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.

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.

Brazilian clinical trial of uniform multidrug therapy for leprosy patients: the correlation between clinical disease types and adverse effects

This study sought to verify the correlation between leprosy types and the adverse effects of treatment drugs. This quantitative, prospective, nested study was developed at the Dona Libânia Dermatology Centre in Fortaleza, Brazil. Data were collected from November 2007-November 2008. During this period, 818 leprosy patients were diagnosed and began treatment. Forty patients with tuberculoid leprosy (TT) were selected. Twenty patients followed a standard therapy of dapsone and rifampicin and 20 were administered dapsone, rifampicin and clofazimine (U-MDT). Twenty patients with borderline lepromatous (BL) and lepromatous leprosy (LL) were also selected and treated with U-MDT. All of the subjects received six doses. With the exception of haemolytic anaemia, there was a low incidence of adverse effects in all the groups. We did not observe any differences in the incidence of haemolytic anaemia or other side effects across groups of patients with TT, BL or LL treated with U-MDT.

Avaliação de hansenianos tratados com esquema alternativo dose única ROM (rifampicina, ofloxacina e minociclina), após sete a nove anos / Evaluation years in leprosy patients treated with single dose alternative scheme ROM (rifampin, ofloxacin, minocycline), after seven to nine

INTRODUÇÃO: Em 1997, após a realização de estudo multicêntrico, duplo cego e randomizado, em nove centros de tratamento de hanseníase na Índia, o Ministério da Saúde adotou o esquema alternativo dose única ROM para casos de lesão única, paucibacilar, sem nervo periférico afetado, índice baciloscópico negativo, em Centros de Referência da doença no Brasil. O estudo se propôs a avaliar a efetividade do esquema ROM em pacientes tratados no período de 1997 a 1999 no Serviço de Dermatologia da Santa Casa de Vitória. MÉTODOS: Foram selecionados e tratados com o esquema ROM, 54 pacientes das formas indeterminada e tuberculóide. Estes pacientes foram convocados de março a outubro de 2006 para reavaliação clínica. RESULTADOS: Vinte e nove pacientes avaliados (85,2 por cento; IC95 por cento: 70-100,4) estavam curados, cinco (14,7 por cento; IC95 por cento: 7,4-22,0) recidivaram e 20 pacientes não retornaram; porém, não havia outra notificação de reingresso ao tratamento no banco de dados da Secretaria Estadual de Saúde. CONCLUSÕES: O estudo evidenciou taxa de cura de 90,8 por cento e taxa bruta de recidiva de 9,2 por cento, após período de sete a nove anos da dose ROM. O tratamento alternativo ROM demonstrou melhor efetividade para lesão única menor que quatro centímetros e aparecimento há menos de cinco anos.

INTRODUCTION: In 1997, after obtaining a combined multi-state double-blind randomly controlled clinical trial study from nine Indian centers involved in the treatment of Hansen's Disease, the Ministry of Health adapted the single dose ROM Therapy approach in those cases involving the treatment of a single skin lesion, paucibacillary leprosy without evidence of peripheral nerve trunk involvement and indication of negative baciloscope, in the Referral Centers in Brazil. The study aimed to evaluate the effectiveness of the single dose ROM Therapy approach in those patients who were treated from the period of 1997 to 1999 in the Ambulatory Dermatologic Unit in the Hospital in Vitória, ES. METHODS: Fifty-four patients with tuberculoid and indeterminate leprosy were selected and treated with the single dose ROM Therapy approach. These patients were contacted from March 2006 up and until October 2006 for further clinical reevaluation. RESULTS: From the studies conducted, the following results were found to exist: 29 patients (85,2 percent; 95 percentCI: 70-100,4) were cured, 5 patients (14,7 percent; 95 percentCI: 7,4-22,0) relapsed, and 20 patients didn't return; however, there are no additional records of any notification of other treatment(s) in the State Department of Health's informational data banks. CONCLUSIONS: The study demonstrated a rate of cure of 90.8 percent and a rate of relapse of 9.2 percent after a period of seven to nine years using the single dose ROM Therapy approach. Additionally, this alternative treatment further demonstrated a better effectiveness for a single skin lesion smaller than four centimeters and with an appearance in less than five years.

Hanseníase tuberculóide apresentando-se como lesão em raquete de tênis: relato de um caso típico / Tuberculoid leprosy presenting as a racket lesion: report of a typical case

A hanseníase tuberculóide é caracterizada, em geral, por lesões únicas ou em pequeno número, sendo o comprometimento neural intenso e localizado. A lesão "em raquete de tênis" caracteriza de forma particular esse comprometimento, e se apresenta sob a forma de um ramo nervoso sensitivo cutâneo emergindo de uma placa tuberculóide. Relata-se o caso de paciente masculino, 61 anos, com diagnóstico de hanseníase tuberculóide, cuja lesão permaneceu ativa mesmo após o término de poliquimioterapia paucibacilar (PQT-PB). Ao exame dermatológico, foi observado espessamento do ramo superficial do nervo ulnar, emergindo da placa tuberculóide típica em dorso da mão direita, caracterizando a "lesão em raquete de tênis". A sorologia para anti-PGL-1 foi negativa, o exame histopatológico da lesão demonstrou hanseníase de padrão tuberculóide com baciloscopia negativa, e a reação de Mitsuda foi fortemente positiva, confirmando o diagnóstico de hanseníase tuberculóide ativa. Foi reiniciado o tratamento com PQT-PB, além de emprego de neurolépticos, com discreta regressão da lesão. Neste relato, ao ser apresentado um caso típico de lesão "em raquete de tênis", evidencia-se a evolução clássica dos casos de hanseníase em pacientes Mitsuda positivos, onde a PQT geralmente não modifica a história natural da doença.

Tuberculoid leprosy is characterized, usually, by single or few lesions which show intense and localized neural impairement. The "racket lesion", which is described as pathognomonic of tuberculoid leprosy, presents with a cutaneous nerve branch emerging from a tuberculoid plaque. It is reported a case of a 61 years old male, with diagnosis of tuberculoid leprosy, whose lesion remained active even after the end of PB multidrugtherapy (PB-MDT). At dermatologic examination, enlargement of the sensitive cutaneous branch of the ulnar nerve was observed, which emerged from a tuberculoid plaque on the dorsum of the right hand, characterizing a "racket lesion". Serology for anti-PGL-1 was negative, and the Mitsuda reaction was highly positive, confirming the diagnosis of tuberculoid leprosy. MDT-PB was reinitiated, with little improvement. In this report, we describe the typical evolution of leprosy in individuals with positive Mitsuda reaction, where the MDT does not change the natural history of the disease.

Pure neural leprosy: steroids prevent neuropathy progression

Multidrug therapy (MDT), with rifampicin, dapsone, and clofazimine, treats leprosy infection but is insufficient in arresting or preventing the nerve damage that causes impairments and disabilities. This case-series study evaluates the benefits of the combined use of steroids and MDT in preventing nerve damage in patients with pure neural leprosy (PNL). In addition to MDT, 24 patients (88 percent male aged 20-79 years, median=41) received a daily morning dose of 60 mg prednisone (PDN) that was gradually reduced by 10 mg during each of the following 5 months. PNL was clinically diagnosed and confirmed by nerve histopathology or PCR. A low prevalence (8.3 percent) of reaction was observed after release from treatment. However, most of the clinical parameters showed significant improvement; and a reduction of nerve conduction block was observed in 42 percent of the patients. The administration of full-dose PDN improved the clinical and electrophysiological condition of the PNL patients, contributing to the prevention of further neurological damage.

A poliquimioterapia (PQT), com rifampicina, dapsona, e clofazimina, trata a infecção na hanseníase, mas é insuficiente para interromper ou prevenir o comprometimento neurológico que causa as incapacidades e desabilidades, nesta enfermidade. Este estudo de série de casos avalia o benefício do uso combinado de prednisona e PQT na prevenção do dano neurológico em pacientes com a forma neural pura da hanseníase (FNP). Além do PQT, 24 pacientes (88 por cento homens, com idade variando entre 20-79, mediana=41) receberam uma dose diária de 60 mg prednisona que foi reduzida gradualmente na dose de 10 mg durante cada um dos 5 meses subseqüentes. FNP foi diagnosticada clinicamente e confirmada através do estudo histopatológico ou PCR. Baixa prevalência de reação (8,3 por cento) foi observada apenas após o final do tratamento. A maioria dos parâmetros clínicos mostrou melhora significativa e redução do bloqueio de condução foi observada em 42 por cento dos pacientes. A administração de doses altas de prednisona melhora a evolução clínica e eletrofisiológica de pacientes com a FNP de hanseníase, contribuindo na prevenção de novos comprometimentos neurológicos.

Leprosy in HIV infection: a study of three cases.

The course of leprosy in patients with HIV infection has been a controversial issue for a long time. It is still a matter of debate whether the HIV status of an individual has any impact on the natural history of leprosy and response to anti-leprosy treatment. We report here three HIV-positive leprosy cases (two BT and one BB) along with their CD4 counts and HIV staging with anti-leprosy therapeutic response. Both BT cases responded well to conventional WHO MDT (PB) for 6 months, whereas the BB case relapsed 3 months after completion of MDT (MB) for one year. However, he became inactive again following a further one-year course of MDT (MB).

Reactions in borderline leprosy.

This is a retrospective study of 276 patients consisting of 157 active and 119 reactive patients of borderline leprosy. They were followed up for 10 years after sulphone monotherapy. The presenting symptoms were carefully examined from the records and systematically presented. Frequency of reactions was least in BT cases and most in BL cases. Risk factors of reaction appear to be the type of leprosy, multiplicity of lesions, high BI and, possibly, psychological stress. Biopsy of skin lesions was performed in all cases initially, and at the subsidence of the disease. Histological findings closely correlated with clinical classification. While all the cases showed clinical subsidence, histological subsidence was found in 200 (73%) cases, and the condition was static in 36 cases (13%). Immunological upgrading was seen in 110%, while 4% showed downgrading. Bacteriological status and lepromin reaction of active and reactive cases were compared. All these factors need to be taken into consideration for instituting prompt and proper treatment.

Chemotherapy trial in paucibacillary leprosy using clofazimine.

In a double blind study, 300 PB patients (smear negative, indeterminate, tuberculoid and borderline tuberculoid) were randomly allotted to two regimens, the control subjects (150 patients) receiving the standard WHO multidrug regimen of six doses of once a month rifampicin with daily dapsone therapy for six months, while the study group (150 patients) receiving 50 mg of clofazimine daily for six months in addition to the WHO regimen. After stoppage of therapy all the patients were followed up on placebo. The regimens were well tolerated. In 7.5% of patients on clofazimine containing regimen, the lesions showed persisting activity at the time of stoppage of therapy, compared with 16% on the control regimen. This activity subsided spontaneously, more rapidly, in the study group (80% compared with 30% in the control group) in six months. Two patients in the control group and one patient in the study group developed late reaction. There were no relapses in the study group, whereas, two patients have relapsed in the control group during a follow-up of 2.5 to 3.5 years.

A sequential study of circulating immune complexes, complement and immunoglobulins in borderline tuberculoid leprosy patients with and without reactions.

Sequential estimates of the levels of circulating immune complexes (CIC), complement catabolic fragment C3d, complement-mediated immune complex solubilization (CMS) and immunoglobulins were made in 24 newly diagnosed with borderline tuberculoid leprosy over a 20 month period after initiation of chemotherapy. Fourteen of these patients had not suffered from reversal reactions either at the time of presentation or during the follow-up. The levels of CIC were evaluated in them from the third to the eleventh month after starting chemotherapy and immunoglobulin G (IgG) levels were evaluated up to eight months. The concentrations of C3d and immunoglobulins A (IgA) and M (IgM) were normal in these patients. The other ten patients had reversal reaction at the time of diagnosis which subsided by the third month after starting treatment. They did not have reversal reactions later. The levels of CIC and IgG were elevated and those of CMS were depressed throughout the study period. Serum C3d level was initially elevated but came down to normal by the third month while IgA and IgM levels were within normal limits. The relevance of these findings to the genesis of reversal reaction is discussed in this communication.

Neurites na hanseníase: significado de parâmetros clínicos e epidemiológicos na indução e agravamento das incapacidades físicas em pacientes multibacilares / ?

Com o objetivo de determinar a influência dos episódios de neurite franca no estabelecimento e/ou agravamento de incapacidades físicas, foram estudados 103 pacientes portadores de formas multibacilares de hanseníase (18,4 por cento pacientes BB; 47,6 por cento pacientes BL; e 34 por cento LL), durante o período médio de 64,6 meses a partir do início da poliquimioterapia para multibacilares, acompanhadas no Ambulatório Souza Araújo da Fundação Oswaldo Cruz, Rio de Janeiro. Os pacientes foram avaliados por ocasião do diagnóstico, mensalmente durante a poliquimioterapia e anualmente após o tratamento medicamentoso, ou a intervalos menores quando da vigência de episódios reacionais. A avaliação de incapacidades foi feita pelo grau e índice de incapacidades, utilizando o teste dos músculos voluntários (VMT) e o teste de sensibilidade com monofilamentos de nylon (Semmes-Weinstein). Observou-se uma correlação estatisticamente significativa entre maiores tempo de evolução da enfermidade previamente ao diagnóstico e a presença de incapacidades físicas no exame inical, conforme avaliada pelo grau de incapacidade antes do tratamento (p < 0,05). A presença de nervos periféricos afetados (espessados e/ou dolorosos) no momento do diagnóstico correlacionou-se estatisticamente com incapacidades físicas presentes ao exame inicial (grau de incapacidade antes do tratamento maior que zero - p < 0,005), bem como com a ocorrência de episódios de neurite franca durante o período total de seguimento (p < 0,05). Quarenta e seis pacientes (44,7 por cento) tiveram episódios de neurite franca durante o seguimento (34 por cento durante a poliquimioterapia). Houve uma correlação estatisticamente siginificativa entre a ocorrência de episódios de neurite franca e o estabelecimento de incapacidades físicas ao término de tratamento, conforme avaliadas pelo grau de incapacidade (p = 0,000274) e pelo índice de incapacidades (p = 0,002165), mesmo entre aqueles pacientes que iniciaram o tratamento com grau de incapacidade zero (com relação ao grau de incapacidade no término do tratamento, p = 0,013246). De modo semelhante, observamos uma correlação significativa entre a ocorrência de episódios de neurite franca e a presença de incapacidades ao término do episódio de seguimento, seja avaliada quanto ao grau de incapacidade (p = 0,006886), ou quanto ao índice de incapacidade (p = 0,006274), mesmo naqueles pacientes que não apresentavam incapacidades no início do tratamento.

Agranulocitose induzida pelo uso de dapsona em paciente com hanseníase tuberculóide / Agranulocytosis induced by use of dapsone in pacient with tuberculoid leprosy

Relato de caso de paciente do sexo feminino, sob tratamento para hanseníase tuberculóide, em uso de 100mg/dia de dapsona, que desenvolveu agranulocitose e septicemia. Discutem-se os mecanismos envolvidos, as causas possíveis e o quadro clínico. Sugere-se cautela durante o uso dessa droga, principalmente nas primeiras 12 semanas de tratamento.