Hansen Disease (Leprosy) and Armadillo Exposure in Arkansas: A Case Series.

BACKGROUND: Although most cases of Hansen disease (HD) in the United States are imported from endemic areas, a subset of cases are relate to exposure to nine-banded armadillos. Several recent cases of HD in Arkansas occurred in patients who had not traveled to endemic areas and who reported variable degrees of armadillo exposure. OBJECTIVE: The purpose of this study was to report 6 cases of HD diagnosed in Arkansas between 2004 and 2016. The secondary purpose was to explore the correlation between exposure to the nine-banded armadillo as it pertains to transmission of the disease. METHODS: The referring clinician of each patient was contacted to gather information regarding the patient's clinical presentation, armadillo exposure, and travel history. In addition, the Arkansas Department of Health was consulted to review the demographics of individuals diagnosed with HD in the past 15 years and to review the distribution of HD throughout the state of Arkansas. RESULTS: Six domestic cases of HD were associated with both direct and indirect exposure to armadillos. LIMITATIONS: Armadillo exposure may be underreported in patients with HD because of fear of stigmatization and/or lack of access to care. CONCLUSIONS: Direct exposure to armadillos does not appear to be required for transmission of HD making a soil-mediated mechanism of indirect exposure plausible.

Diffuse Multibacillary Leprosy of Lucio and Latapí with Lucio's Phenomenon, Peru.

Diffuse multibacillary leprosy of Lucio and Latapí is mainly reported in Mexico and Central America. We report a case in a 65-year-old man in Peru. He also had Lucio's phenomenon, characterized by vascular thrombosis and invasion of blood vessel walls by leprosy bacilli, causing extensive skin ulcers.

Bullous Erythema Nodosum Leprosum as the First Manifestation of Multibacillary Leprosy: A Rare Phenomenon.

Erythema nodosum leprosum (ENL) may uncommonly present before treatment in patients with multibacillary leprosy. Atypical manifestations are known in ENL and may be clinically misleading. Such wide variations in the clinical presentation of leprosy in reaction make histopathology an important tool for supporting clinical diagnosis. We report bullous ENL presenting as the first manifestation of leprosy in a 30-year-old Indian man diagnosed using histopathology.

Association of viable Mycobacterium leprae with Type 1 reaction in leprosy.

UNLABELLED: The working hypothesis is that, viable Mycobacterium leprae (M. leprae) play a crucial role in the precipitation of Type 1 reaction (T1R) in leprosy. MATERIAL AND METHODS: A total of 165 new multibacillary patients were studied. To demonstrate presence of viable M. leprae in reactional lesion (T1R+), three tests were used concurrently viz. growth in the mouse foot pad (MFP), immunohistochemical detection of M. leprae secretory protein Ag85, and 16s rRNA--using in situ RT-PCR. Mirror biopsies and non reactional lesions served as controls (T1R-). FINDINGS: A significantly higher proportion of lesion biopsy homogenates obtained at onset, from T1R(+) cases have shown unequivocal growth in MFP, proving the presence of viable bacteria, as compared to T1R(-) (P < 0.005). In contrast, few Mirror biopsies were positive in both T1R(+) and T1R(-). With respect to Ag85, while the overall positivity was higher in T1R(+) (74%), however the intensity of staining (Grade 2+) was disproportionately higher in T1R(+) BT-BB lesions 11/20 (55%). In the rebiopsies obtained during a repeat episode of T1R, Ag 85 as well as 16s rRNA, positivity (62% & 100%) was higher in T1R(+). It is inferred therefore 'viable' bacteria are an essential component in T1R and difference in the quality of bacilli, not the quantity or the ratio of dead to viable play a role in the precipitation of T1R. In conclusion, the findings show that 'metabolically active' M. leprae is a component/prerequisite and the secretory protein Ag 85, might be the trigger for precipitation of T1R.

Unilateral Cauliflower Ear due to Leprosy or Trauma - A Diagnostic Challenge.

The terminology Cauliflower ear refers to the appearance of external ear, sequelae to underlying primary inflammation/infection. Trauma and secondary infection or perichondritis being the most common cause of cauliflower ear, this article describes a case of unilateral cauliflower ear due to multibacillary leprosy. This case shows the importance of skin smears and the histopathological identification and classification of leprosy patients with unusual lesions.

Multibacillary leprosy mimicking systemic lupus erythematosus: case report and literature review.

Leprosy is an infectious chronic disease with a wide range of clinical and serological manifestations. We report a case of a woman presenting with a malar rash, painless oral ulcers, photosensitivity, arthritis, positive antinuclear antibodies test and leuko-lymphopenia. Our case illustrates an unusual presentation of leprosy initially diagnosed as systemic lupus erythematosus (SLE). After the confirmation of multibacillary leprosy and multidrug therapy recommended by the World Health Organization, a good clinical response was observed. Recognition of rheumatic manifestations in leprosy is important as they may be confused with SLE. A literature review is presented to encourage clinicians to consider leprosy as a differential diagnosis. Specifically in patients with unusual rheumatic manifestations and persistent skin lesions, and when neurological symptoms are present. Leprosy has not been eradicated, so misdiagnosis can be frequent. It is necessary to increase medical practitioner awareness in order start proper treatment.

Factors associated with anti-phenolic glycolipid-I seropositivity among the household contacts of leprosy cases.

BACKGROUND: The diagnosis of leprosy is based on clinical symptoms of the disease, which may not be sufficient to ensure early diagnosis. The development of effective tools for the early detection of infection, such as rapid tests that can be applied by non-specialists for early-stage leprosy identification, has been considered a research priority and may contribute to overcoming the complications associated with late diagnosis. The aim of this study was to analyze the factors associated with anti-phenolic glycolipid-I (PGL-I) seropositivity among the household contacts of leprosy cases. METHODS: A cross-sectional study of individuals from the northeastern municipalities of the state of Minas Gerais, Brazil, was performed. Anti-PGL-I seropositivity was evaluated by assessing specific antibody production using the ML Flow test. A Poisson regression with a robust error variance was used to evaluate the relationship between anti-PGL-I seropositivity and the independent variables investigated. RESULTS: The overall anti-PGL-I seropositivity was 13.5 %, and among the contacts of leprosy cases that were classified as paucibacillary or multibacillary, it was 8.4 and 17.3 %, respectively. The factors associated with the variation of anti-PGL-I seropositivity among the study population were the presence of signs suggestive of leprosy (PR = 3.68; 95 % CI: 1.56-8.71), the operational leprosy classification (PR = 2.17; 95 % CI: 1.22-3.86) and grade 1 (PR = 1.83; 95 % CI: 1.02-3.26) or grade 2 disability (PR = 2.42; 95 % CI: 1.02-5.47) of the index leprosy case. CONCLUSIONS: The presence of signs suggestive of leprosy and the operational classification of leprosy cases were associated with anti-PGL-I seropositivity. The serological tests available for leprosy are not considered to be diagnostic tests but can be used as auxiliary assessments in combination with clinical parameters to identify exposed individuals at high risk of developing leprosy and those exhibiting the initial stages of this disease.

Progression of leprosy disability after discharge: is multidrug therapy enough?

OBJECTIVE: To evaluate the risk factors related to worsening of physical disabilities after treatment discharge among patients with leprosy administered 12 consecutive monthly doses of multidrug therapy (MDT/WHO). METHODS: Cohort study was carried out at the Leprosy Laboratory in Rio de Janeiro, Brazil. We evaluated patients with multibacillary leprosy treated (MDT/WHO) between 1997 and 2007. The Cox proportional hazards model was used to estimate the relationship between the onset of physical disabilities after release from treatment and epidemiological and clinical characteristics. RESULTS: The total observation time period for the 368 patients was 1 570 person-years (PY), averaging 4.3 years per patient. The overall incidence rate of worsening of disability was 6.5/100 PY. Among those who began treatment with no disability, the incidence rate of physical disability was 4.5/100 PY. Among those who started treatment with Grade 1 or 2 disabilities, the incidence rate of deterioration was 10.5/100 PY. The survival analysis evidenced that when disability grade was 1, the risk was 1.61 (95% CI: 1.02-2.56), when disability was 2, the risk was 2.37 (95% CI 1.35-4.16), and when the number of skin lesions was 15 or more, an HR = 1.97 (95% CI: 1.07-3.63). Patients with neuritis showed a 65% increased risk of worsening of disability (HR = 1.65 [95% CI: 1.08-2.52]). CONCLUSION: Impairment at diagnosis was the main risk factor for neurological worsening after treatment/MDT. Early diagnosis and prompt treatment of reactional episodes remain the main means of preventing physical disabilities.

Wade histoid leprosy: histological and immunohistochemical analysis.

Histoid leprosy is a rare multibacillary form that presents with disseminated papule-nodular cutaneous lesions. To study the inflammatory infiltrate of the histoid form and to compare it with other lepromatous forms, we performed histological and immunohistochemical analysis on skin biopsies. Fifteen patients were included for histopathological analysis (10 histoid and five lepromatous) via the haematoxylin-eosin and Ziehl-Neelsen-Faraco stains. Thus, immunohistochemical techniques using immunoperoxidase assay were performed for: anti-BCG, anti-M. leprae, anti-CD8, anti-CD3, anti-CD20, anti-S100, anti-CD1a, anti-CD68 and antivimentin. Spindle cells were present in all histoid patients. A pseudocapsule was observed in half of both studied forms. A comparison using the Ziehl-Neelsen-Faraco stain to evaluate anti-BCG and anti-M.leprae showed no major differences. The CD3+ cells were more pronounced in the histoid form than the lepromatous form. There was greater immunoreactivity toward CD8+ cells in the histoid form, as well as the CD20+ cell count. A similar count of S100+ cells in the epidermis of both leprosy forms was observed. There was a slight increase of dendritic cells in the histoid patients in the superficial and deep dermis. For CD1a marker, we observed expression in the epidermis and superficial dermis in both forms. A diffuse and intense infiltrate of CD68+ cells was also observed in the histoid and lepromatous forms. The high positivity for vimentin did not allow for a positive cell count. We concluded that the activation of both the cellular and humoral response is more pronounced in the histoid form because the T and B cells showed greater infiltration than those in the lepromatous form. The activation of dendritic and Langerhans cells is similar in both forms. The spindle cells likely belong to the macrophage population, thus maintaining phagocytic ability. The quantities of pseudocapsules and bacilli are similar and cannot serve as criteria for diagnosis.

Twelve months fixed duration WHO multidrug therapy for multibacillary leprosy: incidence of relapses in Agra field based cohort study.

BACKGROUND & OBJECTIVES: The reported low relapse rates after 24 months multidrug therapy (MDT) for multibacillary leprosy (MB) led to the recommendation of reducing duration of therapy to 12 months. However, only a few reports exist on long term follow up data after 12 months fixed duration therapy (FDT). The present study was done to assess the incidence of relapse in MB leprosy patients after 12 months treatment. METHODS: The leprosy patients detected in field surveys during 2001-2006 in Agra district, Uttar Pradesh, India, were put on WHO-MDT and followed up for treatment completion, relapse, reactions and development of disability. The assessment was done clinically by following up the patients until January 2011. Data collected were analyzed for risk and survival analysis. RESULTS: The incidence of relapse was found to be 1.97/100 person years of follow up. The incidence of relapse by age (34 yr vs >34 yr), sex (male vs female), delay in detection (<36 months vs >36 months) and smear status (smear +ve vs -ve) was not found to be significantly different but patients with no nerve involvement were observed to have significantly higher relapses than those with three or more nerve involvement (P<0.05). Similarly, borderline-borderline and BB with reaction (BB/BBR) patients were observed to have significantly high relapses than among those with borderline tuberculoid or BT with reaction (BT/BTR) or borderline lipromatous/lepromatous/neuritic (BL/LL/N) type of leprosy (P<0.01). INTERPRETATION & CONCLUSION: From the observations in the study, it can be suggested that relapses occur in 12 months FDT and almost as much as reported in 24 months FDT for MB leprosy. Although, early relapses may be due to insufficient treatment, late relapses may be due to persistent dormant mycobacteria. However, a study relating to immunological response of treatment and change in immunological profile relating to the occurrence of relapses and its clinical correlates may suggest better information on causes of relapses.

Gynaecothelia--a common yet ignored sign of multibacillary leprosy in males: a case series with review of literature.

Leprosy is one of the causes of gynaecothelia (enlargement of nipples), however little has been published about this common but usually ignored sign. Herein, we report nine male patients with multibacillary leprosy who had gynaecothelia although only two of them had associated gynaecomastia. None of these patients was aware of gynaecothelia until it was detected by the treating doctor during examination. This study is presented to highlight this common but ignored sign, which may occur specifically in multibacillary leprosy.

A multi-centre study on quality of routine data collection on relapses.

OBJECTIVES: To assess the quality of routine data collection on relapses in leprosy programmes in six countries. DESIGN: Through a questionnaire for project managers. RESULTS: The number of reported relapse cases did not correlate with number of new cases in the individual projects. Even where available, skin smears were not always used for diagnosis of MB cases and relapses. The diagnosis of relapses in the participating projects was exclusively on clinical basis - in 91.8% on the basis of new lesions. CONCLUSION: Criteria for identification of relapses were not known or not applied in uniform manner in the projects involved in the research.

Revalidation of various clinical criteria for the classification of leprosy--a clinic-pathological study.

UNLABELLED: WHO guidelines classify leprosy patients clinically into PB and MB group based on the number of skin lesions (NSL) with > or = 6 skin lesions as a criterion for MB leprosy. Other clinical criteria for classification are based on the number of body areas affected (NBAA) and on size of the largest skin lesions (SLSL). They are also fairly simple and easily practicable in the field. OBJECTIVES: The objective of this study is to explore whether sensitivity and specificity of the WHO classification can be improved by addition of clinical criteria based on NBAA and SLSL to WHO classification. STUDY DESIGN: Among 100 newly diagnosed untreated leprosy patients classified into PB and MB group according to WHO classification, the NSL and NBAA were recorded and the size (longest diameter) of largest skin lesion was measured in centimeters. The Receiver Operator Characteristic (ROC) curves were plotted for each parameter to find the best cut off point (with highest sensitivity and specificity). RESULTS: The sensitivity and specificity of the WHO classification tested, using slit-skin smear (SSS) and skin biopsy results as the gold standard, was found to be 63% and 85% respectively. The ROC curve for NSL found the best cut off of three and more lesions for MB group (sensitivity 90% & specificity 80%). Similarly, ROC curves for NBAA and SLSL found the best cut off points for classification into MB group to be two or more (sensitivity 90% & specificity 75%) and 5 cm or more (sensitivity 87% and specificity 65%) respectively. On combining all these criteria together sensitivity was increased to 98.5% with no significant change in specificity, which was 77.5%. CONCLUSION: The study concluded that the sensitivity of the present clinical classification can be further improved by addition of two other clinical criteria.

Leprosy in transplant recipients: report of a case after liver transplantation and review of the literature.

Leprosy still is an important public health problem in several parts of the world including Brazil. Unlike the diseases caused by other mycobacteria, the incidence and clinical presentation of leprosy seems little affected in immunosuppressed patients. We report the first case, to our knowledge, of a liver transplant patient who developed multi-bacillary leprosy. The patient presented with papules and infiltrated plaques with loss of sensation suggestive of leprosy 3.5 years after living-related liver transplantation for autoimmune hepatitis. A skin biopsy showing non-caseating macrophagic granulomas, neuritis, and intact acid-fast bacilli on Fite-Faraco stain, confirmed the diagnosis of borderline lepromatous leprosy. The donor of the liver did not show any evidence of leprosy. During follow-up, the patient presented 2 episodes of upgrading leprosy type I reactions, 1 mild before leprosy treatment, and 1 moderate 3 months after receiving standard multi-drug treatment (rifampicin, clofazimine, and dapsone). These reactions were accompanied by increase in liver function tests, especially of canalicular enzymes. This reaction occurred despite the patient's triple immunosuppression regimen. The moderate reaction was successfully treated with further immunosuppression (prednisone, 0.5 mg/kg). Currently, the patient is asymptomatic, off leprosy medication, with routine liver transplant follow-up. The dilemmas in diagnosis and management of such a case are discussed and the literature on leprosy in transplant recipients is reviewed.

[Histoid leprosy in Morocco: retrospective study of 18 cases]. / Lèpre histoïde au Maroc: etude rétrospective de 18 cas.

INTRODUCTION: Histoid leprosy is a special and rare anatomoclinical form of multibacillary leprosy. Most of the few large series describing this entity In literature have been reported from India. The purpose of this study was to analyze the epidemiological, clinical and therapeutic characteristics of patients with histoid leprosy in Morocco. MATERIAL AND METHODS: This retrospective study was conducted at the National Centre of Leprology (CNL) in Casablanca from January 1991 to December 2006. Only histologially confirmed cases of histoid leprosy were included. Epidemiological, clinical, histological and therapeutic characteristics were compiled from records and analyzed using EPI-Info version 6. RESULTS: Confirmed histoid leprosy accounted for 18 of the new cases of leprosy recorded at CNL recorded during the 16-year study period. There were 13 men and 5 women with a mean age of 35.2 years. Family exposure was noted in 9 patients (50%) including two who had more than two relatives with leprosy. Nodules/subcutaneous nodules were the most common morphological pattern (88.8%). Neurological involvement was found in 10 patients. Seven patients had grade 1 deformities. Slit skin smears from histoid lesions revealed abundant bacilli with a high bacteriological index ranging from 3 + to 5 + according to the Ridley index. All patients received antibiotic treatment according to the Moroccan protocol. Outcome was favorable in all patients. Six patients (33.3%) developed erythema nodosum leprosum during the course of disease. With a average follow-up of 9 years, no recurrence has been observed. DISCUSSION: Because of its multibacillary character and despite its rarity, histoid leprosy poses a challenge to the leprosy eradication in Morocco. Like other forms of multibacillary leprosy, histoid leprosy requires early detection and prompt multidrug therapy. These requirments should be made a priority in the national program against leprosy.

Histoid leprosy presenting as a large tumor.

Histoid leprosy is a rare form of multibacillary leprosy, characterized by the presence of papules, plaques, or nodules whose appearance is keloid-like, skin colored, or erythematous. Fusiform cells are the main histopathological feature. Due to the fact that it can simulate other dermatological lesions, for example, dermatofibroma and neurofibroma, it constitutes a diagnostic challenge for clinicians and pathologists. It is a bacilliferous form of leprosy, and it plays an important role in disease transmission. A case of a patient with histoid leprosy living in the Northeast Region of Brazil is reported.