Negligência no diagnóstico precoce de hanseníase na atenção primária: um relato de caso / Negligence in the early diagnosis of leprosy in primary care: a case report

A hanseníase é uma doença infecciosa, transmissível, de caráter crônico, com potencial grau de incapacidade, que ainda persiste como problema de saúde pública no Brasil. A demora e a falta de conhecimento técnico para realizar o diagnóstico resulta em inúmeros prejuízos aos pacientes acometidos pela doença, sendo que, a prevenção das incapacidades está relacionada diretamente com o diagnóstico precoce da doença. Com a finalidade de evitar a negligência diagnóstica e o desenvolvimento de incapacidades físicas, ressaltamos a importância do conhecimento técnico sobre o diagnóstico e o manejo da hanseníase por profissionais da saúde em qualquer nível de atenção à saúde ou especialidade.

Hansen is an infectious disease, transmissible, of a chronic nature, with serious potential for disability, which still persists as a public health problem in Brazil. The delay and the lack of technical knowledge to carry out the diagnosis with numerous prejudices to the patients affected by the disease, since the prevention of disabilities is directly related to the early diagnosis of the disease. In order to avoid diagnostic negligence and the development of physical disabilities, we highlight the importance of technical knowledge about the diagnosis and management of training by health professionals at any level of health care or special care.

Efficacy of fixed duration multidrug therapy for the treatment of multibacillary leprosy: A prospective observational study from Northern India

Background: In endemic regions of several countries, the prevalence of leprosy has not come down to the level of elimination. On the contrary, new cases are being detected in large numbers. Clinically, it is frequently noted that despite completion of multibacillary multidrug therapy for 12 months, the lesions remain active, especially in cases with high bacteriological indices. Aim: The present study focused on finding out the viable number of Mycobacterium leprae during the 12-month regimen of multibacillary multidrug therapy, at six and 12 months intervals and, attempting to determine their role in disease transmission. Methods: Seventy eight cases of multibacillary leprosy cases were recruited from leprosy patients registered at The Leprosy Mission hospitals at Shahdara (Delhi), Naini (Uttar Pradesh) and Champa (Chhattisgarh), respectively. Slit skin smears were collected from these patients which were transported to the laboratory for further processing. Ribonucleic acid was extracted by TRIzol method. Total Ribonucleic acid was used for real-time reverse transcription-polymerase chain reaction (two-step reactions). A standard sample with a known copy number was run along with unknown samples for a reverse transcription-polymerase chain reaction. Patients were further assessed for their clinical and molecular parameters during 6th month and 12th month of therapy. Results: All 78 new cases showed the presence of a viable load of bacilli at the time of recruitment, but we were able to follow up only on 36 of these patients for one year. Among these, using three different genes, 20/36 for esxA, 22/36 for hsp18 and 24/36 for 16S rRNA cases showed viability of M. leprae at the time of completion of 12 months of multidrug therapy treatment. All these positive patients were histopathologically active and had bacillary indexes ranging between 3+ and 4+. Patients with a high copy number of the Mycobacterium leprae gene, even after completion of treatment as per WHO recommended fixed-dose multidrug therapy, indicated the presence of live bacilli. Limitations: Follow up for one year was difficult, especially in Delhi because of the migratory nature of the population. Patients who defaulted for scheduled sampling were not included in the study. Conclusion: The presence of a viable load of bacilli even after completion of therapy may be one of the reasons for relapse and continued transmission of leprosy in the community

Necrotic erythema nodosum reaction associated with histological alterations of Lucio's phenomenon

Abstract Patients with lepromatous or borderline leprosy may present two types of vasculonecrotic reactions: Lucio's phenomenon (LP) and necrotic erythema nodosum leprosum (nENL). These are serious conditions, which mostly lead to life-threatening infectious and thrombotic complications. The authors report the case of a patient with leprosy recurrence associated with an atypical type II reaction with LP characteristics on histopathology.

Aspectos clínicos y epidemiológicos en pacientes timorenses con enfermedad de Hansen / Clinical and epidemiological aspects in Timor patients with the Hansen disease

Se realizó un estudio observacional, descriptivo y transversal de 14 pacientes con lepra, diagnosticados y atendidos en la consulta de Dermatología del Hospital Nacional Guido Valadares, en Dili, Timor Oriental, de julio a diciembre del 2015, con vistas a caracterizarles y describir algunos aspectos clínicos y epidemiológicos de la enfermedad. En la serie se obtuvo una mayor frecuencia de pacientes en el grupo etario de 26 a 35 años y del sexo masculino, la forma clínica predominante fue la multibacilar y el tiempo de evolución de los síntomas hasta que fuera establecido el diagnóstico fue de menos de un año. Igualmente, en la mayoría existieron reacciones agudas, principalmente de tipo II o eritema nudoso leproso, y diferentes grados de discapacidad.

An observational, descriptive and cross-sectional study of 14 patients with leprosy, diagnosed and assisted in the Dermatology Service of Guido Valadares National Hospital, in Dili, Timor Lester was carried out from July to December, 2015, with the aim of characterizing them and to describe some clinical and epidemiological aspects of the disease. In the series a higher frequency of patient was obtained in the age group 26 to 35 years and of the male sex, the predominant clinical form was the multibacilar and the time of clinical course of the symptoms up to reaching the diagnosis was shorter than a year. Equally, in most of them acute reactions were presented, mainly of type II or from the erythema leprosy group, and different degrees of inability.

Lepra histioide o de Wade: Presentación de un caso clínico / Wade histioid leprosy: A case report

La lepra histioide de Wade, es una forma clínico-histopatológica especial de lepra multibacilar, caracterizada por la presencia de tubérculos (lesiones papulosas o nodulares) correspondientes a lepromas formados por histiocitos de morfología fusiforme. La misma es una variante infrecuente de lepra lepromatosa, que se desarrolla generalmente como recaída, en pacientes que recibieron monoterapia con dapsona, aunque se reportó su presentación de novo. En el presente trabajo, se comunica el caso clínico de una paciente de sexo femenino de 45 años de edad, con diagnóstico tardío de lepra histioide. Además, se examinan las características principales de esta forma particular de expresión de la lepra, sus diferencias con la forma clásica de presentación, los diagnósticos diferenciales que deben considerarse y la importancia de tener a esta patología entre las sospechas diagnósticas, para comenzar el tratamiento adecuado y evitar su propagación.

Wade's hystioid leprosy is a special clinical-pathological form of multibacillary leprosy, characterized by papular and nodular lepromas that consist of spindle histiocytes. It is a variant of lepromatous leprosy. Most of the cases have been related to dapsone resistance in the context of longterm monotherapy. De novo cases, not associated with previous anti-leprosy treatment, have been less frequently reported. This article presents the case of 45 years old female, with late diagnosis of histoid leprosy. There will be explained the main features of this particular form of leprosy, its differences with the classic presentation, the differential diagnoses to be considered, and the importance of having this pathology among diagnostic suspicions to begin adecuate treatment.

Enfermedad de Hansen. Una condición emergente en Chile / Hansen disease, an emergent condition in Chile

Resumen La enfermedad de Hansen (EH) es una infección secundaria a Mycobacterium leprae, de curso crónico y que afecta preferentemente la piel y los nervios periféricos. Es una condición emergente en Chile debido a la migración humana. Presentamos un caso que afectó a un trabajador inmigrante desde Haití, que presentó antes de su arribo múltiples lesiones cutáneas en placas, con hipoestesia y madarosis unilateral. El diagnóstico de la forma multibacilar se estableció por los hallazgos clínicos, la presencia de bacilos ácido-alcohol resistentes en una muestra cutánea y la detección de células inflamatorias alrededor de los terminales nerviosos y granulomas en una biopsia de piel, desde donde se amplificaron segmentos específicos de los genes rpoB y hsp65 de M. leprae. El paciente fue tratado con el esquema combinado estándar de la OMS para la forma multibacilar durante un año, con regresión parcial de las lesiones. Durante el seguimiento se detectó por RPC una disminución del número de copias del gen rpoB de M. leprae en muestras nasofaríngeas hasta el sexto mes. El registro de enfermedades de notificación obligatoria demuestra un incremento reciente de casos de EH en Chile, todos relacionados con población migrante. La enfermedad de Hansen es una nueva condición clínica y los clínicos deben estar atentos a esta posibilidad diagnóstica. Las pruebas moleculares pueden ayudar en el diagnóstico y seguimiento.

Hansen's disease (HD) is caused by Mycobacterium leprae. It has a chronic course and preferentially affects the skin and the peripheral nerves. It's an emergent infection in Chile due to migration waves. This case report affecting a migrant worker from Haiti that presented several compatible skin lesions, with hypoesthesia and unilateral madarosis that appeared before arrival. The diagnosis of a multibacillary form was established by clinical findings, presence of fast acid bacilli on a direct skin smear, and inflammatory cell surrounding nerve endings and granulomas on skin biopsy. Besides, specific rpoB and hsp65 gene segments from M. leprae were amplified from skin samples. Patient was treated with the WHO standard combined regimen for multibacillary forms during one year showing partial regression of skin lesions. Nasopharyngeal samples showed the presence of M. leprae rpoB copies detected by PCR decreasing until six months of therapy. Notifiable diseases databases showed a recent increment of cases, all related to migrant population. Hansen's disease is a new condition in Chile and clinicians should be aware of this possibility. Molecular tools may facilitate diagnosis and follow up.

Wade's histoid leprosy in a 14-year-old teenage boy

Abstract Wade's histoid leprosy (HL) is a rare variant of multibacillary leprosy, with characteristic clinical, immunologic, histopathologic, and bacteriologic features. It is associated with resistance to sulfa drugs or polychemotherapy and is rarely observed in patients who have not undergone prior treatment. Clinically, HL resembles keloid or dermatofibroma. Furthermore, HL is rare in children and is difficult to diagnose even by experts. This report describes a case of HL in a 14-year-old Brazilian boy, who presented with multiple nodular and tumor-like lesions, simulating keloids. He had not undergone prior treatment with anti-leprosy drugs, which accentuates the relevance of this case report.

Verrucous lepromatous leprosy: a rare form of presentation - Report on two cases

Leprosy skin lesions are described as hypochromic or erythematous macules, pale erythematous or reddish-brown plaques, papules, nodules, and diffuse cutaneous infiltration, depending on the clinical form of the disease. They may be accompanied by hypo or anesthesia, alopecia, and hypo or anhidrosis. Verrucous lesions are now quite uncommon in leprosy. The literature is sparse, with only 25 reported cases of this association, especially in the lepromatous pole of the disease. This work is a report on two cases of lepromatous leprosy of long evolution, coursing with vegetant verrucous lesions.

Reação reversa macular da hanseníase: relato de caso / Macular reversal reaction in leprosy: a case report

A reação reversa maculosa consiste no aparecimento abrupto de máculas hipocrômicas, ocorrendo em pacientes hansenianos dimorfos que completaram o tratamento com poliquimioterapia para hanseníase multibacilar. Em geral, surgem entre 6 a 12 meses da alta, com baciloscopia negativa e boa resposta a corticoterapia sistêmica. Ressaltamos a dificuldade em diferenciar recidiva de um episódio reacional, já que não existem critérios clínicos bem estabelecidos que possibilitem este diagnóstico, além de existirem poucos relatos em literatura. Relatamos um caso clínico com diagnóstico de reação reversa macular após período variável de alta do tratamento de hanseniase dimorfa-dimorfa. Foi feita investigação por meio de anamnese rigorosa, exame dermatológico, exame histopatológico da lesão e baciloscopia, excluindo-se os critérios de recidiva, além de analisados dados anteriores do prontuário.O paciente foi submetido a corticoterapia sistêmica,apresentando melhora das lesões. Conclui-seque a reação reversa maculosa deve ser lembrada nos diagnósticos diferenciais com hanseníase recidivada e episódios reacionais clássicos, evitando retratamentos desnecessários.

Macular reversal reaction is the abrupt onset of hypochromic lesions, occurring in borderline leprosy patients who completed treatment with multidrugtherapy for multibacillary leprosy. In general, these reactions appear 6 to 12 months after medical discharge, showing negative skin smear and good response to systemic corticosteroid therapy. We emphasize the difficulty in differentiating relapse cases from leprosy reactions, as there are no well-established clinical criteria that allow this diagnosis, and moreover there are few reports about it in the literature. We report a borderline leprosy case diagnosed with macular reversal reaction after variable period of discharge from treatment. Detailed anamnesis, dermatological and histopathological examination and bacilloscopy, analysis of previous medical records, excluding the relapse criteria, were used for the investigation. The patient was submitted to systemic corticosteroid therapy, with improvement of the lesions. It is concluded that macular reversal reaction should be considered in the differential diagnosis of relapsed leprosy and classic reactional episodes, avoiding unnecessary retreatment.

Acometimento hepático na síndrome sulfona dapsona-induzida durante tratamento de hanseníase / Hepatic involvement in sulfone dapsone-induced syndrome during treatment of leprosy

A hanseníase é uma doença endêmica no Brasil e constitui grave problema de saúde pública. Em números absolutos, o Brasil é o segundo país que mais registra novos casos da doença por ano no mundo. O tratamento da hanseníase compreende: quimioterapia específica, supressão dos surtos reacionais, prevenção de incapacidades físicas, reabilitação física e psicossocial. A síndrome sulfona é uma condição multissistêmica potencialmente grave que pode ocorrer durante o tratamento de algumas dermatoses, entre elas a hanseníase. Relatamos um caso de síndrome de hipersensibilidade à dapsona (SHD) em um paciente masculino, de 32 anos, ocorrida durante o tratamento de hanseníase multibacilar...

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.

Treatment of Erythema Nodosum Leprosum (ENL) with high-dose Clofazimine in patients with Lepromatous Leprosy

ENL is a type ll leprosy reaction and occurs in people with borderline lepromatous and lepromatous leprosy, usually as a complication following treatment. The treatment of choice for ENL is prednisolone in view of its’ ready availability and affordability1. Howeve r, glucocorticoid therapy, even in low doses, can produce substantial toxicity. The risk is clearly greater as the dose increases. However, in cases where there are steroid-induced complications, high-dosed clofazimine may be used to reduce or withdraw corticosteroids in steroid-dependant cases2,3. We described 2 steroid-dependent ENL patients with steroid-induced complications who are successfully managed with the addition of highdosed clofazimine and the resultant weaning down of systemic glucocorticoids.

Delayed diagnosis of multibacillary leprosy: a report of eight cases

Leprosy is an important public health problem in Brazil. However, this disease is still poorly diagnosed in its early stages, leading to permanent disability and disfigurement. We examined eight patients with clinical and histologicaldiagnosis of multibacillary leprosy who were being treated for other diseases for about three years without clinicalhypothesis of leprosy. These cases illustrate the importance of medical education and public information aboutleprosy’s signs and symptoms for prompt recognition and treatment, which are necessary to prevent permanentdisabilities and eradicate the disease.

O uso da sorologia como ferramenta adicional no apoio ao diagnóstico de casos difíceis de hanseníase multibacilar: lições de uma unidade de referência / The use of serology as an additional tool to support diagnosis of difficult multibacillary leprosy cases: lessons from clinical care

Sete casos de hanseníase multibacilar (MB) e dois casos com suspeição de hanseníase atendidos em situações distintas do atendimento clínico-dermatológico na Universidade Federal do Rio de Janeiro são descritos. Todos apresentaram dificuldades no diagnóstico visto que não tinham sinais e sintomas cardinais da hanseníase. Um teste sorológico utilizado como ferramenta auxiliar foi útil no processo de diagnóstico ou exclusão de cada caso e facilitou as discussões acadêmicas na hora do exame clínico. A sorologia e baciloscopia de linfa são consideradas como os únicos instrumentos rápidos e de baixo custo para a confirmação de casos MB atípicos, e as vantagens e desvantagens de cada exame são discutidas. Ambos os testes complementam o processo diagnóstico e classificação dos casos para fins terapêuticos. A vantagem da baciloscopia está na sua capacidade de confirmação do diagnóstico. As vantagens da sorologia são: (a) sua aplicabilidade para uso direto por profissionais de saúde no momento da consulta, visto que os resultados são imediatos, (b) a possibilidade da participação dos pacientes no processo, e (c) oferece uma oportunidade para melhor ensino da patogênese da hanseníase.

Seven multibacillary leprosy and two suspected cases assisted in different situations during clinical care activities at the university in Rio de Janeiro city are described. All cases presented some difficulties for diagnosis, since they evolved with few or no cardinal signs or symptoms of leprosy. A serological test used as an auxiliary tool was helpful in the diagnosis or exclusion procedure of each case, facilitating academic discussions at the time of case examination. Considering serology and bacilloscopy (skin smear) as the only rapid and relatively cheap available tests for confirmation of atypical MB leprosy, the advantages and disadvantages of their use were discussed. Both tests support the diagnostic procedure and the classification of cases for treatment purposes. The advantage of bacilloscopy is its capacity for diagnosis confirmation. The advantages of serology are: (a) its applicability for direct use by health workers, providing immediate results; (b) the potential for patient participation in the process; and (c) it provides a learning opportunity, allowing for improved teaching of leprosy pathogenesis.

Estudo terapêutico comparando a associação de rifampicina, ofloxacina e minociclina com a associação rifampicina, clofazimina e dapsona em pacientes com hanseníase multibacilar / Therapeutic study comparing the association rifampin, ofloxacinand minocycline, with the association rifampin, clofazimine anddapsone in multibacillary leprosy patients

Foram comparados dois esquemas terapêuticos em pacientes com hanseníase multibacilar. O grupo controle com 14 pacientes recebeu o tratamento convencional.O grupo teste com 12 pacientes recebeu a associação de rifampicina 600 mg, ofloxacina 400 mg,e minociclina 100 mg, uma vez por mês, durante dois anos. Na avaliação inicial foram realizados exames clínicos, baciloscópicos e histológicos. A baciloscopia e a biópsia foram repetidas no final do primeiro e segundo ano de tratamento. As avaliações clínicas realizadas mensalmente. Todos pacientes apresentavam lesões cutâneas, que os caracterizavam como virchovianos ou peri-virchovianos. No grupo controle, o índice baciloscópico antes do tratamento variou de 2 a 4,8 e no grupo teste de 1,6 a 4,8. Histologicamente apresentavam quadro de hanseníase virchoviana ativa, exceto um paciente do grupo teste. Ao final do primeiro ano de tratamento estavam todos clinicamente melhorados,o índice baciloscópico diminuído e quadro histológico regressivo. Essa tendência se mantinha e ao final do segundo ano todos estavam clinicamente, baciloscopicamente e histologicamente ainda melhores. Análise estatística mostrou não haver diferença significante entre os grupos, sendo os esquemas equivalentes. No grupo controle todos apresentaram pigmentação cutânea devido a clofazimina. Os resultados deste estudo demonstraram que o esquema com rifampicina, ofloxacinae minociclina, teve eficácia e segurança equivalente a poliquimioterapia convencional para multibacilar. Além disso, não causa pigmentação cutânea, pode ser totalmente supervisionado, podendo ser utilizado como esquema alternativo

Two therapeutic schemes in multibacillary leprosy patients were compared. The control group with 14 patients received the conventional treatment (MDT-MB). The test group with 12 patients, received the association rifampin 600 mg, ofloxacin 400 mg and minocycline 100 mg (ROM), administrated under supervision once a month, during two years. Initial evaluations include clinical, bacilloscopic and histological exams. The bacilloscopy and the biopsy were repeated at the end of first and second year of treatment. Clinical evaluations were performed monthly. All patients presented skin lesions characteristic of the lepromatous type. In the control group, the bacterial index (BI) before treatment ranged from 2 to 4.8 and in the test group it ranged from 1.6 to 4.8. Histological picture resembled active lepromatous leprosy, except one patient from the test group. At the end of the first year of treatment all patients showed clinical improvement, decreased BI and regressive histological picture. This tendency was maintained and at a final evaluation at the end of the second year all patients showed improvement on clinical, bacilloscopic and histological evaluations. Statistical analysis showed no significant differences between the groups, therefore, the two schemes were similar. In the control group all patients presented skin pigmentation after clofazimine intake. The results demonstrated that monthly administration of ROM is as efficacious and safe as MDT-MB. Besides, it doesn’t cause skin pigmentation, it can be given under supervision and can be used as alternative scheme.

Recidivas postratamiento de la lepra multibacilar / Relapses after multibacillary leprosy treatment

La persistencia de bacilos viables y la monoterapia con diamino-difenil-sulfona (DDS) son los principales factores que favorecen las recidivas de la lepra. Presentamos 33 pacientes con lepra lepromatosa (LL) diagnosticada 7 a 48 años antes de la recidiva, que recibieron monoterapia con DDS durante 4 a 38 años. Veintiocho fueron tratados, además, con poliquimioterapia (PQT) irregular, no supervisada, desde 1983. Cinco sólo recibieron DDS. Éstos presentaron la recidiva entre 13 y 20 años después de suspenderla. Las recidivas se diagnosticaron por reaparición de las lesiones clínicas o por la presencia de nuevas zonas anestésicas; todas se confirmaron con la baciloscopia y, en 20 casos, por la biopsia de piel. Cuatro pacientes presentaron en la biopsia de la recidiva, lepra indeterminada (LI) y uno lepra dimorfa tuberculoide (LDT), todos con presencia de bacilos intraneurales; los demás fueron LL. Dos pacientes recidivaron, aun con PQT razonablemente supervisada. Los demás curaron con PQT supervisada. Los factores predisponentes para la recidiva fueron: monoterapia con DDS por varios años; PQT irregular con dosis inadecuadas, sin supervisión del tratamiento; abandono de la PQT, y relación inadecuada entre el paciente y el personal de salud. Las recidivas de la lepra se deben buscar en todos los pacientes colombianos con lepra multibacilar que fueron tratados con DDS solo durante años. La clínica, la baciloscopia y la biopsia individualmente o en conjunto son métodos confiables para establecer las recidivas.

Leprosy relapses are mainly due to bacillary persistence and diamino-diphenyl-sulphone (DDS) monotherapy. Case histories were examined for 33 patients with lepromatous leprosy (LL), diagnosed 7-48 years before the relapse and treated only with DDS during 4 to 38 years. Twenty-eight patients received irregular non-supervised polychemotherapy (PCT) since 1983. Five patients received only DDS, and presented relapses 13-20 years after the treatment was stopped. Relapses were diagnosed by clinical methods, including the reappearance of lesions or presence of new anesthetic areas. All cases were confirmed by bacilloscopy, and a subset of 20 cases by skin biopsy. Four patients presented indeterminate leprosy (IL) and one patient borderline tuberculoid leprosy (BT) in the biopsy. The latter 5 demonstrated presence of intraneural bacilli; the remainder were LL. Two patients relapsed even with PCT treatment. The others were cured with supervised PCT. Predisposing factors for relapses were as follows: DDS monotherapy, irregular PCT with inadequate dosage, unsupervised treatment, treatment uncompliance, and inadequate relationship between the patient and the health staff. Inspections for relapse in leprosy is recommended for in all multibacillary patients that were treated with DDS. The clinical appearance of new lesions or new anesthetic zones, the bacilloscopy and skin biopsy, used together, are effective in establishing the presence of relapses.