Retinopatia hipertensiva maligna secundária a lúpus eritematoso sistêmico em adolescente / Malignant hypertensive retinopathy secondary to systemic lupus erythematous in adolescent

RESUMO O lúpus eritematoso sistêmico é uma doença que pode apresentar comprometimento oftalmológico geralmente benigno, sendo as alterações mais encontradas a síndrome do olho seco e a catarata. Nos pacientes com a doença estável, o dano oftalmológico parece estar relacionado ao tratamento sistêmico a longo prazo, o que enfatiza a importância do exame oftalmológico completo de rotina. Porém, quando a doença está em franca atividade e, em especial, quando há o envolvimento renal, deve-se iniciar o tratamento precoce com corticoterapia sistêmica e com medidas de suporte, para se evitarem repercussões mais complexas, como as crises hipertensivas que podem levar ao óbito.

ABSTRACT Systemic lupus erythematosus may present ophthalmological involvement, usually benign, and the most common changes are dry eye syndrome and cataract. In patients with stable disease, ophthalmologic damage appears to be related to long-term systemic treatment, emphasizing the importance of routine complete ophthalmologic examination. However, in full-blown disease, especially when there is renal involvement, early treatment should start with systemic steroid therapy and supportive measures, to avoid major repercussions, such as hypertensive crises that may lead to death.

Vogt-Koyanagi-Harada syndrome: a discussion about resistance to corticotherapy / Vogt-Koyanagi-Harada: uma discussão sobre resistência à corticoterapia

ABSTRACT Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition of unknown etiology that can affect the eye. The most common ocular manifestation related to VKH is bilateral diffuse uveitis associated to exudative retinal detachment. Although these patients respond well to steroid pulse therapy, we report a case of a 44-year-old female patient presenting bilateral exudative retinal detachment and clinical diagnosis of VKH, who did not respond to the first cycle of 3-day pulse therapy with methylprednisolone. The exudation was reabsorbed only after a second cycle of steroid therapy.

RESUMO A doença de Vogt-Koyanagi-Harada é inflamatória e de etiologia desconhecida, podendo afetar o olho. A manifestação ocular mais comum relacionada à doença de Vogt-Koyanagi-Harada é a uveíte difusa bilateral associada ao descolamento exsudativo da retina. Embora esses pacientes respondam bem à pulsoterapia com esteroides, relatamos um caso de paciente de 44 anos que apresentou descolamento exsudativo bilateral da retina com diagnóstico clínico de doença de Vogt-Koyanagi-Harada que não respondeu ao primeiro ciclo de pulsoterapia de 3 dias com metilprednisolona. A exsudação apenas reabsorveu após uma segunda rodada de terapia com esteroides.

Síndrome hemofagocítica pós vacinal: um relato de caso / Post vacinal hemofagocytic syndrome: a case report

Introdução: síndrome hemofagocítica (HLH) é uma condição agressiva e potencialmente fatal que acomete principalmente crianças pequenas. Relato: menino, 2 meses, apresentou quadro catarral após realização de vacinas, evoluindo com anemia hemolítica e posterior coagulação intravascular disseminada, com necessidade de uso de drogas vasoativas e ventilação mecânica. Realizado tratamento com pulsoterapia de dexametasona, evoluindo com melhora completa do quadro. Discussão: a HLH corresponde a um distúrbio na homeostase do sistema imune que atinge principalmente crianças. O tratamento deve ser feito através de corticoterapia e, em alguns casos, drogas imunomoduladoras, imunossupressoras ou transplante alogênico de células hematopoiéticas. Conclusão: a HLH é uma patologia pouco comum, e sua ocorrência após a administração de vacinas é pouco relatada. Apresenta sintomas que mimetizam infecções comuns e deve ser aventada sua hipótese em casos de febre sem sinais de localização. Em geral, a evolução da doença é rápida e cursa com alta morbimortalidade.

Introduction: Hemophagocytic syndrome (HLH) is an aggressive and potentially fatal condition that affects mainly young children. Report: A 2-month-old boy presented with catarrhal symptoms after vaccinations, evolving with hemolytic anemia and later disseminated intravascular coagulation, requiring the use of vasoactive drugs and mechanical ventilation. Treatment with dexamethasone pulse therapy was performed, progressing with complete improvement of the condition. Discussion: HLH is a disorder in the homeostasis of the immune system that affects mainly children. Treatment should be done through corticotherapy and, in some cases, immunomodulatory, immunosuppressive drugs or allogeneic hematopoietic cell transplantation. Conclusion: HLH is an uncommon pathology, and its occurrence after vaccine administration is rarely reported. It presents symptoms that simulates common infections and its hypothesis should be considered in cases of fever without signs of localization. In general, the evolution of the disease is fast and progresses with high morbidity and mortality.

Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

Esclerite posterior bilateral simultânea e unilateral recorrente / Simultaneous bilateral and unilateral recurrent posterior scleritis

Resumo Esclerite posterior é uma doença inflamatória ocular que acomete a esclera e cujo diagnóstico é difícil. A clínica da doença envolve a piora da qualidade visual com dor ocular e achados do segmento posterior, como descolamento de retina seroso, dobras de coroide, edema de disco óptico e espessamento escleral. Deve-se ficar atento aos achados sistêmicos, pois, muitas vezes, está associada a doenças como artrite reumatoide, lúpus eritematoso sistêmico, doença inflamatória intestinal e doenças infecciosas, como sífilis e tuberculose. Este trabalho objetiva descrever dois casos: uma paciente com quadro de esclerite posterior bilateral simultânea, sem alterações sistêmicas correlacionadas com a doença. A apresentação bilateral é incomum nesta patologia; e outro caso de esclerite anterior e posterior unilateral recorrente.

Abstract Posterior scleritis is an ocular inflammatory disease that affects the sclera e whose diagnosis is difficult. The main clinical manifestations are worsening of visual quality, ocular pain and finding in the posterior segment, such as serous retinal detachment, choroidal folds, optic disc edema and sclera thickening. We should award for systemic findings, because it is often accompanied by rheumatoid arthritis, systemic lupus erythematosus, inflammatory bowel disease and infections, such as syphilis and tuberculosis.This paper aims to describe two cases: one patient with simultaneous bilateral posterior scleritis without systemic alterations correlated with the disease. Bilateral presentation is uncommon in this pathology; and another case of recurrent unilateral anterior and posterior scleritis.

Retinopatia vaso-oclusiva por lúpus eritematoso sistêmico associada à síndrome do anticorpo antifosfolipídeo / Vaso-occlusive retinopathy by systemic lupus erythematosus associated with the antiphospholipid antibody syndrome

Resumo Relatar um caso de paciente com Retinopatia vaso-oclusiva por Lúpus Eritematoso Sistêmico (LES) associado à Síndrome do Anticorpo Antifosfolipídeo (SAF), que se iniciou com um quadro de anemia hemolítica autoimune acompanhado por baixa visual súbita monocular. Poucos casos foram descritos na literatura nacional e mundial em que o LES se manifeste primeiramente com alterações oculares. O screening dos Anticorpos antifosfolípideos (APAs) é de suma importância para pacientes com retinopatia lúpica para que seja instituída a terapia imediata com anticoagulantes como forma de prevenir a trombose vascular, o que piora o prognóstico visual.

Abstract To report the case of a patient with vaso-occlusive retinopathy due to systemic lupus erythematosus (SLE) associated with antiphospholipid antibody syndrome (APAS), which started with signs and symptoms of autoimune hemolytic anemia accompanied by sudden monocular visual loss. Few cases of SLE manifestation primarily involving ocular changes have been reported in the Brazilian and international literature. Screening for antiphospholipid antibodies is of the greatest importance for patients with lupus retinopathy, so that immediate therapy with anticoagulants may be instituted in order to prevent vascular thrombosis, which worsens the visual prognosis.

Oral pemphigus treated with minipulse therapy

Pemphigus is a chronic potentially fatal autoimmune disorder that causes blisters and erosions of the skin and oral mucous membrane. most of the cases present oral manifestations as the first clinical sign along with dermal lesions. only 0.5 to 3.2 of cases are reported each year per 1,000,000 population with oral manifestations without dermal participation, and is at times difficult to diagnose. we report a case of oral pemphigus vulgaris in a 20 year old female patient without dermal manifestations treated with oral mini pulse therapy. pénfigo oral tratado con terapia minipulse. resumen: el pénfigo es un trastorno autoinmune crónico potencialmente fatal que causa ampollas y erosiones de la piel y la membrana mucosa oral. la mayoría de los casos presentan manifestaciones orales como el primer signo clínico junto con lesiones dérmicas. solo se reportan de 0.5 a 3.2 casos cada año por cada 1,000,000 de personas con manifestaciones orales sin afectación de la piel, y algunas veces es difícil de diagnosticar. presentamos un caso de pénfigo vulgar oral en un paciente de 20 años, sin manifestaciones cutáneas tratadas con mini terapia del pulso oral.

Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review / Pneumomediastino espontâneo na dermatomiosite: uma série de casos e revisão de literatura

OBJECTIVES: To describe a case series of spontaneous pneumomediastinum in dermatomyositis and to review the literature. METHODS: This was a retrospective single-center case series, reporting 9 patients with pneumomediastinum and defined dermatomyositis, followed from 2005 to 2017. RESULTS: Median age of patients: 33 years; cutaneous and pulmonary involvement in all cases; constitutional symptoms in 88.8% of patients; involvement of the joints in 11.1%, gastrointestinal tract in 44.4%, and muscles in 77.7%; subcutaneous emphysema was observed in 55.5% and pneumothorax in 11.1%, respectively. Muscle weakness was observed in 77.7% of cases and with a median level of serum creatine phosphokinase of 124U/L. Drawing on results for our literature review, the overall analysis showed that the risk factors associated with spontaneous pneumomediastinum were: (a) a history of interstitial pneumopathy; (b) normal or low levels of muscle enzymes; (c) previous use of systemic glucocorticoid; (d) over 50% of patients had subcutaneous emphysema; (e) high mortality as a consequence of severity of the interstitial lung disease. CONCLUSIONS: Our case series revealed that pneumomediastinum is a rare complication in dermatomyositis that occurs in patients with a history of interstitial pneumopathy and may be accompanied by subcutaneous emphysema and pneumothorax.

OBJETIVOS: Descrever série de casos de pneumomediastino espontâneo em portadores de dermatomiosite e revisar a literatura. MÉTODOS: Trata-se de série de casos, único centro, relatando 9 pacientes com pneumomediastino e dermatomiosite definida, acompanhados de 2005 a 2017. RESULTADOS: A mediana da idade dos pacientes foi de 33 anos. Sintomas constitucionais estavam presentes em 88,8% dos pacientes. Houve acometimento cutâneo e pulmonar em todos os casos, acometimento das articulações em 11,1%, trato gastrointestinal em 44,4% e musculatura em 77,7% dos pacientes. Enfisema subcutâneo foi observado em 55,5% e pneumotórax em 11,1%, respectivamente. A fraqueza muscular foi observada em 77,7% dos casos, com um nível médio de creatinofosfoquinase sérica de 124U/L. Com base nos resultados da revisão da literatura, a análise geral mostrou que: os fatores de risco associados ao pneumomediastino espontâneo foram: história de pneumopatia intersticial, níveis normais ou baixos de enzimas musculares, uso prévio de glicocorticoide sistêmico; >50% dos pacientes tiveram enfisema subcutâneo; houve alta mortalidade como consequência da gravidade da doença pulmonar intersticial. CONCLUSÕES: Nossa série de casos revelou que o pneumomediastino é uma complicação rara na dermatomiosite e que ocorre em pacientes com história de pneumopatia intersticial e pode ser acompanhada por enfisema subcutâneo e pneumotórax.

Eficacia y seguridad de rituximab para el tratamiento de pacientes menores de 18 años con encefalitis autoinmune refractario al tratamiento de primera línea (inmunoglobulina, pulsos corticoides y plasmaféresis) / Efficacy and safety of rituximab for the treatment of patients younger than 18 years with autoimmune encephalitis refractory to first-line therapy (immunoglobulin, corticoid pulses and plasmapheresis)

INTRODUCCIÓN: Antecedentes: El presente dictamen expone la evaluación de la eficacia y seguridad del medicamento rituximab para el tratamiento de pacientes pediátricos con diagnóstico de encefalitis autoinmune refractarios a la inmunoterapia de primera línea. Aspectos Generales: La Encefalitis se refiere a un trastorno inflamatorio del cerebro que resulta en un estado mental alterado, convulciones, o problemas en el funcionamento del cerebro. Es de progresión rápida (menos de 6 semanas) y su incidencia estimada en paises industrializados de occidente (Finlandia, EEUU, Jutlandia, Inglaterra, Francia, Grecia, Canadá, Eslovenia) es de 6.3 a 7.4 casos por 100,000 habitantes (adultos y niños) por año y aproximadamente 10.5 a 13.8 casos por 100,000 niños por ano. Las causas de encefalitis mayormente reconocidas son las de origen infeccioso; no obstante, recientemente se ha descubierto que un 4% de las encefalitis son el resultado de la producción de anticuerpos que atacan los receptores neuronales y proteínas de superfície de las células involucradas en la transmisión sináptica, plasticidad, o excitabilidad neuronal. Tecnología Sanitaria de Interés: Rituximab: Rituximab es un anticuerpo monoclonal quimérico murino/humano que se une al antígeno CD20 localizado en la superficie de los linfocitos pre-B y B maduros. Este antígeno se encuentra tanto en células B normales como malignas. Tras la unión a CD20, rituximab destruye las células B. Los posibles mecanismos de lisis celular incluyen la citotoxidad dependiente del complemento (CDC) y la citotoxidad mediada por células dependientes de anticuerpos (ADCC); debido a ello es usado para el tratamiento de sídromes linfoproliferativoscrónicos de estirpe B, en enfermedades autoinmunes y en otras entidades donde hay proliferación de linfocitos B. METODOLOGÍA: Estrategia de Búsqueda: Se realizó una búsqueda sistemática de la evidencia, especialmente la proveniente de ensayos clínicos, con respecto a la eficacia y seguridad de rituximab en pacientes pediátricos con diagnóstico de encefalitis autoinmune en las bases de datos MEDLINE, TRIPDATABASE, ScienceDirect y LILACS. Una vez identificados los artículos que respondían a la pregunta PICO, se pasó a revisar la bibliografia incluida en dichos artículos seleccionados, con la finalidad de identificar evidencia adicional. Asimismo, se realizó una búsqueda dentro de bases de datos pertenecientes a grupos que realizan revisiones sistemáticas, evaluación de tecnologías sanitarias y guías de práctica clínica, tales como The National Guideline for Clearinghouse (NGC), Scottish Intercollegiate Guidelines Network (SIGN), The National Institute for Health and Care Excellence (NICE), The Canadian Agency for Drugs and Technologies in Health (CADTH), The Agency for Healthcare Research and Quality (AHQR) y The Cochrane Collaboration. Se hizo una búsqueda adicional en www.clinicaltrials.gov, para poder identificar ensayos clínicos en curso o que no hayan sido publicados. RESULTADOS: Sinopsis de la Evidencia: Se realizó una búsqueda de la literatura con respecto a a eficacia y seguridad de rituximab, en comparación a la terapia de primera línea (inmunoglobulina intravenosa, pulsos de corticoides y plasmaféresis) o placebo, como tratamiento de segunda línea en pacientes pediátricos con diagnóstico de encefalitis autonmune refractarios a la inmunoterapia de primera línea.No se encontratón ensayos clínicos aleatorizados por lo que se incluyeron resultados de estudios observacionlaes y series de casos que aportaran información relevante. CONCLUSIONES: A la fecha, no existe evidencia suficiente sobre la eficacia científica de rituximab, con respecto a la inmunoterapia de primera línea (corticoesteroides, inmunoglobulina intravenosa y plasmaféresis) o placebo, en pacientes pediátricos con diagnóstico de encefalitis autoinmune refractarioa a la inmunoterapia de primera línea en términos de mayor calidad de vida y disminución de secuelas neurológicas. No se encontraron ensayos clínicos que hayan evaluado el uso de rituximab en la población de la presente evaluación de tecnologia sanitaria. La GPC clínica encontrada para el tratamiento de este tipo de pacientes no incluyen a rituximab dentro de sus recomendaciones. Del mismo modo, la única revisión sistemática identificada concluye que existe un aparente beneficio en el uso de la inmunoterapia de segunda línea con respecto al no uso de esta, pero que estos resultados se ven afectados por sesgos de selección y de reporte. El Instituto de Evaluación de Tecnologías en Salud e Investigación (IETSI) aprueba el uso de rituximab como alternativa de tratamiento para pacientes pediátricos con diagnóstico de encefalitis autoinmune refractarios a la inmunoterapia de primera línea. El periodo de vigencia de este dictamen es de un año y la continuación de dicha aprobación estará sujeta a los resultados obtenidos de los pacientes que se beneficien con dicho tratamiento y a nueva evidencia que pueda surgir en el tiempo.

Eosinophilic fasciitis

Abstract Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen. Clinical features begin acutely, with local edema and a painful and symmetrical stiffening of the limbs, progressing rapidly to fibrosis, which can limit joint movements. Some cases have a history of strenuous physical exercise or trauma. The diagnosis is confirmed by a deep skin biopsy. Glucocorticoids in high doses is the treatment of choice. We report a typical eosinophilic fasciitis case with peripheral eosinophilia and dramatic response to pulse therapy with methylprednisolone.

A Comparative Study of Oral Cyclosporine and Betamethasone Minipulse Therapy in the Treatment of Alopecia Areata

BACKGROUND: Various systemic agents have been assessed for the treatment of alopecia areata (AA); however, there is a paucity of comparative studies. OBJECTIVE: To assess and compare cyclosporine and betamethasone minipulse therapy as treatments for AA with regard to effectiveness and safety. METHODS: Data were collected from 88 patients who received at least 3 months of oral cyclosporine (n=51) or betamethasone minipulse therapy (n=37) for AA. Patients with ≥50% of terminal hair regrowth in the alopecic area were considered responders. RESULTS: The responder of the cyclosporine group was 54.9% and that of the betamethasone minipulse group was 37.8%. In the cyclosporine group, patients with mild AA were found to respond better to the treatment. Based on the patient self-assessments, 70.6% of patients in the cyclosporine group and 43.2% of patients in the betamethasone minipulse group rated their hair regrowth as excellent or good. Side effects were less frequent in the cyclosporine group. CONCLUSION: Oral cyclosporine appeared to be superior to betamethasone minipulse therapy in terms of treatment effectiveness and safety.

Oclusão da artéria central da retina em paciente com poliangeíte microscópica / Central retinal artery occlusion in patient with microscopic polyangiitis

RESUMO A poliangeíte microscópica é uma vasculite necrotizante sistêmica que acomete arteríolas, capilares e vênulas, mas também pode atingir pequenas e médias artérias. É considerada uma doença rara, idiopática e autoimune. Diversas anormalidades oculares e sistêmicas estão associadas às oclusões arteriais retinianas. Dentre as doenças vasculares do colágeno, a literatura cita como possíveis causas de obstrução das artérias retinianas o lúpus eritematoso sistêmico, a poliarterite nodosa, a arterite de células gigantes, a granulomatose de Wegener e a granulomatose linfóide de Liebow. Até o presente momento, não se encontrou na literatura relatos da associação de casos de oclusão arterial retinana associados à PAM. Os autores relatam o caso de um paciente com poliangeíte microscópica que apresentou comprometimento renal importante e oclusão da artéria central da retina unilateral. Atenta-se para a inclusão de pesquisa da PAM, através do p-ANCA, na avaliação de possível origem sistêmica em pacientes acometidos por oclusão arterial retiniana.

ABSTRACT The microscopic polyangiitis is a systemic necrotizing vasculitis that affects arterioles, capillaries and venules, but can also reach small and medium-sized arteries. It is considered a rare disease, idiopathic in nature but clearly autoimmune. Several ocular and systemic abnormalities are associated with retinal arterial occlusions. Among the collagen vascular diseases, the literature cited as possible causes of retinal artery obstruction lupus erythematosus, polyarteritis nodosa, giant cell arteritis, Wegener’s granulomatosis and lymphoid Liebow. Until now, there were no reports in the literature of the association of cases of arterial occlusion retinana associated with PAM. The authors report a case of a 53 years old patient diagnosed with microscopic polyangiitis who presented with important renal artery occlusion and associated unilateral central retinal artery occlusion. An extended systemic evaluation of patients presenting with central retinal artery occlusion should include research of PAM through analysis op p-ANCA.

Comparative efficacy of continuous and pulse dose terbinafifine regimes in toenail dermatophytosis: A randomized double-blind trial.

Introduction: Dermatophytes are the most frequently implicated agents in toenail onychomycosis and oral terbinafi ne has shown the best cure rates in this condition. The pharmacokinetics of terbinafi ne favors its effi cacy in pulse dosing. Objectives: To compare the effi cacy of terbinafi ne in continuous and pulse dosing schedules in the treatment of toenail dermatophytosis. Methods: Seventy-six patients of potassium hydroxide (KOH) and culture positive dermatophyte toenail onychomycosis were randomly allocated to two treatment groups receiving either continuous terbinafi ne 250 mg daily for 12 weeks or 3 pulses of terbinafi ne (each of 500mg daily for a week) repeated every 4 weeks. Patients were followed up at 4, 8 and12 weeks during treatment and post-treatment at 24 weeks. At each visit, a KOH mount and culture were performed. In each patient, improvement in a target nail was assessed using a clinical score; total scores for all nails and global assessments by physician and patient were also recorded. Mycological, clinical and complete cure rates, clinical effectivity and treatment failure rates were then compared. Results: The declines in target nail and total scores from baseline were signifi cant at each follow-up visit in both the treatment groups. However, the inter-group difference was statistically insignifi cant. The same was true for global assessment indices, clinical effectivity as well as clinical, mycological, and complete cure rates. Limitations: The short follow-up in our study may have led to lower cure rates being recorded. Conclusion: Terbinafi ne in pulse dosing is as effective as continuous dosing in the treatment of dermatophyte toenail onychomycosis.

Weekly azathioprine pulse versus daily azathioprinein the treatment of Parthenium dermatitis: A non-inferiority randomized controlled study.

Background: Azathioprine in daily doses has been shown to be effective and safe in the treatment of Parthenium dermatitis. Weekly pulses of azathioprine (WAP) are also effective, but there are no reports comparing the effectiveness and safety of these two regimens in this condition. Aims: To study the effi cacy and safety of WAP and daily azathioprine in Parthenium dermatitis. Methods: Sixty patients with Parthenium dermatitis were randomly assigned to treatment with azathioprine 300 mg weekly pulse or azathioprine 100 mg daily for 6 months. Patients were evaluated every month to assess the response to treatment and side effects. Results: The study included 32 patients in the weekly azathioprine group and 28 in the daily azathioprine group, of whom 25 and 22 patients respectively completed the study. Twenty-three (92%) patients on WAP and 21 (96%) on daily azathioprine had a good or excellent response. The mean pretreatment clinical severity score decreased from 26.4 ± 14.5 to 4.7 ± 5.1 in the WAP group, and from 36.1 ± 18.1 to 5.7 ± 6.0 in the daily azathioprine group, which was statistically signifi cant and comparable (P = 0.366). Patients on WAP had a higher incidence of adverse effects (P = 0.02). Limitations: The study had a small sample size and the amount of clobetasol propionate used in each patient was not determined, though it may not have affected the study outcome due to its comparable use in both groups. Conclusions: Azathioprine 300 mg weekly pulse and 100 mg daily dose are equally effective and safe in the treatment of Parthenium dermatitis.

Nodular scleritis and granulomatous polyangiitis (Wegener) mimicking tuberculosis / Esclerite nodular e poliangiite granulomatosa (Wegener) mimetizando tuberculose

Granulomatous polyangiitis is a systemic disease that may affect any organ, with a predilection for the upper respiratory tract, lungs and kidneys. This article aims to report a case of a patient with atypical nodular scleritis as the initial manifestation of granulomatous polyangiitis (Wegener), mimicking a case of tuberculosis. The patient presented ocular hyperemia and lower progressive visual acuity for 1.5 years, followed by eye pain for two months. The patient had subpleural nodules with soft tissue density, increased pulmonary lymph nodes and discrete bilateral pleural thickening, with negative alcohol-resistant acid bacilli (BAAR). The histological diagnosis revealed a granulomatous vasculitis suggestive of non-infectious vasculitis (granulomatous polyangiitis). Cyclophosphamide pulse therapy was initiated.

Poliangiite granulomatosa é uma doença sistêmica que afeta qualquer órgão, com predileção pelo trato respiratório superior, pulmões e rins. Este artigo tem como objetivo relatar um caso atípico de uma paciente com esclerite nodular como manifestação inicial da poliangiite granulomatosa (Wegener), mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuía nódulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos álcool-ácido resistentes (BAAR). O diagnóstico histológico revelou uma vasculite granulomatosa sugestiva de vasculite não infecciosa (poliangiite granulomatosa). Foi iniciada pulsoterapia com ciclofosfamida.

Intravenous methylprednisolone pulse therapy in severe alopecia areata.

Background: Severe, extensive, therapy resistant alopecia areata represents a clinical challenge. Systemic corticosteroids are a therapeutic tool that still needs to be evaluated. Aim: The purpose of this study was to assess the efficacy and safety of methylprednisolone pulse therapy in alopecia areata and to find prognostic factors for a favourable outcome. Methods: A total of 32 patients with severe multifocal alopecia areata (more than 40% scalp hair loss), alopecia totalis, and alopecia universalis were treated with infusions of 500 mg methylprednisolone for 3 days every month for 3 consecutive months. The end point of the study was 12 months. Results: Of 32 patients, 26 (81.3%) reported a clinical response. Four patients (12.5%) showed complete hair regrowth, 6 patients (18.8%) showed >50% hair regrowth, ten (31.3%) had <50% hair regrowth, 6 (18.75%) were non responders, and another 6 patients (18.8%) had relapse after an initial regrowth. Multivariate analysis revealed that patients reporting at the first episode and those with multifocal disease had the best results. Conclusion: Methylprednisolone infusions represent a possible therapeutic option for patients with multifocal alopecia areata and those presenting with the first episode of the disease.

Monthly rifampicin, ofloxacin, and minocycline therapy for generalized and localized granuloma annulare.

Background: The localized form of granuloma annulare is usually self‑limiting, resolving within 2 years. Generalized granuloma annulare, on the other hand, runs a protracted course, with spontaneous resolution being rare. It is also characterized by a later age of onset, an increased incidence of diabetes mellitus, poor response to therapy, and an increased prevalence of HLA Bw35. Objective: To assess the efficacy of monthly pulsed rifampicin, ofloxacin, and minocycline (ROM) therapy in the management of granuloma annulare.Methods: Six biopsy proven patients of granuloma annulare were included in the study, five of the generalized variety, and one localized. Three of these patients were resistant to standard modalities of treatment. All six patients were treated with pulses of once monthly ROM till complete resolution of all lesions. Results were analyzed in terms of complete resolution of lesions and side effects. Presence of comorbid conditions was noted. Result: All six patients were successfully treated with 4-8 pulses of monthly ROM. None of the patients reported any adverse effects. Limitations: Small sample size and the lack of a control group are limitations. Conclusion: Treatment with pulses of once monthly ROM caused complete resolution of lesions in both localized and generalized granuloma annulare, even in cases recalcitrant to conventional therapy. There were no side effects in any of the patients. Larger trials are needed to substantiate the efficacy of monthly ROM in granuloma annulare.

Homonymous hemianopia in a patient with Behcet's disease

No abstract available.