Erythroderma as a manifestation of leprosy

Abstract: Erythroderma consists of erythema and scaling involving most or all of the body surface. This generalized eruption may be idiopathic, drug-induced or secondary to cutaneous or systemic disease. A 71-year-old man is reported presenting generalized erythema and desquamation with deck-chair sign, nail dystrophy, and plantar ulcers associated with loss of local tactile sensitivity. Biopsies from three different sites demonstrated diffuse lymphocytic infiltrate with incipient granulomas. Fite-Faraco staining showed numerous isolated bacilli and globi. The skin smear was positive. Clinical and pathological diagnosis of borderline lepromatous leprosy was confirmed. This report demonstrates that chronic multibacillary leprosy can manifest as erythroderma and thus should be included in the differential diagnosis.

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.

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.

Neural relapse in multibacillary leprosy 6 years after end of treatment / Recidiva neural em hanseníase multibacilar após 6 anos de tratamento

This article presents a case of relapse, with isolated neural manifestation, in a multibacillary patient previously treated with multidrug therapy for multibacillary leprosy (24 doses). The patient returned to the service six years after the end of treatment, with pain in hands and legs. He was investigated, and the serological monitoring showed an important increase in anti-phenolic glycolipid serum levels. A neural recurrence was suspected, since the patient had no new skin lesions. A new biopsy in the right ulnar nerve showed a bacilloscopy of 2 +, compatible with relapse. This is a literature review of the etiological, clinical, propedeutical and diagnostic aspects of this situation so poorly understood.

O presente artigo relata um caso de recidiva, com manifestação neural isolada, em paciente multibacilar previamente tratado com poliquimioterapia para multibacilar 24 doses. O paciente retorna ao serviço, seis anos depois do fim do tratamento, com dores em mãos e pernas. Na investigação, o acompanhamento da sorologia anti-glicolipídeo fenólico 1 demonstrou aumento importante dos níveis séricos, e foi aventada a hipótese de recidiva neural, já que o paciente não apresentava lesões cutâneas novas. Uma nova biópsia, em nervo ulnar direito, demonstrou baciloscopia de 2+, compatível com recidiva. Faz-se revisão da literatura sobre aspectos etiológicos, clínicos, propedêuticos e diagnósticos dessa situação tão pouco compreendida.

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.

Leprosy and multiple endocrine neoplasia type I: a case report and review of literature / Hanseníase e neoplasia endócrina múltipla tipo 1: relato de caso e revisão de literatura

Context: Leprosy is an infectious disease in which progress to disease and clinical expressions are influenced by environmental factors and by the genetics of the host. The multiple endocrine neoplasia type 1 syndrome (MEN-1) is an inherited disorder that predisposes to endocrine and non-endocrine tumors. Case report: A case of multi-bacillary leprosy in the 18-year-old female patient with MEN- 1, this association was probably an incidental case of an endemic disease in a susceptible individual with a rare syndrome. The investigation of comorbities, especially neglected or emergency disease, is very importance, because the lack of early diagnosis may result in serious complications as development of disabilities especially when there is a leprosy late diagnosis.

Contexto: A hanseníase é uma doença infecciosa cuja evolução e expressões clínicas são influenciadas por fatores ambientais e pela genética do hospedeiro. A síndrome neoplasia endócrina múltipla tipo 1 (NEM-1)é uma desordem hereditária que predispõe a tumores endócrinos e não-endócrinos . Relato de caso: Relata-se um caso de hanseníase multibacilar em paciente de 18 anos de idade com NEM- 1. Esta associação foi, provavelmente, um caso incidental de uma doença endêmica em um indivíduo suscetível com uma síndrome rara. A pesquisa de comorbidades, especialmente entre as doenças negligenciadas ou emergentes é muito importante, porque a falta de diagnóstico precoce pode resultar em complicações graves como o desenvolvimento de deficiências especialmente quando há um diagnóstico de hanseníase tardio.

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.

Lacaziose simulando hanseníase dimorfa tuberculóide / Lacaziosis mimicking borderline tuberculoid leprosy

Lacaziose é infecção crônica causada pelo fungo Lacazia loboi, originalmente descrita por Jorge Lobo (1931) em paciente oriundo da Amazônia brasileira. Manifesta-se, sobretudo, por lesões cutâneas queloidiformes. Relata-se o caso de paciente com placa eritêmato-violácea, cuja hipótese diagnóstica inicial foi de hanseníase dimorfa tuberculóide, doença também endêmica na região amazônica. Ressalta-se a importância da inclusão de formas paucibacilares de hanseníase no diagnóstico diferencial da lacaziose.

Lacaziosis is a chronic skin infectious disease caused by the fungus Lacazia loboi, which usually results in indolent cutaneous keloid-like lesions. It was first described by Jorge Lobo, in 1931, in a patient from the Amazon region. We report a case of lacaziosis, manifested by an erythematous infiltrated plaque, causing misdiagnosis with paucibacillary leprosy, since both diseases are endemic in Amazon basin.

Reação reversa atípica em paciente com hanseníase dimorfa co-infectado pelo HIV / Atypical reversal reaction in a borderline leprosy patient co-infected with HIV

A infecção pelo HIV não altera a história natural da hanseníase. Observa-se maior incidência de estados reacionais nos pacientes co-infectados, além de casos mais graves de neurite. Paciente soropositiva com hanseníase dimorfa tuberculóide manifestou reação reversa exuberante. Lesões cutâneas atípicas e raras surgiram após a introdução da terapia anti-retroviral que promoveu o início da recuperação imunológica com aumento de linfócitos T CD4+ e queda da carga viral. A restauração da imunidade celular nos pacientes soropositivos pode precipitar reações reversas, descritas recentemente como uma das manifestações da síndrome inflamatória de reconstituição imunológica associada à hanseníase.

HIV infection does not modify the natural course of leprosy. However, HIV co-infection seems to be associated with an increased rate of reactional states and more severe cases of neuritis. The authors describe a case of borderline tuberculoid leprosy in a HIV-positive patient who developed a marked reversal reaction. Atypical and rare skin manifestations, such as verrucous lesions and ulcers, appeared after highly active antiretroviral therapy, which resulted in increased CD4+ T-lymphocyte count and drop in viral load. The restoration of the cellular immunity in HIV-seropositive patients can trigger reversal reactions that are one of the manifestations of the immune reconstitution inflammatory syndrome. Only recently the syndrome has been associated with leprosy.

Manifestações de padrão tuberculóide reacional na hanseníase dimorfa: estudo histoquímico e imuno-histoquímico comparativo, em biópsias cutâneas, entre reações tipo 1 ocorridas antes e durante a poliquimioterapia / Manifestations of reactional tuberculoid pattern in borderline leprosy: comparative, histochemical and immunohistochemical study, in skin biopsies, between type 1 reactions ocurred before and during multidrugtherapy

FUNDAMENTOS: Na hanseníase dimorfa é comum a ocorrência de reações tipo 1 antes, durante ou depois da poliquimioterapia (PQT). Trabalhos recentes sugerem que a reação tipo 1 seria um desequilíbrio imunológico entre citocinas pró-inflamatórias e antiinflamatórias. OBJETIVOS: Compreender melhor a fisiopatologia das reações tipo 1. MÉTODOS: Estudaram-se biópsias cutâneas de 10 indivíduos com hanseníase dimorfa-tuberculóide reacional não tratada (DTR) e 10 dimorfos em reação reversa após o início da PQT (DRR), comparando-se os parâmetros morfológicos e imunológicos por meio de colorações HE e Faraco-Fite, e técnicas imuno-histoquímicas (CD4, CD8, CD20, CD79a, CD57, iNOS, IL-10, LAM e BCG). RESULTADOS: Houve, nos DRR, mais macrófagos multivacuolados, maior marcação nos macrófagos para a enzima óxido nítrico sintase induzível (iNOS) e menos linfócitos T CD8+ (p<0,05). Afora a presença de bacilos típicos nos DTR e sua ausência nos DRR, não houve diferenças na baciloscopia ou na marcação para antígenos micobacterianos (LAM e BCG) entre os grupos. O número de células IL-10+ foi similar nos dois grupos, porém houve correlação negativa entre essa citocina e a proporção CD4/CD8 apenas nos pacientes DRR (p<0,05). Houve tendência à redução do infiltrado específico e ao maior número de células NK nos DRR. CONCLUSÃO: Na presença de muitos bacilos viáveis em um paciente sem imunidade celular plena, haveria tendência à piora imunológica (downgrading). A PQT, ao reduzir a carga bacilar, melhoraria a imunidade celular (upgrading), com posterior desvio da imunidade adquirida para a inespecífica (resposta Th3), evoluindo para a cura.

BACKGROUND: Type 1 reactions are common in borderline leprosy, and can occur before, during or after multidrugtherapy (MDT). Recent papers suggest that these reactions could be a result of an imbalance between proinflammatory and anti-inflammatory citokines. OBJECTIVE: To understand better the physiopathology of type 1 reactions. METHODS: We studied skin biopsies from 10 non-treated patients with reactional borderline tuberculoid leprosy (BTR) and 10 from borderline leprosy with reversal reactions after the beginning of MDT (BRR), to compare morphological and immunological parameters by routine staining (H-E and Faraco-Fite) and immunohistochemical technics (CD4, CD8, CD20, CD79a, CD57, iNOS, IL-10, LAM and BCG). RESULTS: We found, in BRR group, stronger staining for iNOS into macrofages, fewer CD8+ T cells and more multivacuolated macrofages than BTR group (p<0,05). Despite the presence of viable bacilli in BTR and its absence in BRR, there weren't differences in baciloscopy and staining for mycobacterial antigens (LAM and BCG) between the groups. The number of IL-10+ cells was similar in both groups, but there was a negative correlation between this cytokine and the CD4:CD8 ratio only in BRR group (p<0,05). It was seen a tendency for a decreased specific infiltrate and increased number of NK cells in BRR group. CONCLUSIONS: The presence of many viable bacilli in a patient with partial cellular immunity could worse the immunological status (downgrading). Once started MDT, the reduction bacilli charge would improve cellular immunity (upgrading), with latter shift to innate immunity (Th3 response), evolving to cure.

Thyroid functional status in leprosy patients in Sudan

We studied 45 adult patients with untreated lepromatous leprosy and borderline leprosy, presenting at clinics in Khartoum and Omdurman, to assess clinical and biochemical effects of the disease on thyroid function. A matching control group of 30 subjects, without symptoms or signs of thyroid disease, were included for comparison. Thyroxine, triiodothyronine and thyrotrophin levels were within normal range. Mean serum thyroxine was low in both groups [significant in lepromatous leprosy patients only]. Mean serum triiodothyronine was high in both groups [significant in neither group]. Mean thyrotrophin was significantly higher in both groups compared with controls

BORDERLINE TUBERCULOID LEPROSY: CLINICOPATHOLOGICAL EVALUATION OF MULTIDRUG THERAPY.

Efficacy of multi-drug therapy as per WHO recommendation in 125 fresh cases of borderline tuberculoid (BT) leprosy was evaluated. There were 116 (92.8%) male and 9 (7.2%) female patients. Age of the patients ranged from 18-50 years but the majority (80.8%) were young adults in the age group 21-35 years. The commonest site of lesion was the upper extremity in 65 (52%) cases. Skin smear for acid fast Mycobacterium leprae was positive in 11 (8.8%) patients. All patients were given multidrug therapy consisting of rifampicin 600 mg once a month and dapsone 100 mg daily for 6 months. At the end of 6 months, 42 (33.6%) patients had shown marked improvement, 14 (11.2%) had increase in activity, 51 (45.6%) had shown regression and 12 (9.6%) cases became clinically inactive. Histologically complete clearance of the infiltration was not observed in any patient. Compact granulomas persisted in 30 (24%) cases. In 1 (0.8%) patient M. leprae were found in the skin smear at the end of 6 months. This study indicates that treatment with MDT for 6 months is inadequate to treat all types of BT leprosy cases.