sector provision and the significant contribution to total household costs of lost productivity (indirect cost). Further work is needed to explore this area and identify solutions.

Clinical aspects of leprosy.

Leprosy is a chronic, infectious disease caused by Mycobacterium leprae. It mainly affects the peripheral nervous system, skin, and certain other tissues such as the reticulo-endothelial system, bones and joints, mucous membranes, eyes, testes, muscles, and adrenals. Leprosy clinical presentation varies from few to widespread lesions. In most patients, early leprosy presents as macular and hypopigmented lesions. This initial clinical presentation is known as indeterminate leprosy and occurs in individuals who have not developed cell-mediated immunity against M. leprae yet. The number of lesions depends on the genetically determined cellular immunity of the patient. Individuals presenting a vigorous cellular immune response and limited humoral immune responses to M. leprae, usually present few skin lesions. Without treatment, those patients tend to evolve into the polar tuberculoid or borderline tuberculoid form of leprosy. Due to the inability to mount an effective cellular-mediated response to M. leprae and the consequent hematogenous spread of the bacilli, some patients may present with numerous and symmetrically distributed hypochromic lesions. Without treatment these patients evolve to a nonresistant form of leprosy, polar lepromatous.

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.

Genetic diversity of mycobacterium leprae isolates from Brazilian leprosy patients.

INTRODUCTION: Leprosy is a chronic disease caused by infection with Mycobacterium leprae, an obligate intracellular parasite. A problem in studying the transmission of leprosy is the small amount of variation in bacterial genomic DNA. The discovery of variable number of tandem repeats (VNTRs) allowed the detection of strain variation in areas with a high prevalence of leprosy. Four genotypes of M. leprae based on three single-nucleotide polymorphism (SNPs) were also discovered to be useful for analysis of the global spread of leprosy. METHODS: In this present study, we examined the allelic diversity of M. leprae at 16 select VNTR and three SNP loci using 89 clinical isolates obtained from patients mainly from the neighbouring states of São Paulo and Rio de Janeiro Brazil. RESULTS AND CONCLUSION: By use of a PCR-RFLP-based procedure that allows the recognition of SNP types 3 and 4 without the need for the more expensive DNA sequencing steps, characterisation of the main M. leprae genotypes was easy. When applied on the study population, it was found that the SNP type 3 is most frequent in these two states of Brazil, and that VNTRs provided further discrimination of the isolates. Two Short Tandem Repeats (STRs) were monomorphic, with the remaining 14 STRs represented by two to 18 alleles. Epidemiological associations with township or state were not evident in this random collection and require further investigations. In phylogenetic trees, branches formed by all 16 STRs clearly separated SNP type 3 organisms from the other types while the allelic patterns of two minisatellite loci 27-5 and 12-5 were highly correlated with SNP type 3. This strain typing study provide the basis for comparison of M. leprae strain types within Brazil and with those from other countries, and informed selection of genomic markers and methods for future studies.

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...

Clinical course of erythema nodosum leprosum: an 11-year cohort study in Hyderabad, India.

Erythema nodosum leprosum (ENL) or type 2 lepra reactions complicate lepromatous leprosy and borderline lepromatous leprosy. We report an 11-year retrospective case record analysis of 481 outpatients with borderline lepromatous and lepromatous leprosy at the Dhoolpet Leprosy Research Center in Hyderabad, India.. The overall prevalence of ENL was 24%, 49.4% among cases of lepromatous leprosy (LL) and 9% among cases of borderline lepromatous (BL) leprosy. Logistic regression analysis identified LL (odds ratio [OR] = 8.4, 95% confidence interval [CI] = 4.6-15.4, P < 0.001) and BL with a bacterial index > or = 4+ (OR = 5.2, 95% CI = 2.1-12.9, P = 0.001) as major risk factors. The average patient with ENL was male, 34.7 years of age, and had multiple episodes of ENL (mean = 3.1) over an 18.5-month period. Three types of ENL were identified: single acute ENL, multiple acute ENL (repeated discrete episodes), and chronic ENL (continuous episodes). Acute single ENL is rare, accounting for only 8% of cases. Chronic ENL accounted for 62.5% of the cohort. Chronic ENL was of longer duration and more severe. An age > or = 35 years was a risk factor for developing chronic ENL. Patients with chronic ENL were more compliant with multi-drug therapy, especially during the first six doses of multi-drug therapy. Distinguishing these different types of ENL would be useful for patient management and developing improved treatment of these debilitating reactions. Improved strategies for treatment and management of these reactions need to be developed.

Perfil clinico-epidemiológico dos casos de hanseníase atentidos no Hospital Universitário em Campo Grande, Mato Grosso do Sul, de janeiro de 1994 a julho de 2005 / Clinical-epidemiological profile of leprosy patients assisted at the University Hospital of Mato Grosso do Sul Federal University, Campo Grande, MS, from January 1994 to July2005

Estudo descritivo, realizado a partir da coleta de dados de 192 fichas de notificação e controle da hanseníase, do total de pacientes atendidos no período de janeiro de 1994 a julho de 2005, no Ambulatório do Hospital Universitário da Universidade Federal de Mato Grosso do Sul, com o objetivo de traçar o perfil epidemiológico da hanseníase no grupo de pacientes estudados e gerar subsídios à política de controle da hanseníase. As variáveis estudadas constam da ficha de notificação e controle da hanseníase. Observou-se a predominância de casos no sexo masculino (62,5%); na faixa etária de 40 a 59 anos (45,8%); multibacilares (67,2%); da forma clinica dimorfa (35,9%) e virchowiana (27,6%). Setenta e três (73%) por cento dos casos foram avaliados em relação à incapacidade ao inicio do tratamento, encontrando-se 66,7% desses casos sem nenhum problema com as mãos, pés ou olhos e 33,3% com incapacidade ou deformidade ao início do tratamento.

Increased incidence of cytoplasmic ANCA (cANCA) and other autoantibodies in leprosy patients from western India.

The prevalence of various autoantibodies was studied in 75 leprosy patients comprising eight patients with lepromatous leprosy (LL), 36 patients with borderline lepromatous leprosy (BL) and 31 patients with borderline tuberculoid leprosy (BT), along with 100 normal controls. Certain autoantibodies such as anti-nuclear antibodies (ANA), anti-single stranded DNA (anti-ssDNA) and anti-neutrophil cytoplasmic antibodies (ANCA) were raised among leprosy patients. When ANCA specificities to anti-myeloperoxidase (anti-MPO), anti-proteinase3 (anti-PR3) and anti-lactoferrin (anti-LF) were studied, it was found that the patterns of immunofluorescence such as perinuclear (p-ANCA), cytoplasmic (c-ANCA) and atypical (X-ANCA) and specificity by ELISA to anti-MPO, anti-PR3 and anti-LF varied in the LL, BL and BT groups. However, a higher amount of c-ANCA was observed in 62.5% of leprosy cases, while the incidences of p-ANCA and X-ANCA were lower. The LL group showed a higher incidence of autoantibodies as compared with the BL and BT groups, along with a male preponderance for autoantibody development. Some unusual antibody profiles such as 'X'-ANCA were also observed. The study suggests that autoantibody formation could be quite prevalent and also variable in the spectrum of leprosy cases, and there seems to be a serological overlap among leprosy and autoimmune disease, which could have pathogenetic importance in the leprosy patients developing complications.

Analysis of 6000 skin biopsies of the national leprosy control program in Mexico.

Six thousand skin biopsy specimens taken from April 1978 to January 2002 under conditions as specified by the National Leprosy Control Program (NLCP), were analyzed to obtain information about the work of the program and contribute to the knowledge of this illness in the Mexico. Six-thousand request forms for histologic exam of the NLCP were reviewed. Sixty-two percent of the requests had all the required information and in 38% one or more data items were omitted. The age range was 2 to 98 yrs with a median of 50 yrs; a small number of cases was observed in the age group of 0 to 14 yrs, and the peak was in the age group of 41 to 50 yrs. Of the 6000 biopsies, 3693 were classified. Polar lepromatous (LL) was the most common form of the disease, in 60.3% of cases. Twice as many cases were multibacillary leprosy (MB) as paucibacillary (PB). MB predominated in males, and PB predominated in females. The Cohen's kappa index (kappa) of clinical-histological agreement was 0.202 (95% CI 0.184-0.219) and showed a poor grade of agreement between clinical and histologic diagnosis, with a level of significance of 0.05 (p <0.001). The results may indicate the end of leprosy in Mexico, a country in which the national goal of elimination was reached in 1994, with a prevalence since the year 2000 of 0.17/10 000.

The relapse rate in MB leprosy patients treated with 2-years of WHO-MDT is not low.

A group of multibacillary patients is clearly at high risk for relapse following 2-yr WHO-MDT. Relapse is largely confined to BL or LL patients with a high BI initially, and occurs long after the discontinuation of therapy. This important group of patients at risk for treatment failure presents several important issues: the need to identify those at risk and the operational requirements needed for their long term follow-up. Also, this group of patients might well benefit from an alternative antimicrobial regimen from the outset, as well as upon relapse.

Ocular complications in newly diagnosed borderline lepromatous and lepromatous leprosy patients: baseline profile of the Indian cohort.

AIM: To describe ocular manifestations in newly diagnosed borderline lepromatous (BL) and lepromatous leprosy (LL) patients in India. METHODS: Ocular complications, at enrolment, occurring in all new borderline lepromatous and lepromatous leprosy patients detected by active case finding within the geographically defined leprosy endemic area of the Gudiyattam Taluk in India from 1991 to 1997 who consented to ocular examinations every 6 months, during and 5 years after treatment with multidrug therapy (MDT), were studied. RESULTS: Orbicularis oculi weakness (4.62%), lagophthalmos (4.20%), ectropion (0.42%), trichiasis (0.84%), blocked nasolacrimal ducts (1.68%), pterygium (11.34%), impaired corneal sensation (53%), corneal opacity (10.5%), corneal nerve beading (1.68%), punctate keratitis (1.26%), keratic precipitates (4.62%), iris atrophy (1.68%), and cataract (12.6%) were ocular complications seen in the 301 lepromatous patients at enrolment. 4.6% had blind eyes. Increasing age was associated with ocular complications. 80% of patients were skin smear acid fast bacilli (AFB) positive. The LL/BL ratio was 1:6.4. 71% had some limb deformity. 44% had only leprosy related ocular complications (LROC), 28% had only general ocular complications (GOC) while 14% had both LROC and GOC. Ocular complications were significantly related to leg deformities. Corneal nerve beading was seen most in LL patients (100%) having high bacterial content. Lagophthalmos and muscle weakness were associated with reversal reactions. CONCLUSIONS: Corneal nerve beading occurs in LL patients with high bacillary count. Patients with reversal reaction are more likely to present with orbicularis oculi weakness and lagophthalmos. Leprosy related ocular complications and general ocular complications are significant problems in newly diagnosed lepromatous patients. Elderly, deformed, skin smear positive, lepromatous patients are associated with increased ocular morbidity and form a group that require acceptable and accessible eye care.