[Hansen's disease. Case report of tuberculoid leprosy in Chile]. / Enfermedad de Hansen. Comunicación de un caso de lepra tuberculoide en Chile.

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.

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.

Follow-up assessment of patients with Pure Neural Leprosy in a reference center in Rio de Janeiro-Brazil.

INTRODUCTION: Pure Neural Leprosy (PNL) is a rare clinical form of leprosy in which patients do not present with the classical skin lesions but have a high burden of the disability associated with the disease. Clinical characteristics and follow up of patients in PNL are still poorly described in the literature. OBJECTIVE: This paper aims to describe the clinical, electrophysiological and histopathological characteristics of PNL patients, as well as their evolution after multidrug therapy (MDT). METHODS: Fifty-two PNL patients were selected. Clinical, nerve conduction studies (NCS), histopathological and anti-PGL-1serology were evaluated. Patients were also assessed monthly during the MDT. At the end of the MDT, all of the patients had a new neurological examination and 44 were submitted to another NCS. RESULTS: Paresthesia was the complaint most frequently reported by patients, and in the neurological examination the most common pattern observed was impairment in sensory and motor examination and a mononeuropathy multiplex. Painful nerve enlargement, a classical symptom of leprosy neuropathy, was observed in a minority of patients and in the motor NCS axonal injuries, alone or in combination with demyelinating features, were the most commonly observed. 88% of the patients did not present any leprosy reaction during MDT. There was no statistically significant difference between the neurological examinations, nor the NCS pattern, performed before and after the MDT. DISCUSSION: The classical hallmarks of leprosy neuropathy are not always present in PNL making the diagnosis even more challenging. Nerve biopsy is an important tool for PNL diagnosis as it may guide therapeutic decisions. This paper highlights unique characteristics of PNL in the spectrum of leprosy in an attempt to facilitate the diagnosis and management of these patients.

Leprosy with ulnar nerve abscess: ultrasound findings in a child.

We report the ultrasound findings of a typical case of nerve abscess due to leprosy in an 11-year-old boy. The patient had previously undergone pediatric multibacillary leprosy multidrug therapy (MDT) in accordance with World Health Organization guidelines. He presented to our service with bilateral ulnar neuritis with no response to prednisone (1 mg/kg). Right ulnar nerve ultrasound revealed nerve hypoechogenicity, fascicular pattern disorganization, marked fusiform thickening, and a round anechoic area suggestive of intraneural abscess. Intense intraneural power Doppler signal was detected, indicating active neuritis. Intravenous methylprednisolone had a poor response and the patient was submitted to ulnar nerve decompression, which confirmed nerve abscess with purulent discharge during surgery. As the patient weighed more than 40 kg, treatment with a pediatric dose was considered insufficient and adult-dose MDT was prescribed, with improvement of nerve pain and function. Although leprosy is rare in developed countries, it still exists in the USA and it is endemic in many developing countries. Leprosy neuropathy is responsible for the most serious complications of the disease, which can lead to irreversible impairments and deformities. Nerve abscess is an uncommon complication of leprosy and ultrasound can efficiently demonstrate this condition, allowing for prompt treatment. There is scant literature about the imaging findings of nerve abscess in leprosy patients. Radiologists should suspect leprosy in patients with no other known causes of neuropathy when detecting asymmetric nerve enlargement and nerve abscess on ultrasound.

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.

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.

Clinical presentation of tuberculoid leprosy in an epidermodysplasia verruciformis patient.

Epidermodysplasia verruciformis (EV) is triggered by a variety of mechanisms that at least partly include genetic background. We present a Brazilian man with a 30-year history of flat, wart-like lesions with clinical, histopathological, and evolutive aspects consistent with papillomavirus (HPV)-associated EV. Histological analysis of the wart lesions showed epidermis with hyperkeratosis, regular acanthosis, hypergranulosis, and cells with abundant basophilic cytoplasm. Moreover, a perivascular lymphocytic infiltrate was found in the superficial dermis, consistent with a viral wart. Type-2-HPV DNA was detected in various fragments of skin-wart lesions using the polymerase chain reaction (PCR). Two years after the EV diagnosis, the patient presented with an anesthetic well-demarcated, erythematous and mildly scaly plaque on his right forearm. A histopathological analysis of this lesion demonstrated the presence of a compact tuberculoid granuloma. Ziehl-Neelsen staining demonstrated the presence of rare acid-fast bacilli and confirmed the tuberculoid leprosy diagnosis. The patient's Mitsuda Intradermal Reaction was positive. To elucidate the possible mechanism involved in this case of EV, we genotyped the HLA genes of this patient. DQB genotyping showed the polymorphic HLA alleles DQB1*0301 and 0501. The patient was treated with a paucibacillary multi-drug therapy scheme, and the disease was cured in six months. This report describes an EV patient with an M. leprae infection, confirming that tuberculoid leprosy patients possess a relatively specific and efficient cell-mediated immunity against the bacillus and, therefore, localized forms of the disease. Moreover, we show the possible involvement of the polymorphic HLA alleles DQB1*0301 and 0501 in EV induction mechanisms.

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.

Oral lesion in leprosy: borderline tuberculoid diagnosis based on detection of Mycobacterium leprae DNA by qPCR.

Oral lesions are rarely reported in paucibacillary forms of leprosy. We report here a case with an erythematous hyposensitive lesion in the palate and no skin lesions. In addition to routine tests, biopsies of the lesion in the palate and of clinically normal surrounding areas were performed and subjected to real-time PCR for detection of Mycobacterium leprae DNA. The biopsy of the oral lesion was positive for bacilli DNA, followed by positive serum anti-PGL-1 and Mitsuda test, but with negative histopathology. The patient was diagnosed with a borderline tuberculoid form. After multidrug therapy the lesion had significantly regressed and the bacilli DNA detection in the former lesion was negative. The bacilli DNA detection in an oral lesion by real-time PCR not only improved leprosy diagnosis, but also helped in the classification of clinical form, and in the establishment of the appropriate therapeutic regime.

[Evaluation years in leprosy patients treated with single dose alternative scheme ROM (rifampin, ofloxacin, minocycline), after seven to nine]. / Avaliação de hansenianos tratados com esquema alternativo dose única ROM (rifampicina, ofloxacina e minociclina), após sete a nove 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%; 95%CI: 70-100,4) were cured, 5 patients (14,7%; 95%CI: 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% and a rate of relapse of 9.2% 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.

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% 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%) 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% 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.

Utility of measuring serum levels of anti-PGL-I antibody, neopterin and C-reactive protein in monitoring leprosy patients during multi-drug treatment and reactions.

OBJECTIVE: To verify the validity of measuring the levels of Mycobacterium leprae-specific anti-phenolic glycolipid (PGL)-I antibody, neopterin, a product of activated macrophages, and C-reactive protein (CRP), an acute phase protein, in serial serum samples from patients for monitoring the leprosy spectrum and reactions during the course of multi-drug treatment (MDT). METHODS: Twenty-five untreated leprosy patients, 15 multi-bacillary (MB) and 10 paucibacillary (PB), participated. Eight patients developed reversal reaction and five developed erythema nodosum leprosum (ENL) during follow-up. The bacterial index (BI) in slit-skin smears was determined at diagnosis and blood samples collected by venipuncture at diagnosis and after 2, 4, 6 and 12 months of MDT. PGL-I antibody and neopterin were measured by enzyme-linked immunosorbent assay, whereas the CRP levels were measured by the latex agglutination method. RESULTS: The levels of PGL-I antibodies and neopterin were higher in the sera of MB than PB patients, which correlated with the patients' BI. The serum levels of CRP did not differ significantly between the MB and PB patients. The serum levels of PGL-I and neopterin were no higher in reactional patients than non-reactional patients prone to such reactions. However, ENL patients had higher serum CRP levels than non-reactional MB patients. The serum PGL-I antibody levels declined significantly during MDT, in contrast to neopterin and CRP levels. CONCLUSION: Measuring the serum levels of PGL-I antibodies and neopterin appeared to be useful in distinguishing MB from PB patients, whereas monitoring the levels of PGL-I antibodies appeared to be useful in monitoring MB patients on MDT. Measuring serum CRP, although not useful in monitoring the patients, has limited significance in detecting ENL reactional patients.