Enfermedad de Hansen: comunicación de un caso de lepra tuberculoide en Chile / Hansen's disease: case report of tuberculoid leprosy in Chile

Resumen La enfermedad de Hansen o lepra es una enfermedad infecciosa crónica causada por Mycobacterium leprae, y que afecta principalmente la piel y nervios periféricos. En los últimos años, se ha logrado un mejor control de la enfermedad en forma global. Sin embargo, en Chile la incidencia de la lepra ha ido en aumento. Presentamos el caso clínico de un paciente de 40 años, haitiano, con lesiones e histopatología compatibles con una lepra tuberculoide. Se discute la importancia en la educación sobre enfermedades menos prevalentes en Chile y se hace una revisión sobre la fisiopatología, clínica y clasificación de la enfermedad de Hansen.

Abstract Hansen's disease or leprosy is a chronic infectious disease caused by Mycobacterium leprae, mainly affecting the skin and peripheral nerves. In recent years, a better control of the disease has been achieved globally. However, in Chile the incidence of leprosy has continued to increase. We present a case of a 40-year-old patient Haitian nationality with lesions and histopathology compatible with tuberculoid leprosy. We discuss the importance of education on less prevalent diseases in Chile, in conjunction with a review of the pathophysiology, clinic, and classification of Hansen's disease.

Pure neural leprosy or amyloid neuropathy? Systematic review and clinical case report

SUMMARY OBJECTIVE: To review the literature and to report a clinical case with initial suspicion of pure neural leprosy and final diagnosis of amyloid neuropathy. METHODS: The study was conducted in two stages. In stage one, a systematic literature review was carried out, with searches performed in the PubMed, Medline, and Lilacs databases, as well as in the leprosy sectoral library of the Virtual Health Library, using the following descriptors: neuritic leprosy, pure neural leprosy, primary neural leprosy, pure neuritic leprosy, amyloid polyneuropathy, amyloid neuropathies, and amyloid polyneuropathy. The search was carried out on May 28, 2020. Clinical trials, cohort studies, cross-sectional studies, clinical cases, and case studies published in Portuguese, English or Spanish between 2010 and 2020 were included. Stage two reports a case with initial suspicion of pure neural leprosy. Laboratory tests, electroneuromyography, ultrasound, and biopsy of the sural nerve were requested. RESULTS: Twenty-three scientific texts were included. No publications were found that contained both topics together. The challenging diagnosis of pure neural leprosy and the possibility of using auxiliary resources in diagnosis were the most emphasized themes in the studies. In the clinical case, the patient's electroneuromyography showed sensitive and motor polyneuropathy of the lower limbs, which was predominantly sensory and axonal, symmetrical, of moderate intensity, and the mixed type (axonal-demyelinating). Ultrasonography of the sural nerve revealed changes in the contour of the deep fibular nerves; biopsy of the sural nerve showed an accumulation of amorphous eosinophilic material in the nerve path, and Congo red stain showed apple-green birefringence of the deposit under polarized light. The final diagnosis was amyloid neuropathy. CONCLUSIONS: The final clinical diagnosis was amyloid neuropathy. The diagnosis of pure neural leprosy in endemic areas in Brasil is still a challenge for the health system.

Perfil de pacientes que concluíram o tratamento poliquimioterápico da hanseníase: um estudo transversal / Perfil de pacientes que concluyeron el tratamiento poliquimioterapéutico de la lepra: un estudio transversal / Profile of patients who completed multidrug therapy treatment for leprosy: a cross-sectional study

RESUMO Introdução: a hanseníase é uma doença infectocontagiosa que prevalece como problema de saúde pública no Brasil. Objetivo: descrever o perfil sociodemográfico, de tratamento e clínico de pacientes que concluíram o tratamento poliquimioterápico para a hanseníase. Método: estudo transversal, desenvolvido de novembro de 2017 a fevereiro de 2018 no município de Natal. A população-alvo foi constituída por 113 indivíduos cadastrados no Sistema de Informação de Agravos de Notificação e a amostra por 90, que foram notificados para a hanseníase e concluíram o tratamento poliquimioterápico, selecionados por sorteio. Os dados foram coletados por formulário, tabulados e analisados pelo SPSS 21. Quanto ao teste de Kolmogorov-Smirnov, não se observou uma distribuição de normalidade dos achados, optando-se por trabalhar com testes não paramétricos: qui-quadrado de Pearson (ou exato de Fisher) e o teste de Mann-Whitney. Resultados: predominaram indivíduos do sexo feminino, até 59 anos, com baixa renda, baixo grau de escolaridade, tratados nos centros de referência e classificados como paucibacilares. Conclusão: as condições de vulnerabilidade associada à predominância de casos tratados nos centros de referência reforçam a necessidade de organização da atenção básica para acompanhamento dos casos de hanseníase.

RESUMEN Introducción: la lepra es una enfermedad infectocontagiosa que prevalece como problema de salud pública en Brasil. Objetivo: describir el perfil sociodemográfico, de tratamiento y clínico de pacientes que concluyeron el tratamiento poliquimioterapéutico para la lepra. Método: estudio transversal, desarrollado de noviembre de 2017 a febrero de 2018 en el municipio de Natal. La población objetivo fue constituida por 113 individuos registrados en el Sistema de Información de Agravios de Notificación y la muestra por 90, que fueron notificados para la lepra y concluyeron el tratamiento poliquimioterapéutico, seleccionados por sorteo. Los datos fueron recogidos por formulario, tabulados y analizados por el SPSS 21. En cuanto a la prueba de Kolmogorov-Smirnov, no se observó una distribución de normalidad de los hallazgos, optándose por trabajar con pruebas no paramétricas: chi-cuadrado de Pearson (o exacta de Fisher) y la prueba de Mann-Whitney. Resultados: predominaron individuos del sexo femenino, hasta 59 años, con bajos ingresos, bajo grado de escolaridad, tratados en los centros de referencia y clasificados como paucibacilares. Conclusión: las condiciones de vulnerabilidad asociadas al predominio de casos tratados en los centros de referencia refuerzan la necesidad de organizar la atención básica para el seguimiento de los casos de lepra.

ABSTRACT Introduction: leprosy is an infectious and contagious disease that persists as a public health problem in Brazil. Objective: to describe the sociodemographic, treatment and clinical profile of patients who completed polychemotherapy treatment for leprosy. Method: cross-sectional study developed from November 2017 to February 2018 in the city of Natal. The target population consisted of 113 individuals registered in the Information System for Notifiable Diseases, and the sample consisted of 90, who were reported for leprosy and completed the multidrug therapy, selected by drawing lots. Data were collected using a form, tabulated and analyzed using SPSS 21. In the Kolmogorov-Smirnov test, non-normal distribution of the findings was found, and thus non-parametric tests were used, namely, Pearson's chi-square (or exact Fisher test) and the Mann-Whitney test. Results: there was a predominance of female individuals, up to 59 years old, with low income, low level of education, treated in reference centers and cases classified as paucibacillary. Conclusion: the conditions of vulnerability associated with the predominance of cases treated in reference centers reinforce the need to organize primary care in order to monitor leprosy cases.

Fibrosis: a distinguishing feature in the pathology of neural leprosy

BACKGROUND Fibrosis in the peripheral nerve is the end stage of leprous neuropathy and the cause of the resulting permanent neural function impairments. Preventive measures to avoid this irreversible pathological state are a relief strategy for leprosy sufferers. OBJECTIVES The present study describes the frequency of fibrosis along with its characterisation and pathogenic development. METHODS Six-hundred-and-thirteen nerve samples were sorted from 278 neural leprosy (NL) and 335 non-leprosy neuropathy patients (ON). The total number of samples was histologically examined by routine staining methods (haematoxylin-eosin, Wade staining and Gomori's trichrome) and fibrosis was evaluated via semi-quantitative estimation. FINDINGS Fibrosis was most frequent in the NL group (33% against 0.4% in ON) while fibrosis in association with endoneurial microfasciculation was found in 38 (41.3%) of the NL samples in the examination of semithin sections. Pericytic activation in the perivascular environment was confirmed to be the source of the fibroblasts and perineurial cells delimiting microfascicles. End-stage fibrosis in leprosy displays an arrangement of microfascicles devoid of neural components (i.e., Schwann cells and axons) lined by an intermediate phenotype of fibroblastic-perineurial cells filled with bundles of collagen fibres. MAIN CONCLUSIONS The present study underscores that fibrosis is frequently the severe end stage of neural leprosy NL pathogeny after analysing the notably distinct development of fibrosis within the neural environment.

A case series of pure neural leprosy in patients diagnosed in a specialized center for the control of Hansen's disease in Colombia / Serie de casos de lepra neural pura en pacientes diagnosticados en un centro especializado en el control de la enfermedad de Hansen en Colombia

Abstract Pure neural leprosy, defined as a peripheral neuropathy in which the patient has no skin lesions, is difficult to diagnose. Its verification by bacteriological index and histopathology is not possible in the majority of the patients. We describe four cases of pure neural leprosy diagnosed by clinical criteria. The clinical outcome of three of the patients after specific treatment was satisfactory, while the other one developed progressive neural damage despite the therapy. All patients were treated in a specialized center for the management and control of Hansen's disease in the municipality of Contratación, Santander, Colombia.

Resumen La lepra neural pura se presenta como una neuropatía periférica sin presencia de lesiones cutáneas. La verificación del diagnóstico mediante el índice bacilary la histopatología, no es posible en la mayoría de los pacientes. Se describen cuatro casos de lepra neural pura diagnosticados por clínica; la evolución de tres de los pacientes que recibieron tratamiento específico fue satisfactoria, en tanto que la otra paciente presentó deterioro progresivo a pesar de las medidas terapéuticas. Todos los pacientes fueron atendidos en un centro especializado en el manejo y control de la enfermedad de Hansen, ubicado en el municipio de Contratación, Santander, Colombia.

Peripheral nerve tumor, a differential diagnosis with primary neural leprosy: five case reports / Tumor de nervo periférico, diagnóstico diferencial com hanseníase neural primária: relato de cinco casos

From December 1999 to May 2015, five patients with nerve tumors were sent to Lauro de Souza Lima Institute. It was suspected that they suffered from primary neural leprosy towards the tuberculoid clinical form, a prevalence of 4.5:10000 among the new patients assessed during the study period. All of the patients had similar clinical conditions characterized by mononeuropathy with nerve tumor associated with pain, absence of skin lesions and positive Mitsuda reaction. The authors report the main clinical characteristics and complementary tests: immunologic investigation of Mitsuda's reaction and the antigen Phenolic GlicoLipid-1 test (PGL-1), bacilloscopic index, neurophysiologic study and image procedures. All patients were submitted to tumor resection and anatomopathological study. Four out of the five patients were diagnosed with peripheral nerve tumor (one of them with malignant schwannoma, two of them with benign schwannomas and the other with neural fibrolipoma), whereas the fifth patient was diagnosed with tuberculoid leprosy.

No período de dezembro de 1999 a maio de 2015, foram encaminhados ao Instituto Lauro de Souza Lima (ILSL) cinco pacientes com tumoração em nervos suspeitos de hanseníase neural primária (HPN) da forma clinica tuberculoide, uma prevalência de 4,5/10000 entre os casos novos atendidos nesse período. Todos os pacientes apresentavam quadro clínico semelhante caracterizado por mononeuropatia com tumoração do nervo associada à dor, ausência de lesões de pele e reação de Mitsuda positiva. Os autores relatam as principais características clinicas e os exames complementares: investigação imunológica da reação de Mitsuda e o teste do antigeno Glicolipídeo-Fenólico-1, índice baciloscópico, avaliação neurofisiológica e estudos de imagem. Todos os pacientes foram submetidos a ressecção cirúrgica do tumor e estudo anatomopatológico. Dentre os cinco pacientes, quatro foram diagnosticados como tumor de nervo periférico (um Schwannoma maligno, dois Schwannomas benignos e um fibrolipoma neural) e um como hanseníase tuberculoide.

Modified simple decompression in the treatment of cubital tunnel syndrome: avoiding ulnar nerve subluxation / Descompressão simples modificada no tratamento da síndrome do túnel cubital: evitando subluxação do nervo ulnar

ABSTRACT Objective In this study, we propose a modification to the simple decompression technique that contains the ulnar nerve in the cubital fossa, thus preventing subluxation during forearm flexion movements. Methods Five consecutive patients with leprosy-associated cubital tunnel syndrome underwent surgery with the modified technique between July 2011 and October 2012. Results The most common symptoms were neuropathic pain and sensory changes (both 60%). On the McGowan scale, three patients maintained their preoperative score and two patients improved by two points, while on the Louisiana State University Health Sciences Center scale, two patients maintained the same scores, two improved by two points, and one improved by one point. Four patients were able to discontinue corticosteroid use. The mean follow-up time was 25.6 months (range 2-48 months). There were no recurrences or subluxations in the long-term. Conclusion This alternative technique resulted in excellent functional results, as well as successful withdrawal from corticosteroids. Furthermore, it resulted in no ulnar nerve subluxations.

RESUMO Objetivo Neste manuscrito apresentamos uma modificação da técnica de descompressão simples do nervo ulnar no túnel cubital que impede a subluxação do nervo em movimentos de flexão do antebraço. Métodos Foram incluídos cinco pacientes consecutivos acometidos por síndrome do túnel cubital (Hanseníase) submetidos à cirurgia entre 2011 e 2012. Resultados Os sintomas mais comuns foram dor neuropática e alterações sensitivas (60%). No pós-operatório, três pacientes mantiveram o mesmo escore e dois melhoraram dois pontos na escala de McGowan, enquanto na escala Louisiana State University Health Sciences Center, dois pacientes mantiveram o mesmo escore, dois melhoraram dois pontos e um melhorou um ponto. Os corticosteróides foram descontinuados em quatro pacientes. O tempo médio de seguimento foi 25,6 meses (variação 2-48 meses). Não foram observadas recorrência ou subluxação no longo prazo. Conclusões A técnica alternativa apresentou excelentes resultados funcionais e foi bem sucedida na retirada dos corticosteróides. Ademais, subluxações do nervo ulnar não foram observadas.

Paracoccidioidomycosis with sarcoid-like lesions: a diagnostic challenge

Abstract Clinical presentation of paracoccidioidomycosis (PCM) can be diverse. Morphology and quantity of skin lesions depends on interactions between host immunity and fungus virulence. Diagnosis can be a challenge considering that this fungus has low virulence and some individuals have immunity to microorganism, which results in well-marked granulomas without visible microorganisms. We report herein a clinical presentation of sarcoid-like PCM, initially diagnosed as tuberculoid leprosy. This rare type of PCM is often mistaken for other types of chronic granulomatous diseases. Diagnosis was confirmed after 4 years when a special stain analysis helped in the identification of the specific etiologic agent.

Lepra neural pura de 18 años de evolución / 18 years of pure neuritic leprosy

La lepra puede presentar dificultades diagnósticas, especialmente en la forma neural primaria. Un hombre de 38 años, exsoldado y trabajador rural, presentó durante más de 10 años hiperestesia supraclavicular y supraescapular izquierdas y anestesia progresiva escapular y en guante de la mano y brazo izquierdos, con reabsorción ósea de las falanges distales del primero y segundo dedos de la misma mano, cambios que le dificultaban realizar sus labores; no tenía lesiones cutáneas de lepra. El nervio cubital izquierdo presentaba engrosamiento epitroclear, por lo que se sospechó lepra neural pura. Las baciloscopias de moco, pabellones auriculares y codos fueron negativas, al igual que los anticuerpos IgM contra el glicolípido fenólico 1; dos biopsias de piel de zonas anestésicas fueron normales también. La electromiografía evidenció disminución notoria de los potenciales sensitivos de los nervios cubital, radial y mediano izquierdos, lo cual favoreció el diagnóstico de lepra neural primaria. Se inició tratamiento para lepra multibacilar por tener afección de más de un tronco nervioso, dos meses después comenzó a presentar mejoría notoria de los síntomas y logró reanudar sus actividades comunes y laborales. La lepra neural primaria cursa con zonas de hipoestesia y anestesia cutánea y engrosamiento neural troncular, puede permanecer sin diagnóstico durante años. La electromiografía, que demuestra disminución de los potenciales sensitivos, es una ayuda diagnóstica considerable, mientras la biopsia de piel anestésica ayuda al diagnóstico solo en la tercera parte de los casos. La sospecha clínica razonable es suficiente para iniciar tratamiento antileproso, cuando no se dispone de otros medios diagnósticos.

Leprosy can be difficult to diagnose, in particular the pure neuritic leprosy type. A 38-year-old male, former soldier and rural worker, presented with a 10-year history of supraclavicular and suprascapular hyperesthesia of the left side and progressive scapular left arm anesthesia, including the hand, associated with bone resorption in distal phalanges of the first and second finger. No typical leprosy skin changes were present. An examination of the skin revealed epitrochlear thickening of the left side cubital nerve, therefore primary neural leprosy was suspected. Skin smears from the routine sites were normal, as were two skin biopsies and the serologic assay specific for M leprae to detect phenolic glycolipid-1. Electromyography revealed an important reduction in the sensory action potential of the ulnar, radial and medial nerves, which favored the primary neural leprosy diagnosis. After two months of multibacillar leprosy treatment, the symptoms started to disappear and the patient could recommence his old job. Pure neuritic leprosy presents with skin areas of hypesthesia and anesthaesia associated with nerve thickening. It is a disease that is commonly misdiagnosed for several years before the correct diagnosis is made and effective treatment is started. Electromyography can be a helpful tool in the diagnosis, typically showing reduced sensory action potential, while a biopsy of anesthetic skin is only helpful in one-third of cases. Reasonable clinical suspicion is sufficient to initiate antileprosy treatment when no other diagnostic methods are available.

Follicular mucinosis: an important differential diagnosis of leprosy in an endemic area

Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.

New cases and types of leprosy in Interior Sindh: an analysis of 3 years [2012-2014]

Objective: To evaluate the new cases and clinical types of leprosy in interior Sindh

Methods: Data analysis and evaluation of 3-year record of leprosy centers/clinics in Interior Sindh

Results: Of total 238 new registered cases, 98 [41%] were diagnosed after a delay of 1 year. The types of disease found were tuberculoid 59 [25%], borderline tuberculoid 100 [42%], borderline 13 [5%], borderline lepromatous 53 [22%] and lepromatous lepromatous 13 [5%] cases. Multibacillary cases were 179 [75%] and paucibacillary 59 [25%]

Conclusion: The 3-year analysis of Interior Sindh shows endemic regions of leprosy in Pakistan which will help in devising strategy for an effective leprosy control program

Epidemiological indicators and clinical profile of leprosy cases in Dhaka

Objective: To evaluate epidemiological indicators and clinical profile of leprosy patients in Dhaka City

Methods: In this cross-sectional observational study 722 new leprosy patients registered in six different clinics of The Leprosy Mission International - Bangladesh, Dhaka program over two and half year [January 2011 to June 2013] were included. Demographic details and clinical characteristics during diagnosis were recorded

Results: Out of 722 patients, 390 [54%] were males and 332 [46%] were females. Proportion of cases under 15 year age was 8.7%. Borderline tuberculoid was the most common form of the disease [81.0%] followed by tuberculoid [9.3%], lepromatous [4.3%], borderline lepromatous [3.5%], borderline [1.8%] and pure neural [0.1%]. Proportion of multibacillary leprosy was 22.4%. Most of the patients had duration of symptoms from 6 months to 1 year [53.0%]. 12% of patients had history of contact with leprosy patients. Type 1 reaction was more prevalent than type 2 [7.6% and 2.9%, respectively]. Proportion of cases with grade 2 disability was 5.9%. Most common presentation was with hypopigmented macule with obvious margin, marked anesthesia and mild infiltration. Ulnar nerve was the most common nerve to be involved [15.8%]

Conclusion: Leprosy cases are being frequently diagnosed in Dhaka city. Epidemiological indicators reflect that there may be ongoing disease transmission and relative delay in diagnosis despite a strong surveillance program

case of neurofibromatosis type1 with coexisting borderline tuberculoid leprosy

Leprosy and neurofibromatosis are different entities. Both diseases present with nerve enlargement and skin lesions but the coexistence is rare. Nodules of neurofibromatosis may be mistaken for that of lepromatous leprosy and vice versa. Their simultaneous occurrence in same person may lead to diagnostic dilemma. We report a case which was at first mistaken as lepromatous leprosy. But proper history and examination aided by slit skin smear and skin biopsy for histopathological examination helped to reach the appropriate diagnosis of borderline tuberculoid leprosy associated with neurofibromatosis

A Case of Tuberculoid Leprosy without Sensory Loss in a Male Immigrant / 대한피부과학회지

Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae. The prevalence and number of new cases have recently markedly decreased in Korea, and the possibility of leprosy can therefore be clinically overlooked. However, leprosy is still endemic in various regions of the world. A 30 year-old male immigrant from Indonesia presented with an erythematous plaque without sensory loss on his face six months after immigration. The skin lesion was diagnosed as tuberculoid leprosy based on clinico-pathology.

Atypical lesions in relapsed leprosy

Relapsed cases of leprosy are not common in patients treated regularly with the recommended polychemotherapeutical schemes. Relapses must be readily identified and distinguished from reactions. This article reports a clinical case of relapse in leprosy, with atypical and exuberant skin changes in patient treated for eleven years with a paucibacillary scheme.