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Erythema nodosum leprosum (ENL) is a serious, often recurring and disabling, immunologically mediated reaction occurring in leprosy which often requires hospitalization. There are published several studies of ENL, but systematic studies regarding the risk factors associated with ENL in the post elimination era are few. The aim of the study was to determine the risk factors associated with ENL in a tertiary care centre in Western Odisha. This is a case control study involving 292 patients of leprosy who attended the Dermatology OPD of this tertiary care centre. These constituted 97 patients with ENL and 195 patients without ENL who attended the OPD during this period. Detailed history, clinical examination, slit skin smears were done. These included gender details, age, area of residence (rural/urban), education and socioeconomic status. The most common subtype of leprosy observed in ENL was lepromatous leprosy followed by borderline lepromatous type. Patients diagnosed with initial high BI and lepromatous leprosy were found to be significant risk factors for development of ENL. Skin diseases, Anaemia and Diabetes Mellitus were found to be more prevalent in ENL patients
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In highly endemic countries like India, where tuberculosis (TB) and leprosy infection may coexist, screening the other disease before initiating treatment is important to prevent Rifampicin resistance since both diseases are treated with and sensitive to Rifampicin. Here, we report a leprosy case involving the unmasking of leprosy in a treated patient with Pulmonary TB. In this case, a high index of suspicion of Erythema Nodosum Leprosum (ENL) in a patient with no history of leprosy disease or treatment with anti-leprosy drugs was observed. He, however, had a history of taking anti-tuberculous medicine 1.5 years earlier. This case report also acknowledges the physician’s prompt referral of this patient to a dermatologist. Taking a detailed family history and screening helped us diagnose leprosy in the patient’s daughter. It also emphasises the atypical presentation of leprosy, which (although described in textbooks) is being reported here.
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Erythema nodosum leprosum (ENL) is an immune complex mediated type III hypersensitivity reaction seen in patients of borderline lepromatous and lepromatous leprosy. It can be caused by a wide array of triggers and can be seen before, during, or after completion of anti-leprosy therapy. There are multiple well-known triggers for type 2 reactions like the initiation of multidrug therapy, Mantoux testing, vaccination, mental and physical stress, and physiological states like pregnancy. Herein, we report a case of exacerbation of ENL in a middle-aged woman, probably due to COVID-19 vaccine while she was well-controlled on immunosuppressive therapy. The episode was treated with non-steroid anti-inflammatory drugs and oral steroids and the symptoms resolved within 2 weeks. Although causality was highly possible between the occurrence of ENL and COVID-19 vaccine, physicians should be aware that it can be easily managed with proper care and medicines and this should not be a basis for deferring the vaccine.
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Leprosy is a chronic disease with clinical presentations according to the immunologic spectrum. Lepromatous form is the most advanced, with the highest transmissibility and risk of causing disabilities. Lucio’s phenomenon is a rare manifestation among lepromatous patients with a rapid and severe evolution and high mortality. It is difficult to differentiate from ulcerative/necrotic erythema nodosum leprosum and has no consensus on how it should be treated. This article is a qualitative review of the literature after the introduction of multidrug therapy, aiming to bring consensus related to the clinical, laboratory and histopathological diagnostic criteria of the disease and its management
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Recurrent erythema nodosum leprosum (ENL) is a type 2 leprosy reaction, which often poses a challenge for the treating physician. Patient may be non-ambulatory due to systemic symptoms like fever, body aches, joint pains, and painful skin lesions. Moreover, drugs like corticosteroids and thalidomide in refractory cases pose an increased risk of deep vein thrombosis (DVT). We have described a case who developed DVT in due course of the treatment.
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Relatamos um caso de uma paciente feminina, gestante de terceiro trimestre, em acompanhamento pré-natal regular na unidade básica de saúde, com boa evolução gestacional, porém apresentando lesões de pele há cerca de um ano, acompanhadas de alteração de sensibilidade, além de fáscies infiltrada e madarose. Sendo o Brasil um país endêmico em Hanseníase, ocupando o 2º lugar no mundo em número de novos casos, chama a atenção o diagnóstico tardio da paciente em questão. Aproveitamos este emblemático relato de caso para discutir aspectos importantes em relação à terapêutica no período gestacional (poliquimioterapia conforme manual do ministério, sem nenhuma alteração por conta da gestação), desfecho obstétrico, orientações quanto à lactação (não contra-indicada com a mãe em tratamento; pelo contrário, devendo ser estimulada) e cuidado ao recém nato. [au]
We report a case of a pregnant female patient in the third trimester undergoing regular prenatal care at a Basic Health Unit, with good gestational evolution, but presenting skin lesions for approximately a year accompanied by changes in sensitivity, in addition to facial infiltration and madarosis. Considering Brazil as an endemic country for leprosy, ranking 2nd in the world concerning the number of new cases, late diagnosis of the patient in question stands out. We use this emblematic case report to discuss important aspects concerning the treatment of leprosy during the gestational period (multidrug therapy according to the Ministry of Health manual, without any changes due to pregnancy), obstetric outcome, guidelines regarding breastfeeding (not contraindicated with the mother in treatment; on the contrary, it should be stimulated) and care for the newborn. [au]
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The cases of leprosy occur rarely in North America and a health care professional may not readily consider a diagnosis of leprosy. We encountered a 23-year-old female with hypopigmented macules and painful nodules along with peripheral neuropathy in Oregon, who had immigrated from Micronesia. A skin biopsy confirmed the diagnosis of leprosy. Patient developed a type 2 reaction, Erythema nodosum leprosum, upon initiation of the multidrug therapy. It is vital to recognize the signs and symptoms of leprosy and associated reactions so the patient can be treated appropriately to prevent debilitating and stigmatizing neurocutaneous and systemic illness.
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Histoid Hansen's disease is a form of lepromatous leprosy with characteristic bacterial morphology and very high bacillary load. The occurrence of de novo' Histoid leprosy in patients without other features of leprosy has been reported. We present our observations on eleven cases of Histoid Hansen's disease who had attended our Outpatient Department. A detailed clinical history and clinical examination was done. Slit skin smear for Bacteriological Index and skin biopsy were performed in all. All eleven cases were males. Cutaneous and subcutaneous nodules were the commonest skin lesions found. Slit skin smear revealed abundant organisms. Histopathology was consistent with Histoid Hansen. Seven were de novo cases. During treatment, one patient developed type II reaction. All patients except one showed clinical clearance of lesions and fragmented bacilli after one year of MB-MDT. The treatment was extended in this single case who did not respond well. Awareness about the clinical features, characteristic histology and early diagnosis of Histoid Hansen is of utmost importance. It will be important to determine the bacteriological and morphological indices at the end of the treatment for all Histoid Hansen disease patients. Continuation of treatment may be decided based upon the bacterial and morphological indices.
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Background: Erythema nodosum leprosum is an immune-mediated complication of leprosy which causes significant morbidity. Biomarkers in the pathogenesis of erythema nodosum leprosum are not yet fully determined. Aim: To determine macrophage migration inhibitory factor levels in the sera of leprosy patients with erythema nodosum leprosum and to correlate the same with clinical parameters. Methods: This cross-sectional study included 37 consecutive leprosy patients with active erythema nodosum leprosum and 31 age- and sex-matched controls. Detailed clinical history and examination findings were recorded including the severity and frequency of erythema nodosum leprosum. Slit skin smears and histopathologic examination were done in all patients at baseline. Serum macrophage migration inhibitory factor levels were determined using an enzyme-linked immunosorbent assay. Results: Most of our patients were males (78.4%) and suffering from lepromatous leprosy (27, 73%) with a mean initial bacillary index of 3.38 ± 1.36. Recurrent and chronic patterns of erythema nodosum leprosum were seen in 15 (40.5%) and 6 (16.3%) patients, respectively. Most (86.5%) of our patients presented with moderate to severe erythema nodosum leprosum. The mean serum macrophage migration inhibitory factor level was 21.86 ± 18.7 ng/ml among patients while it was 11.78 ± 8.4 ng/ml in the control group (P < 0.01). There were no statistically significant correlations of macrophage migration inhibitory factor levels with erythema nodosum leprosum frequency or severity. Limitation: Serum macrophage migration inhibitory factor levels in leprosy patients with no erythema nodosum leprosum and in patients with other inflammatory and autoimmune conditions were not assessed. Hence, this study falls short of providing the predictive value and specificity of higher macrophage migration inhibitory factor concentrations in serum as a biomarker of erythema nodosum leprosum. Conclusion: Macrophage migration inhibitory factor levels are elevated in erythema nodosum leprosum patients as compared to controls. A larger sample size and macrophage migration inhibitory factor gene polymorphism analysis will be needed to elucidate the role of this pro-inflammatory cytokine in erythema nodosum leprosum.
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@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Erythema nodosum leprosum is an immune-mediated complication of leprosy whose underlying mechanism has not yet been fully elucidated, making management difficult.</p><p style="text-align: justify;"><strong>OBJECTIVES:</strong> To determine the serum cytokine profile of ENL compared to non-reactional leprosy states.</p><p style="text-align: justify;"><strong>METHODS:</strong> An open literature search was performed using MEDLINE, Cochrane Library, TRIP and HERDIN electronic databases using the keywords ("cytokines" or "inflammatory mediators") and ("erythema nodosum leprosum" or "ENL") and ("leprosy" or "lepra"). Studies were selected by two independent review authors. Risk of bias was assessed using the Newcastle-Ottawa Scale and statistical analysis was performed using RevMan 5.3 software.</p><p style="text-align: justify;"><strong>RESULTS:</strong> Eight cross-sectional studies with 197 participants were included. Meta-analysis showed that both serum IL-17 and serum IFN-? were significantly decreased (Z 2.39, p = 0.02 and Z 2.74, p = 0.01, respectively) in ENL compared to non-reactional states. However, for IL-1?, IL-6, IL-10, IL-22, TNF-? and TGF-?, no significant differences were found between the two groups.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> ENL appears to be an exacerbation of the Th2 cytokine response seen in the lepromatous pole of leprosy. However, despite pooling of data, sample sizes remain small resulting in significant heterogeneity. Future studies involving large sample sizes and investigating a wider range of cytokines are encouraged.</p>
Subject(s)
Cytokines , LeprosyABSTRACT
BACKGROUND: Lepra reactions occur in 10-30% of patients with leprosy. The standard of treatment is prednisone. However , prolonged steroid use may cause side effects such as osteoporosis, hypertension, hyperlipidemia, atherosclerosis and infections. Fusidic acid targets cytokine systems responsible for the production of Type 1 lepra reaction (T1R) and erythema nodosum leprosum (ENL). It may be given as a steroid-sparing agent in treating lepra reactions. OBJECTIVE: To determine the safety and efficacy of fusidic acid as a steroid-sparing agent in the treatment of Type 1 and Type 2 lepra reactions. METHODS: A randomized controlled trial was conducted on 67 subjects with lepra reactions, aged 18-60, each assigned to receive either prednisone or prednisone + fusidic acid for 12 weeks. Severity of lepra reactions were graded quantitatively using a modified scale by Walker et al and van Brakel et al, and qualitatively using modified National Leprosy Control Program (NLCP) Guidelines at baseline, weeks 2,4,6,8,10 and 12. Doses of prednisone needed to control lepra reactions were also noted at each follow up and statistical analyses were done . Adverse reactions were noted. RESULT: Sixty subjects (89.55%) completed the study. The prednisone + fusidic acid group had lower quantitative and qualitative scores compared to the prednisone group. There were significant differences between the two groups for the quantitative severity scores (p=1.44x10-11) and qualitative severity grading (p=9.36x10-14) at week 12. The mean dose of prednisone was 21.5 mg in the prednisone group and 2 mg in the prednisone + fusidic acid group at week 12 (p=1.01x10-12). No adverse reactions were reported. CONCLUSION: Fusidic acid tablet 250mg/tab two tablets three times a day is an effective and safe steroid-sparing agent for the treatment of lepra reactions.
Subject(s)
Humans , Male , Female , Leprosy , PrednisoneABSTRACT
Leprosy is a chronic infectious disease caused by Mycobacterium leprae, a microorganism that usually affects skin and nerves. Although it is usually well-controlled by multidrug therapy (MDT), the disease may be aggravated by acute inflammatory reaction episodes that cause permanent tissue damage particularly to peripheral nerves. Tuberculosis is predominantly a disease of the lungs; however, it may spread to other organs and cause an extrapulmonary infection. Both mycobacterial infections are endemic in developing countries including Brazil, and cases of coinfection have been reported in the last decade. Nevertheless, simultaneous occurrence of perianal cutaneous tuberculosis and erythema nodosum leprosum is very rare, even in countries where both mycobacterial infections are endemic.(AU)
Subject(s)
Leprosy, Lepromatous , Research Report , Mycobacterium lepraeABSTRACT
Leprosy is a chronic infectious disease caused by Mycobacterium leprae, a microorganism that usually affects skin and nerves. Although it is usually well-controlled by multidrug therapy (MDT), the disease may be aggravated by acute inflammatory reaction episodes that cause permanent tissue damage particularly to peripheral nerves. Tuberculosis is predominantly a disease of the lungs; however, it may spread to other organs and cause an extrapulmonary infection. Both mycobacterial infections are endemic in developing countries including Brazil, and cases of coinfection have been reported in the last decade. Nevertheless, simultaneous occurrence of perianal cutaneous tuberculosis and erythema nodosum leprosum is very rare, even in countries where both mycobacterial infections are endemic.
Subject(s)
Animals , Leprosy/pathology , Infections , Mycobacterium leprae , Review Literature as TopicABSTRACT
In the current scenario of leprosy elimination, lepra reactions (LRs) remain a major persistent problem. Type 1 LR (T1LR) and type 2 LR (T2LR) are the major causes of nerve damage and permanent disabilities. The immunopathogenesis of LR have recently become an important fi eld of research, since it may provide the relevant targets for the early detection and control of these episodes. Presently, there are no uniformly acceptable laboratory markers for LR. Genetic and serum markers in human host may predict susceptibility to reactions as well as progression of nerve damage in leprosy. Therefore, a deeper understanding of the molecular mechanisms involved in LR may provide a rational strategy for early diagnosis and prevention of the catastrophic consequences of LR.
Subject(s)
Animals , Biomarkers/blood , Cytokines/blood , Cytokines/genetics , Humans , Immunity, Innate/physiology , Leprosy/blood , Leprosy/diagnosis , Leprosy/genetics , Mycobacterium leprae/genetics , Mycobacterium leprae/metabolismABSTRACT
Os autores relatam o caso de uma paciente de 14 anos, do sexo feminino, com eritema nodoso hansênico, em tratamento com predinisona em altas doses, de difícil controle há dois anos, que iniciou o quadro concomitante à introdução de poliquimioterapia para tratamento de hanseníase multibacilar. O manuseio do eritema nodoso hansênico em pacientes pediátricos é um grande desafio na área médica. O único tratamento formalmente indicado é a prednisona, já que a talidomida está contraindicada em pacientes menores de 12 anos e mulheres em idade reprodutiva. É sabida a grande quantidade de efeitos colaterais deletérios da corticoterapia crônica em crianças; além disso, ainda há pacientes refratários ao tratamento. Portanto, fica evidente a necessidade de discussão da terapêutica atual, sendo de suma importância o incentivo à pesquisa de novas drogas para o combate a essa moléstia...
Subject(s)
Humans , Female , Adolescent , Child , Erythema Nodosum , LeprosyABSTRACT
The clinical course of leprosy is often interrupted by reactions, which are acute inflammatory episodes that can be classified as type I or type II. Type II reactions can present as cutaneous lesions that resemble erythema multiforme (EM). EM is classically associated with drug allergies or pre-existing viral infections. However, the differential diagnostic criteria of the diverse causative agents remain controversial. The aim of this study was to determine both the clinical relevance and the morphological and immunohistochemical characteristics of the EM-like lesions during the course of type II leprosy reactions. Twenty-seven skin biopsies were taken from typical EM-like lesions of multibacillary patients and reviewed; their histological features were correlated to their clinical aspects. Then, a computer-assisted morphometric analysis was performed to measure the extent of angiogenesis during these acute episodes. The histopathological and immunohistochemical analysis of the EM lesions revealed that they shared the same features that have been previously described for ENL, including immunopositivity in the identical cell-mediated immune markers. Our results point to leprosy as the cause of the EM-like lesions in our patients. Therefore, leprosy should be considered in the differential diagnosis of EM.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Erythema Multiforme/pathology , Leprosy, Lepromatous/pathology , Biopsy , Diagnosis, Differential , ImmunohistochemistryABSTRACT
Type-1 (T1R) and Type-2 (T2R) leprosy reactions (LR), which affect up to 50% of leprosy patients, are aggressive inflammatory episodes of sudden onset and highly variable incidence across populations. LR are often diagnosed concurrently with leprosy, but more frequently occur several months after treatment onset. It is not uncommon for leprosy patients to develop recurring reactional episodes; however, they rarely undergo both types of LR. Today, LR are the main cause of permanent disabilities associated with leprosy and represent a major challenge in the clinical management of leprosy patients. Although progress has been made in understanding the immunopathology of LR, the factors that cause a leprosy patient to suffer from LR are largely unknown. Given the impact that ethnic background has on the risk of developing LR, host genetic factors have long been suspected of contributing to LR. Indeed, polymorphisms in seven genes [Toll-like receptors (TLR)1, TLR2, nucleotide-binding oligomerisation domain containing 2, vitamin D receptor, natural resistance-associated macrophage protein 1, C4B and interleukin-6] have been found to be associated with one or more LR outcomes. The identification of host genetic markers with predictive value for LR would have a major impact on nerve damage control in leprosy. In this review, we present the recent advances achieved through genetic studies of LR.
Subject(s)
Humans , Biomarkers , Leprosy , Leprosy/genetics , Leprosy/immunology , Leprosy/pathologyABSTRACT
There are two types of leprosy reactions: reversal reactions or type 1 and erythema nodosum leprosum or type 2. Deformity and disability associated with leprosy are frequently the result of uncontrolled or untreated reactions. Although there is current availability of glucocorticoids as the mainstay of therapy, much needs to be learned about the etiology, risk factors, and pathogenesis of leprosy reactions. There is some evidence that leprosy reactions may represent, particularly, erythema nodosum leprosum autoinflammatory disease due to the aberrant activation of the innate immune system. The role for herpesviruses influencing autophagy in macrophages needs to be evaluated in the pathogenesis of leprosy reactions.
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INTRODUÇÃO: A talidomida é um fármaco utilizado atualmente no tratamento do eritema nodoso hansênico no Brasil. MÉTODOS: Estudo prospectivo para acompanhar a evolução clínica, registrar os eventos adversos e determinar as concentrações plasmáticas de talidomida em dose diária de 100mg/dia, em 20 pacientes com manifestações clínicas de eritema nodoso hansênico, divididos em dois grupos: após ou em curso da poliquimioterapia para hanseníase. RESULTADOS: Não foram observadas diferenças significativas nos grupos no decorrer do estudo, tanto na evolução clínica favorável dos pacientes, de 70 por cento e 90 por cento, quanto nos eventos adversos registrados que foram tontura e sonolência. Os teores plasmáticos de talidomida em D7 e D14 foram de 0,82±0,4μg/mL e 0,79±0,3μg/mL no grupo 1 e de 0,82±0,4 e 1,55±1,0 no grupo 2, respectivamente. CONCLUSÕES: Na amostra estudada, a poliquimioterapia não interferiu na evolução clínica, na incidência dos efeitos adversos e nos níveis plasmáticos de talidomida.
INTRODUCTION: Thalidomide is a drug currently used in Brazil for treating erythema nodosum leprosum. METHODS: This was a prospective study to follow up clinical evolution, record adverse events and determine plasma thalidomide levels from a dose of 100 mg/day, among 20 patients with clinical manifestations of erythema nodosum leprosum, divided into two groups: during or after leprosy multidrug therapy. RESULTS: No significant differences between the groups were seen during the study, either in relation to favorable clinical evolution among the patients (70 percent and 90 percent), or in relation to the adverse events recorded, which were dizziness and somnolence. The plasma thalidomide levels on D7 and D14 were 0.82 ± 0.4μg/ml and 0.79 ± 0.3 μg/ml in group 1 and 0.82 ± 0.4 and 1.55 ± 1.0 in group 2, respectively. CONCLUSIONS: In this sample, the multidrug therapy had no effect on the clinical evolution, incidence of adverse events and plasma thalidomide levels.
Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Erythema Nodosum/drug therapy , Leprostatic Agents/therapeutic use , Leprosy, Lepromatous/drug therapy , Thalidomide/therapeutic use , Erythema Nodosum/blood , Leprostatic Agents/adverse effects , Leprostatic Agents/blood , Leprosy, Lepromatous/blood , Prospective Studies , Thalidomide/adverse effects , Thalidomide/blood , Young AdultABSTRACT
ENL is a type ll leprosy reaction and occurs in people with borderline lepromatous and lepromatous leprosy, usually as a complication following treatment. The treatment of choice for ENL is prednisolone in view of its’ ready availability and affordability1. Howeve r, glucocorticoid therapy, even in low doses, can produce substantial toxicity. The risk is clearly greater as the dose increases. However, in cases where there are steroid-induced complications, high-dosed clofazimine may be used to reduce or withdraw corticosteroids in steroid-dependant cases2,3. We described 2 steroid-dependent ENL patients with steroid-induced complications who are successfully managed with the addition of highdosed clofazimine and the resultant weaning down of systemic glucocorticoids.