Histoid leprosy presenting as a large tumor

Abstract 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.

Células gigantes en lepra lepromatosa: dermatitis difusa con células gigantes exuberantes de tipo cuerpo extraño en lepra lepromatosa tratada / Giant cells lepromatous leprosy: Diffuse dermatitis with exuberant foreign body giant cells in treated lepromatous leprosy

Resumen Los pacientes con lepra lepromatosa que han recibido tratamiento durante años, usualmente requieren seguimiento con biopsias de piel para detectar lesiones persistentes o si la baciloscopia es positiva, incluso si los valores son menores que los iniciales. Se presenta el caso de una mujer de 48 años de edad con lepra lepromatosa de 15 años de evolución, índice bacilar de 4 en el extendido directo y en la biopsia, que recibió tratamiento con múltiples medicamentos durante 32 meses, aunque lo recomendado por la Organización Mundial de la Salud (OMS) es una duración de 12 meses. Se tomó una biopsia de piel para determinar si la enfermedad estaba activa. Se observó inflamación dérmica difusa con numerosas células gigantes de tipo cuerpo extraño y macrófagos vacuolados (células de Virchow). Estas células, CD68 positivas, contenían material granular ácido-alcohol resistente positivo con inmunohistoquímica para BCG. Se encontraron bacilos fragmentados y el índice bacilar fue de 2. Se interpretó como una forma residual de lepra lepromatosa y se concluyó que la paciente no requería prolongar el tratamiento con múltiples medicamentos. Este perfil histológico se ha observado en casos similares, pero sin datos clínicos estas biopsias representan un reto diagnóstico. La acumulación de lípidos en estas células gigantes se debe a la destrucción bacilar y a la fusión de macrófagos vacuolados. Se revisó el papel de los lípidos del bacilo y del huésped en la patogenia de la lepra lepromatosa. En estos casos, no es necesario extender los 12 meses de tratamiento con múltiples medicamentos recomendados por la OMS. En el seguimiento de los pacientes, se recomienda contar con los hallazgos clínicos, la baciloscopia, la biopsia anual de piel y los títulos IgM antiglucolípido fenólico.

Abstract Patients with lepromatous leprosy that have received treatment for many years usually get follow up biopsies for persistent skin lesions or positive bacilloscopy even if the values are lower than in the initial bacilloscopy. We report the case of a 48-year old woman with long-standing lepromatous leprosy of 15 years of evolution, with a bacterial index of 4 in the direct smear and the initial skin biopsy. The patient was treated with multidrug therapy for 32 months although the treatment recommended by the World Health Organization (WHO) is only for 12 months. A skin biopsy was taken to determine if there was an active disease. We observed a diffuse dermal inflammation with numerous foreign body giant cells and vacuolated macrophages (Virchow´s cells). These cells contained granular acid-fast material that was also positive with immunohistochemistry for BCG. There were fragmented bacilli and the BI was 2. These cells were also strongly positive for CD68. The biopsy was interpreted as a residual form of lepromatous leprosy that did not require further multidrug therapy. We have observed similar histological profiles in several cases. The lack of clinical data makes it a histological challenge. The accumulation of lipids in these giant cells is due to bacillary destruction and fusion of vacuolated macrophages. We discuss here the role of bacillary and host lipids in the pathogenesis of lepromatous leprosy. We concluded that there was no need to extend the 12-month multidrug therapy recommended by WHO. Clinical findings, bacilloscopy, annual skin biopsy, and anti-phenolic glycolipid-I IgM titers are recommended procedures for the follow-up of these patients.

Epidemiological profile of leprosy reactions in a referral center in Campinas (SP), Brazil, 2010-2015

Abstract: Leprosy patients can present reactions during the course of the disease. There are no official data on these reactions in Brazil. We aimed to describe the epidemiological characteristics of patients with such reactions, analyzing information from patient records at a referral center in Campinas (SP), from 2010 to 2015.

Hanseníase: graves consequências do diagnóstico tardio: relatos de dois casos e breve abordagem sobre suas sequelas / Leprosy: severe consequences of late diagnosis: reports of two cases and brief approach on its sequels

Hanseníase é doença infectocontagiosa de grande relevância para o Brasil. País que juntamente com a Índia e a Indonésia, notificam 81% dos novos casos mundialmente. Provocam desde lesões em pele até graves lesões em olhos, nervos periféricos, endotélio vascular, ossos e articulações, implicando em importantes sequelas e consequências psicossociais. O diagnóstico precoce dos casos interrompe a cadeia epidemiológica de transmissão da doença e diminui consideravelmente o risco de incapacidade. Relataremos 2 casos de hanseníase em fases avançadas, incapacitantes e transmissíveis, cujo estigma das deformidades, levou ao autoisolamento retardando a procura por diagnóstico(AU).

Leprosy is an infectious contagious disease of great relevance to Brazil, which together with India and Indonesia, report 81% of new cases worldwide. They cause from skin lesions to severe lesions in eyes, peripheral nerves, vascular endothelium, bones, and joints, implying important sequelae and psychosocial consequences. Early diagnosis of cases interrupts the epidemiological transmission chain of the disease and decreases considerably the risk of incapacity. We will report two cases of leprosy in advanced stages, incapacitating and transmissible, whose stigma of deformities led to self-isolation, delaying the search for diagnosis(AU).

Lepra lepromatosa peneal en paciente con enfermedad de chagas. Reporte de un caso / Penile lepromatous leprosy in a patient with chagas disease. a case report

Se presenta el caso de un paciente masculino de 52 años con fimosis secundaria a una masa pseudotumoral prepucial. El paciente presentaba una historia de enfermedad de Hansen con afectación de piel, laringe y bronquios. Previa a la circuncisión, el examen físico revelaba, además de las alteraciones lepromatosas, un mega esófago secundario a estenosis en la porción distal. El análisis laboratorial mediante la técnica de ELISA dio positivo para Trypanosoma cruzi, patógeno responsable de la enfermedad de Chagas. Sólo pudimos encontrar un caso previo reportado de lepra lepromatosa con afectación prepucial. La coexistencia de lepra y miocardiopatía chagásica es inusual pero bien conocida por casos reportados en Brasil e India. Sin embargo, de acuerdo a nuestros conocimientos, éste es el primer caso reportado de una asociación entre lepra lepromatosa y mega esófago chagásico en un paciente con fimosis.

We are presenting a 52-year-old patient with phimosis due a tumor like mass, which on pathological evaluation was diagnosed as lepromatous leprosy. The patient had a history of Hansen's disease involving the skin, the larynx and the bronchial tree. Before a circumcision, a physical examination revealed in addition to the lepromatous changes the presence of megaesophagus secondary to stenosis of the distal portion. Laboratory analysis for Tripanosomacruzii using ELISA technique was positive for Chagas Disease. We could find only one previous report of lepromatous leprosy affecting the foreskin. The coexistence of leprosy and myocardial Chagas disease is unusual but well known in reports from Brazil and India. However, this is, to our knowledge, the first case reported of an association of lepromatous leprosy and chagasicmegaesophagus in a patient with phimosis.

Leprosy in a University Hospital in Southern Brazil

AbstractBACKGROUND:Leprosy is an infectious disease that may lead to irreversible nerve damage, compromising patient's quality of life and leading to loss of working years.OBJECTIVES:To evaluate the epidemiological profile of patients followed at a University Hospital.MATERIALS AND METHODS: This is a retrospective observational study, based on a review of medical records. We studied the clinical and epidemiological features of patients with leprosy monitored at the Hospital de Clínicas of the Federal University of Paraná between January 2005 and January 2010.RESULTS:The mean age was 47.51, while 35.94% of patients were aged 41-60. The male:female rate was 1.8:1. The most prevalent occupations were: retired, students or rural workers. Patients came mainly from Curitiba or nearby areas, but there were also patients from the countryside. The mean diagnostic delay was 24.57 months. Multibacillary forms prevailed, with the lepromatous variety being the most common, closely followed by the borderline type. Neural enlargement was found in more than 50% of the patients and 48.44% of them developed reactional states. Hemolysis was the most commonly detected drug side effect. Initial functional evaluation was possible in 70% of patients, 55% of whom had disabilities upon diagnosis. The most prevalent associated disease was hypertension.CONCLUSIONS:This study showed an important diagnostic delay and a high rate of sequelae in this specific population. Brazil is one of the few remaining countries that has not yet eradicated leprosy and it is important to improve health policies in order to prevent sequelae and achieve eradication.

Hanseníase multibacilar em paciente transplantado renal: relato de caso / Multibacillary leprosy in a renal recipient patient: a case report

O Brasil é um país onde a hanseníase ainda é um problema de saúde pública, apresentando mais de 30.000 novos casos por ano nos últimos anos. Apesar do crescente número de transplante de órgãos sólidos realizados no país, sobretudo o transplante renal, não são frequentes os relatos dessa micobacteriose em pacientes imunossuprimidos pelas medicações póstransplante. Os autores relatam um caso de hanseníase multibacilar manifestada 12 anos depois do transplante renal, acompanhado desde o diagnóstico, durante a poliquimioterapia, tratamento e seguimento do eritema nodoso hansênico.

Leprosy is still a public health concern in Brazil, where more than 30,000 new cases are detected every year. There are few reports of this mycobacteriosis in imunossupressed pacients, despite the increasing number of solid organ transplantation and the use of post-transplant drugs in this country. The autors describe a case of multibacillary leprosy in a renal transplant recipient, detected 12 years after the procedure, and discuss the therapy, adverse effects and management of leprosy reactions in pacients imunosupressed by drugs.

Hanseníase virchowiana difusa e o diagnóstico diferencial com outras doenças sistêmicas / Lepromatous leprosy and the differential diagnosis with other systemic diseases

A Hanseníase é uma doença causada pelo Mycobacterium leprae, com manifestações cutâneas acompanhadas de perda da sensibilidade e envolvimento de sistema nervoso periférico, podendo acometer vísceras e mucosas. O Brasil ocupa o 2° lugar no ranking de prevalência da doença. A classificação própria da hanseníase evidencia sua complexidade clínica e polimorfismo; e possibilita intersecções com patologias como o linfoma não Hodgkin. Este é uma neoplasia maligna de linfonodos, e pode manifestar-se primariamente na pele. Paciente masculino, 51 anos. Procurou o serviço com queixa de “pele rachada”, lesões em boca e língua, e emagrecimento. Ao exame físico, presença de placas em palato, formações esbranquiçadas em dorso de língua e infiltração em lóbulo de orelha. Identificaram-se ainda, alterações sensitivas em extremidades, rash cutâneo eritemato-descamativo generalizado, tumoração em cotovelo, e lesões eritemato-infiltradas com exulcerações em membros inferiores. As hipóteses diagnósticas foram: hanseníase, leishmaniose cutâneo-mucosa, SIDA, Sífilis secundária e linfoma não Hodgkin. Durante a investigação, obtiveram-se resultados negativos para todas as sorologias, exceto a pesquisa de BAAR e biópsia sugerindo Hanseníase Virchowiana. Iniciou-se tratamento poliquimioterápico e houve remissão completa das lesões. Na hanseníase virchowiana, notam-se lesões sólidas papulosas, nodulares, ou em placas com características variáveis. Além disso, é possível encontrar espessamento de pavilhão auricular, madarose e obstrução nasal. Lesões em cavidade oral, também são descritas nestes casos. Os linfomas não Hodgkin de apresentação cutânea primária, podem se assemelhar a formas difusas de Hanseníase virchowiana, pois são neoplasias linforreticulares que se manifestam durante a história natural da doença em tecidos extranodais, dentre eles, a pele.

Leprosy is a disease caused by Mycobacterium leprae, with skin manifestations accompanied by loss of sensation and involvement of the peripheral nervous system that can also affect mucous membranes and viscera. Brazil ranks 2nd in the ranking of disease prevalence. The classification of leprosy itself shows its clinical polymorphism; and allows intersections with diseases such as non-Hodgkin lymphoma. This is a malignant neoplasm of lymph nodes, and may manifest primarily inthe skin. A 51 years-old male patient came to us complaining of “chapped skin”, lesions in the mouth and the tongue and weight loss. On physical examination there was the presence of plaques on the palate, whitish formations on back of tongue and ear lobe infiltration. We identified sensory changes, widespread erythematous rash, a tumor in the elbow, and infiltrated erythematous lesions with exulcerations in lower limbs. The diagnostic hypotheses were: mucocutaneous leishmaniosis, AIDS, secondary syphilis, leprosy and non-Hodgkin lymphoma. During the investigation, all serological test were negative, however, acid fast bacilli staining and biopsy suggested lepromatous leprosy. After multidrugtherapy treatment there was complete resolution of the lesions. In lepromatous leprosy solid nodular, papular lesions or plaques with variable characteristics may be noticed. Additionally, thickening of the pinna, madarosis and nasal obstruction can be found. Lesions in the oral cavity are also described in cases of lepromatous leprosy. Non-Hodgkin lymphomas with primary cutaneous presentation, may resemble indeterminate forms of leprosy, since they are characterized as lymphoreticular neoplasms that arise during the natural history of the disease, in extranodal tissues, including the skin.

Hanseníase históide símile: desafio diagnóstico / Histoid like leprosy: diagnostic challenge

Em 1963, Wade descreveu a hanseníase histoide, que acometia pacientes previamente tratados com dapsona.Caracteriza-se por lesão com aspecto queloideano, na histopatologia vemos histiócitos fusiformes e um grande número de bacilos. Relata-se caso de hanseníase dimorfa virchoviana com padrão de lesão históide, um verdadeiro desafio diagnóstico, a fim de atentar para manifestações atípicas dessa doença e reforçar a importância da confirmação histológica de casos suspeitos de hanseníase históide.

In 1963, Wade described Histoid Leprosy, that affected patients previously treated with dapsone. This type of leprosy is characterized by keloid-like lesions, in which spindle histiocytes and a large number of bacilli can be seen in the histopathology. A case of borderline lepromatous leprosy with histoid pattern is described, a true diagnostic challenge, in order to attempt to atypical manifestations of the disease and reinforce the importance of histological confirmation in suspected cases of histoid leprosy.

Lucio's Phenomenon. Case study of an exceptional response to treatment exclusively with multibacillary multidrug therapy / Fenomeno de Lucio. Relato de um caso exuberante com excelente resposta ao tratamento com Poliquimioterapia Multibacilar

Lucio's phenomenon represents a serious cutaneous necrotizing reaction, which can occur with Lucio's leprosy and also in other forms of lepromatous leprosy. The authors discuss the case of a 63-year-old male patient presenting with ulcers and necrotic cutaneous lesions on the limbs, torso and face, associated with recurring sinusitis, hoarseness and a weight loss of 25 kilos over the last year. Bacilloscopy of intradermal scrapings showed a bacilloscopic index of 5 and the histopathological findings were compatible with the diagnosis of Lucio's phenomenon. The chosen treatment was exclusively multibacillary multidrug therapy, resulting in important dermatological improvement after one month of therapy. The authors present an impressive case of Lucio's phenomenon with an exceptional response to treatment exclusively with multibacillary multidrug therapy.

O fenômeno de Lúcio representa uma reação cutânea necrosante grave que pode ocorrer na hanseníase de Lúcio e em outras formas de hanseníase virchowiana. Os autores relatam o caso de um paciente masculino de 63 anos de idade apresentando há um ano lesões ulceronecróticas nos membros, lóbulos auriculares e tronco, associadas a sinusite de repetição, rouquidão e perda ponderal de 25 kg. A baciloscopia de raspado intradérmico mostrou índice baciloscópico de 5 e o exame histopatologico foi compatível com fenômeno de Lúcio. O tratamento instituído foi exclusivamente com poliquimioterapia multibacilar (PQT-MB), havendo melhora importante do quadro cutâneo após um mês de tratamento. Os autores apresentam um caso exuberante de fenômeno de Lúcio que obteve excelente resposta ao tratamento somente com PQT-MB.

Indeterminate leprosy and lepromatous index case: four cases in the same family / Hanseniase indeterminada e caso indice virchowiano: 4 casos na mesma familia

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, which primarily affects the skin and peripheral nerves. Brazil remains as the country with the second largest number of cases in the world. We report the case of three patients diagnosed with indeterminate leprosy in the same family. Two patients were HIV positive. An active search led to the discovery of the index case. It was crucial to persist in the search of the index case. This report shows how important it is to teach physicians and the general population about the signs and symptoms of leprosy. Early diagnosis and treatment are necessary to prevent sequelae and to eliminate the disease as a public health problem.

A hanseníase é uma doença infecciosa crônica causada pelo Mycobacterium leprae, que afeta principalmente a pele e nervos periféricos. O Brasil continua sendo o segundo país do mundo com maior número de casos. Aqui relatamos três pacientes diagnosticados com hanseníase indeterminada, pertencentes à mesma família, cuja busca ativa levou à descoberta do caso índice. Dois pacientes eram HIV positivos. Foi de imensa importância a insistência na procura pelo caso índice. Este relato ilustra a importância do ensino sobre sinais e sintomas de hanseníase na formação médica e à população. O pronto reconhecimento e tratamento são necessários para prevenir sequelas e eliminar a doença como problema de saúde pública.

Descrição de um caso exuberante de eritema nodoso hansênico e análise crítica dos sistemas de classificação para tratamento da hanseníase / Description of a lush case of erythema nodosum and critical analysis of classification systems for the treatment of leprosy

A hanseníase é doença endêmica e de notificação compulsória no Brasil. Há diversos sistemas de classificação para instituição da poliquimioterapia. Descreve-se caso de jovem com diagnóstico e tratamento de hanseníase paucibacilar que, após sete anos, retorna com recidiva das lesões e tratamento para multibacilar, evoluindo com eritema nodoso necrotizante.

Leprosy is an endemic and notifiable disease in Brazil. There are several classification systems for institution of multidrug therapy. We describe the case of a young man with diagnosis and treatment of paucibacillary leprosy who, after seven years, returns with lesion recurrence and treatment for multibacillary evolving necrotizing erythema nodosum.

Lucio's phenomenon: another case reported in Brazil / Fenômeno de Lúcio: mais um caso relatado no Brazil

Lucio's phenomenon is defined as a variant of type 2 leprosy reaction. It is a rare event, occurring in the evolution of leprosy of Lucio and other forms of lepromatous leprosy. It has an exacerbated proliferation of Hansen bacilli in its pathophysiology, which invade blood vessel walls and injure endothelial cells, causing endothelial proliferation and decreasing the vascular lumen. This fact, associated with inflammatory reactions and changes in the coagulation system causes vascular thrombosis, ischemia, infarction and tissue necrosis, leading to the histopathological characteristic of the phenomenon. We report a case of lepromatous leprosy with irregular treatment that developed Lucio's phenomenon. Treatment with multidrug therapy, antibiotics, steroids and thalidomide achieved a favorable outcome.

Define-se o fenômeno de Lúcio como uma variante da reação hansênica do tipo 2. Evento raro, que ocorre na evolução da hanseníase de Lúcio e de outras formas de hanseníase virchowiana. Tem na sua fisiopatologia uma proliferação exacerbada dos bacilos de Hansen, que invadem a parede dos vasos sanguíneos e agridem as células endoteliais, causando proliferação endotelial e diminuição do lúmen vascular, fato este, que associado a reações inflamatórias e a alterações no sistema da coagulação, causa trombose vascular, isquemia, infarto e necrose tecidual, gerando as alterações histopatológicas características do fenômeno. Relatamos um caso de hanseníase virchowiana, com tratamento irregular, que desenvolveu o fenômeno de Lúcio. Recebeu tratamento com poliquimioterapia, antibióticos, corticosteróide e talidomida, evoluindo com desfecho clínico favorável.

La lepra y el testículo / Leprosy and the testis

Introducción. La afección testicular es frecuente en la lepra lepromatosa, daño que se incrementa cuando cursa con eritema nudoso leproso. Objetivo. Presentar un paciente con lepra lepromatosa y eritema nudoso leproso con grave compromiso testicular. Materiales y métodos. Se estudió un hombre de 28 años con lepra lepromatosa desde los 22, que durante la poliquimioterapia para la lepra presentó eritema nudoso leproso crónico que afectó ambos testículos y no respondió al manejo convencional. El dolor persistente obligó a practicar orquidectomía izquierda. Resultados. Este testículo presentaba atrofia tubular y fibrosis notorias, conglomerados de macrófagos espumosos, sin bacilos, hiperplasia focal de células de Leydig, endarteritis y arteritis linfocitaria y granulomatosa de vasos pequeños y medianos; estos cambios también estaban presentes en el epidídimo. Un estudio llevado a cabo dos años después de terminar su tratamiento y de la orquidectomía izquierda, demostró azoospermia, testosterona total normal, testosterona libre discretamente disminuida y hormonas lutropina (luteinizante) y folitropina (estimulante del folículo) elevadas. No había disminución de la libido ni de su actividad sexual. Se revisaron los conceptos generales sobre el eritema nudoso leproso y las alteraciones que la lepra produce en el testículo. Conclusión. La lepra lepromatosa puede conducir a hipogonadismo. Los programas de lepra deben contemplar esta complicación para corregir y evitar sus secuelas.

Introduction. Damage of testicles is frequent in lepromatous leprosy and worsened by the presence of erythema nodosum leprosum. Objective. A patient is presented who developed lepromatous leprosy and erythema nodosum leprosum with major testicular compromise. Material and methods. The 28-year-old male patient had lepromatous leprosy since age 22. During a polychemotherapy treatment for the lepromatous leprosy, he presented chronic erythema nodosum leprosum that affected both testicles; he did not respond to the conventional treatment. A left orchidectomy was performed to treat the persistent pain. Results. The extracted testis evidenced the following: tubular atrophy, extensive fibrosis, cumulus of foamy macrophages without rods, focal Leydig cell hyperplasia, linfocitary and granulomatous arteritis and endarteritis of small and medium size vessels. These changes were also observed in the epididymis. Two years after the polychemoterapy and the orchidectomy, the patient exhibited azoospermy, normal total testosterone, slightly diminished free testosterone and elevated levels of luteinizing hormone and follicle-stimulating hormone. No loss of libido or sexual activity was reported. General concepts of erythema nodosum leprosum were reviewed, as well as the pathologic changes produced by leprosy in the testis. Conclusion. Lepromatous leprosy may lead to hypogonadism. This condition is recommended for inclusion in leprosy diagnostic programs in order to detect and treat the consequences of the possible hypogonadism.

Assessment of quality of life of patients with leprosy reactional states treated in a dermatology reference center / Avaliação da qualidade de vida de pacientes em surto reacional de hanseníase tratados em centro de referência

BACKGROUND: Disabilities and deformities resulting from reactive outbreaks of leprosy can cause many problems for the patients, interfering with their quality of life. OBJECTIVES: To assess the quality of life of patients with leprosy reactional states identified at the Reference Center in Belo Horizonte - MG. METHODS: This was an epidemiological cross-sectional descriptive and analytical study, involving 120 patients in treatment for leprosy reactional states, from December 2007 to March 2008, held at the Dermatology outpatients clinic of the Hospital Eduardo de Menezes from FHEMIG, BH. We used two instruments for the socio-demographic, economic and clinical variables and a generic instrument WHOQOL WHO. The data were tabulated in SPSS and analyzed using the mean score with the application of statistical tests (p <0.05). RESULTS: We found that the median age of the patients was 48 years, most were males, married, from cities around BH, with incomplete elementary school, retired or pensioner, and with a family income of two minimum wages. Most of them reported that the disease interfered a great deal with their professional activities and leisure. In the assessment of QoL, the lowest rating was observed in the physical domain and the highest was observed in the psychological and social relations. The internal consistency of WHO-QOL-bref was acceptable to the facets and domains. CONCLUSIONS: Leprosy causes suffering that goes beyond the pain and discomfort strictly related to the physical damage, with great social and psychological impact.

FUNDAMENTOS: As incapacidades e deformidades decorrentes dos surtos reacionais de hanseníase podem acarretar inúmeros problemas aos pacientes, interferindo na qualidade de vida. OBJETIVOS: Avaliar a qualidade de vida de pacientes portadores de hanseníase em estado reacional identificados em centro de referência de Belo Horizonte, MG. MÉTODOS: Trata-se de um estudo epidemiológico transversal, descritivo e analítico, envolvendo 120 pacientes em tratamento de surto reacional de hanseníase, no período de dezembro de 2007 a março de 2008, realizado no ambulatório de dermatologia do Hospital Eduardo de Menezes da Fhemig. Foram utilizados dois instrumentos referentes às variáveis sociodemográficas, econômicas e clínicas e o instrumento genérico WHOQOL-bref da OMS. Os dados foram tabulados no SPSS e analisados por meio do escore médio com aplicação de testes estatísticos (p < 0,05). RESULTADOS: Constatou-se que a mediana da idade dos pacientes estudados foi de 48 anos, sendo a maioria deles do sexo masculino, casada, de cidades circunvizinhas a BH, com primeiro grau incompleto, aposentada ou gozando de licença saúde e com renda familiar de um a dois salários mínimos vigentes. A maioria deles relatou que a doença interferiu muito nas atividades profissionais e de lazer. Na avaliação da QV, o índice mais baixo foi observado no domínio físico e os mais altos foram observados nos domínios psicológico e das relações sociais. A consistência interna do WHOQOL-bref foi aceitável para as facetas e domínios. CONCLUSÕES: A hanseníase causa sofrimento que ultrapassa a dor e o mal-estar estritamente vinculados ao prejuízo físico, com grande impacto social e psicológico.

Lepra: sepsis en paciente con reacción tipo II. Reporte de un caso / Leprosy: sepsis in patients with type II reaction. A case report

La lepra es una patología infecciosa de carácter crónico, caracterizada por un amplio compromiso cutáneo, asociado a neuropatía, con bajas tasas de mortalidad, pero con un alto índice de discapacidad. Es causada por la infección por el bacilo, Mycobacterium leprae. A pesar que se conoce desde la antigüedad se considera un problema persistente de salud pública en áreas subtropicales donde es endémica. Su mecanismo de transmisión, es por medio de gotitas respiratorias. Su espectro clínico es muy variado, depende de la forma en que el sistema inmunitario del huésped reacciona frente al agente infeccioso, por lo que se reconocen los polos determinados como tuberculoide y lepromatoso, una forma indeterminada y borderline. Por otro lado se considera que las reacciones lepróticas (tipo 1, tipo 2 y fenómeno de lucio) son complicaciones de la hiperreactividad inmunológica que aparece cuando se afecta el equilibrio inmunológico en el huésped. En 1982 la Organización Mundial de la Salud estableció la terapia multidrogas como herramienta eficaz para el control de esta entidad. Actualmente el régimen farmacológico se establece teniendo en cuenta la clasificación que distingue al enfermo en paucibacilar y multibacilar. Presentamos un paciente masculino de 64 años, con antecedente de lepra lepromatosa multibacilar desde 3 años, con tratamiento irregular, suspendido 6 meses antes de clínica de ingreso consistente sepsis de origen urinario, con posterior septicemia asociada al catéter, a quien se le realiza tratamiento antibiótico de amplio espectro, con evolución satisfactoria del estado hemodinámico y manejo de la lepra ambulatorio. [Escalante, E, Trout, G, Oliva J, Mejía F. Lepra: sepsis en paciente con reacción tipo II. Reporte de un caso. MedUNAB 2012; 15:123-129].

Leprosy is an infectious disease of chronic, characterized by extensive skin involvement, neuropathy associated with low mortality rates, but with a high rate of disability. It is caused by infection with the bacillus, Mycobacterium leprae. Although known since ancient times is considered a persistent public health problem in subtropical areas where it is endemic. It is considered that the transmission mechanism is through respiratory droplets. Its clinical spectrum is varied and depends on how the host's immune system reacts against the infectious agent, so the poles are recognized tuberculoid and lepromatous determined as a definite shape and borderline. On the other hand is considered that the leprotics reactions (type 1, type 2 phenomenon pike) are complications of immune hyperreactivity that occurs when immune balance is affected in the host. In 1982 the World Health Organization established multidrug therapy as an effective tool for control of this disease, currently the drug regimen was determined based on the classification that distinguishes the patient in paucibacillary and multibacillary. We report a 64 year old male with a history of multibacillary lepromatous leprosy 3 years, with irregular treatment, suspended 6 months before admission consistent clinical urinary sepsis, with subsequent catheter-associated septicemia, who was with treatment broad-spectrum antibiotic, with satisfactory hemodynamic status and management of leprosy clinic. [Escalante, E, Trout, G, Oliva J, Mejia F. Leprosy: sepsis in patients with type II reaction. A case report. MedUNAB 2012; 15:123-129].

Angiossarcoma primário em couro cabeludo e hanseníase virchowiana: associação incomum em paciente idosa / Primary angiosarcoma of the scalp and lepromatous leprosy: an uncommon combination in an elderly patient

Introdução: Os sarcomas cutâneos primários são neoplasias mesenquimais raras e de grande heterogeneidade histopatológica. O angiossarcoma é um subtipo de sarcoma cutâneo maligno pouco comum, que atinge preferencialmente homens idosos. De localização típicamente cefálica, apresenta-se como lesões multifocais que levam a edema, sangramento e ulceração locais. São agressivos, de crescimento rápido e difícil delimitação de margens cirúrgicas, o que explica altas taxas de recorrência. Descreve-se caso de angiossarcoma primário em couro cabeludo de paciente idosa em uso de poliquimioterapia para hanseníase virchowiana. Associação de angiossarcoma e hanseníase é incomum. A ausência de relatos semelhantes na literatura motivou a descrição do caso.

Tuberculosis pulmonar en un paciente con lepra lepromatosa. Presentación de un caso / Pulmonary tuberculosis in a patient with lepromatous leprosy. Presentation of a case

La tuberculosis y la lepra fueron enfermedades prevalentes durante el primer milenio en Europa, pero ya después, la lepra disminuyó en frecuencia. Por su parte, la tuberculosis se señala en un tercio de la población mundial, reportándose cada año diez millones de casos nuevos, a la vez que mueren tres millones de los existentes. La lepra, aunque se plantea, que está en vías de extinción, aún afecta a un millón de personas de países, para un 1 por ciento de prevalencia. En la actualidad, en muy raras ocasiones coinciden estas patologías. El objetivo de este trabajo fue mostrar el caso de un paciente con una lepra lepromatosa, que poco tiempo después de tratado por esta enfermedad, desarrolló una tuberculosis pulmonar. Para lograr lo propuesto se relacionó el estudio clínico y de laboratorio practicado al enfermo y se mostraron fotografías ilustrativas de ambos procesos. Luego de la revisión de la literatura, se llegó a la conclusión que estas entidades cuando coinciden en un solo sujeto son inusuales, y no han sido reportadas en Cuba.

Tuberculosis and leprosy were prevalent diseases during the first millennium in Europe , but then leprosy decreased in frequency. On the other hand, tuberculosis affects a third of the world population, reporting ten millions of new cases every year, and dying three millions of them. Leprosy, which is considered in extinction, still affects a million of persons, for 1 percent of prevalence. Nowadays, these diseases rarely coincide. The objective of this work was showing the case of a patient with lepromatous leprosy who, shortly after being treated for this disease, developed pulmonary tuberculosis. To achieve our proposal we made a relation of the clinical and laboratory study of the patient and showed illustrative pictures of both processes. After reviewing the literature, we arrived to the conclusion that these entities are unusual when coinciding in one person, and they have not being reported in Cuba .

UVB suscetibilidade como fator de risco para o desenvolvimento da hanseníase virchowiana / UVB susceptibility as a risk factor to the development of lepromatous leprosy

FUNDAMENTOS: A radiação ultravioleta B (RUVB) é o mais importante fator ambiental capaz de modificar a função imunológica da pele humana. OBJETIVO: estudar a associação entre o fenótipo de suscetibilidade ou resistência à radiação RUVB e as formas polares da hanseníase. MATERIAL E MÉTODOS: foram avaliados 38 pacientes com hanseníase virchowiana (MHV) e 87 pacientes com hanseníase tuberculoide (MHT) de acordo com a classificação de Ridley e Jopling (1966). Todos os pacientes foram submetidos ao teste para determinação do fenótipo de suscetibilidade ou resistência à RUVB por meio da aplicação de um disco de dinitroclorobenzeno (DNCB) a 2 por cento em uma área de pele previamente irradiada com duas vezes a dose eritematosa mínima (DEM). Após 21 dias, outra aplicação de um disco similar de DNCB a 0,05 por cento na região escapular (área não exposta à RUVB) foi realizada para avaliar se houve sensibilização, com leitura após 48 horas. Os pacientes que apresentaram reação positiva ao DNCB foram considerados UVB-resistentes e o oposto foi considerado para aqueles que não apresentaram resposta (UVB-suscetíveis). RESULTADOS: A frequência de UVB-suscetíveis foi de 63,2 por cento (24 pacientes) no grupo MHV e 34,4 por cento (30 pacientes) no grupo MHT (OR = 3,26; IC = 1,36-7,87; x² = 7,73; p = 0,005). CONCLUSÃO: Os resultados sugerem que a UVB-suscetibilidade é um fator de risco para o desenvolvimento da MHV.

BACKGROUNDS: Ultraviolet radiation B (UVRB) is the most important environmental factor capable of altering the immune function of human skin. OBJECTIVE: To evaluate the association of the phenotypes of susceptibility or resistance to ultraviolet radiation B (UVRB) and the polar forms of leprosy. MATERIAL AND METHODS: We evaluated 38 patients with lepromatous leprosy (LL) and 87 patients with tuberculoid (TT) leprosy, according to the classification by Ridley and Jopling (1966). All the patients were submitted to a test to determine the phenotypes of susceptibility or resistance to UVRB through the application of a 2 percent dinitrochlorobenzene (DNCB) disc to a previously irradiated area with twice the minimal erythema dose (MED). After 21 days, a similar disc soaked in 0.05 percent DNCB was applied to the scapular area (unexposed to UVRB) to check for sensitiveness, with reading of the results after 48 hours. The patients that showed a positive reaction to DNCB were considered resistant (UVB-R) and those who did not show any reaction were considered susceptible (UVB-S). RESULTS: The frequency of UVB-S individuals was 63.2 percent (24 patients) in the LL group and 34.4 percent (30 patients) in the TT group (OR=3.26; IC=1.36 - 7.87; x²=7.73; p=0.005). CONCLUSION: Our results suggest that UVB-susceptibility is a risk factor to the development of lepromatous leprosy (LL).

Blefarite e tricomegalia induzidas pelo cetuximabe / Blepharitis and trichomegaly induced by cetuximab

Nós descrevemos uma mulher de 41 anos em tratamento de câncer colorretal avançado que, após a segunda dose de cetuximabe, desenvolveu intensa blefarite e tricomegalia bilateral. A toxicidade ocular decorrente do cetuximabe tem sido relatada, porém ainda tem mecanismos fisiopatogênicos incertos.

This report describes the case of a 41-year old woman in treatment for advanced colorectal cancer who developed severe bilateral blepharitis and trichomegaly after the second dose of cetuximab. Cetuximab-related eyelid toxicity has been described previously; however, its pathogenesis has not yet been clearly established.