Hanseníase virchowiana e eritema nodoso hanseníco em gestante de 34 semanas sem diagnóstico prévio / Virchowian leprosy and erythema nodosum leprosum in a 34-week pregnant woman without any previous diagnosis

Relatamos um caso de uma paciente feminina, gestante de terceiro trimestre, em acompanhamento pré-natal regular na unidade básica de saúde, com boa evolução gestacional, porém apresentando lesões de pele há cerca de um ano, acompanhadas de alteração de sensibilidade, além de fáscies infiltrada e madarose. Sendo o Brasil um país endêmico em Hanseníase, ocupando o 2º lugar no mundo em número de novos casos, chama a atenção o diagnóstico tardio da paciente em questão. Aproveitamos este emblemático relato de caso para discutir aspectos importantes em relação à terapêutica no período gestacional (poliquimioterapia conforme manual do ministério, sem nenhuma alteração por conta da gestação), desfecho obstétrico, orientações quanto à lactação (não contra-indicada com a mãe em tratamento; pelo contrário, devendo ser estimulada) e cuidado ao recém nato. [au]

We report a case of a pregnant female patient in the third trimester undergoing regular prenatal care at a Basic Health Unit, with good gestational evolution, but presenting skin lesions for approximately a year accompanied by changes in sensitivity, in addition to facial infiltration and madarosis. Considering Brazil as an endemic country for leprosy, ranking 2nd in the world concerning the number of new cases, late diagnosis of the patient in question stands out. We use this emblematic case report to discuss important aspects concerning the treatment of leprosy during the gestational period (multidrug therapy according to the Ministry of Health manual, without any changes due to pregnancy), obstetric outcome, guidelines regarding breastfeeding (not contraindicated with the mother in treatment; on the contrary, it should be stimulated) and care for the newborn. [au]

Validation of an algorithm based on clinical, histopathological and immunohistochemical data for the diagnosis of early-stage mycosis fungoides.

BACKGROUND: Diagnosis of mycosis fungoides is challenging due to the non-specificity of clinical and histopathological findings. The literature indicates an average delay of 4-6 years for a conclusive diagnosis. Refinement of the histopathological criteria for the diagnosis of patients in early stages of the disease is considered of interest. OBJECTIVES: To study the histopathological aspects of early-stage mycosis fungoides and the applicability, in a retrospective form, of the diagnostic algorithm proposed by Pimpinelli et al. METHODS: Observational, retrospective, transversal study based on revision of histopathological exams of patients with suspected mycosis fungoides. Medical records were reviewed, and complementary immunohistochemistry performed. RESULTS: Sixty-seven patients were included. The most frequent histopathological features were superficial perivascular lymphoid infiltrate (71.6%), epidermotropism (68.7%), lymphocytic atypia (63.8%), hyperkeratosis (62.7%) and acanthosis (62.7%). Forty-three patients scored 4 points at the algorithm, by clinical and histological evaluation. Immunohistochemistry was performed on 23 of the 24 patients with less than 4 points. Of those 23, 22 scored 1 point, allowing a total of 61 patients (91%) with the diagnosis of early-stage mycosis fungoides. STUDY LIMITATIONS: Its retrospective character, reduced sample size and incomplete application of the algorithm. CONCLUSIONS: Application of the Pimpinelli et al. algorithm, even in an incomplete form, increased the percentage of cases diagnosed as mycosis fungoides. Routine application of the algorithm may contribute to earlier and specific management and improvement of the patients' outcome.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides

Abstract: Background: Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. Objective: To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. Methods: This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. Results: One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. Study Limitations: Small sample and its retrospective analysis. Conclusions: Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. OBJECTIVE: To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. METHODS: This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. STUDY LIMITATIONS: Small sample and its retrospective analysis. CONCLUSIONS: Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.

Clinical and epidemiological profile of patients with early stage mycosis fungoides.

BACKGROUND: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. OBJECTIVE: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. METHODS: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. RESULTS: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. STUDY LIMITATIONS: Small sample and retrospective design. CONCLUSIONS: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.

Clinical and epidemiological profile of patients with early stage mycosis fungoides

Abstract: Background: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. Objective: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. Methods: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. Results: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. Study limitations: Small sample and retrospective design. Conclusions: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.

Pityriasis amiantacea: a study of seven cases.

Pityriasis amiantacea was first described in 1832. The disease may be secondary to any skin condition that primarily affects the scalp, including seborrheic dermatitis. Its pathogenesis remains uncertain. We aim to analyze the epidemiological and clinical profiles of patients with pityriasis amiantacea to better understand treatment responses. We identified seven cases of pityriasis amiantacea and a female predominance in a sample of 63 pediatric patients with seborrheic dermatitis followed for an average of 20.4 months. We reported a mean age of 5.9 years. Five patients were female, with a mean age of 9 years. All patients were successfully treated with topic ketoconazole.

Cutaneous Metastasis of Neuroendocrine Carcinoma with Unknown Primary Site: Case Report and Review of the Literature.

We report a new case of neuroendocrine carcinoma for which it was not possible to find the primary site until now. The recent medical literature about skin metastasis of neuroendocrine carcinoma (neuroendocrine tumor) is discussed.

Avaliação da resposta terapêutica ao infliximabe em pacientes com Doença de Crohn / Evaluation of infliximab treatment for Crohns Disease pacients

Introdução: infliximabe representa grande avanço no tratamento da Doença de Crohn refratária, demonstrada em ensaios clínicos, tanto no regime de indução quanto em manutenção. Objetivo: avaliar o tratamento com infliximabe, estudando sua eficácia, apontando possíveis fatores preditivos de resposta e, por fim, descrevendo possíveis reações adversas registradas. Resultados: após a indução, 86,1% dos pacientes responderam ao tratamento; na 54ª semana de manutenção, 88,9%. Descontinuaram corticosteroides, 83,4%. Ao final da avaliação, 83,4% estavam em remissão de doença. Discussão: as frequências de resposta foram maiores em relação aos ensaios clínicos, no entanto, semelhantes em relação aos trabalhos mais recentes, retrospectivos e prospectivos. Não foram encontrados fatores preditivos de resposta. As respostas de pacientes com Doença de Crohn ativa e fistulizante foram semelhantes. Um pequeno número de pacientes (05) apresentou efeitos adversos atribuíveis ao medicamento, porém nenhum precisou interromper ou suspender tratamento. Conclusão: o infliximabe foi efetivo e seguro entre os pacientes com Doença de Crohn ativa e fistulizante.

Introduction: infliximab represents a great advance in refractory Crohns Disease treatment, showed in clinical trials, for induction as well as for maintenance therapy. Aim: evaluate the infliximab treatment, studying its efficacy, pointing possible predictive factor of response, and describing register collateral effects. Results: after induction, 86.1% of the patients improved with the treatment; 88.9% improved with maintenance therapy at week 54. 83.4% were able to discontinue corticosteroids. By the end of the evaluation, 83.4% were in clinical remission. Discussion: however, the response was superior when compared to the clinical trials; it was similar to more recent studies. It was not found any predictors of response to infliximab. A small number of patients reported side effects possibly associated with the drug, but none of them needed to discontinue treatment. Conclusion: the infliximab was effective and safe among patients with active and fistulizing Crohns Disease.