Case for diagnosis. Cutaneous small vessel vasculitis (anti-proteinase 3 positive), fever, hemoptysis, and lung cavitation in an adult

Abstract Small vessel vasculitis with anti-proteinase antibodies 3 is an atypical clinical presentation of tuberculosis. The authors present the case of a 47-year-old male patient, with palpable purpura and palmoplantar hemorrhagic blisters, with subsequent dissemination. He presented severe pulmonary symptoms with cavitation, fever, hemoptysis, and high levels of anti-proteinase 3. Histopathological assessment of the skin revealed small vessel vasculitis; pulmonary histopathology showed granulomas with caseation. Bronchoalveolar lavage was positive for alcohol-acid-fast bacilli. In countries with a high prevalence of tuberculosis, the presence of autoantibodies in a patient with vasculitis, fever, and pulmonary cavitation requires investigation of infectious causes.

Vasculitis inusual asociada a anticuerpos anticitoplasma de neutrófilos / Unusual vasculitis associated with antineutrophil cytoplasmic antibodies

Introducción: Las vasculitis asociadas a autoanticuerpos citoplasmáticos de neutrófilos son afecciones infrecuentes con una incidencia reportada de 46 a 184 casos por millón de personas y aparecen, generalmente, en edades tardías de la vida. Objetivo: Describir los hallazgos de una paciente en la que se diagnosticó vasculitis pauciinmune tipo poliangeítis microscópica tardíamente. Caso clínico: Se presenta el caso de una paciente de 60 años con antecedente de enfermedad renal crónica y compromiso respiratorio manifestado por disnea, tos productiva, malestar general y fiebre. En las imágenes se evidenció bronquiectasias y en los exámenes de laboratorio factor reumatoideo alto, autoanticuerpos citoplasmáticos antineutrófilos perinucleares positivos, anticuerpos antinucleares negativos y hemosiderófagos en el lavado broncoalveolar. Se instauró manejo con corticoides sistémicos con franca mejoría clínica. Sin embargo, una malformación arteriovenosa que originó una hemorragia en la fosa posterior, le ocasionó la muerte. Conclusiones: El diagnóstico de las vasculitis es complejo y depende en gran medida del criterio clínico, por lo que el médico debe estar presto a la interpretación de las manifestaciones clínicas y de los hallazgos paraclínicos(AU)

Introduction: Vasculitis associated with neutrophil cytoplasmic autoantibodies are infrequent conditions with a reported incidence of 46 to 184 cases per million people and it generally appears late in life. Objective: To describe the findings of a patient in whom microscopic polyangiitis-type pauciimmune vasculitis was late diagnosed. Clinical case report: We report a case of a 60-year-old patient with history of chronic kidney disease and respiratory compromise exhibited by dyspnea, productive cough, general malaise and fever. The images showed bronchiectasis and laboratory tests revealed high rheumatoid factor, positive perinuclear antineutrophil cytoplasmic autoantibodies, negative antinuclear antibodies, and bronchoalveolar lavage exposed hemosiderophages. Management with systemic corticosteroids was established with clear clinical improvement. However, an arteriovenous malformation that caused a hemorrhage in the posterior fossa caused his death. Conclusions: The diagnosis of vasculitis is complex and depends largely on clinical criteria, so physicians must be ready to interpret the clinical manifestations and paraclinical findings(AU)

Enfermedad de Kawasaki y su posible asociación con el nuevo SARS-CoV-2 (Covid-19) / Kawasaki Disease and its possible Association with the new SARS CoV-2 (Covid-19)

La Enfermedad de Kawasaki (EK) o Síndrome de Kawasaki, es una enfermedad febril aguda, de etiología desconocida, que afecta mayormen-te a población pediátrica menor de 5 años y es una causa común de patología cardíaca en estos grupos etarios. Su diagnóstico es definido por la presencia de ciertos criterios clínicos caracterís-ticos como fiebre persistente de al menos 5 días de evolución, linfadenopatías cervicales unila-terales, inyección conjuntival, alteraciones en la mucosa oral, edema en extremidades y como parte de las complicaciones severas que pue-de presentar, se incluyen la formación de aneu-rismas en las arterias coronarias, entre otros.Actualmente, el mundo se ha detenido por la aparición del nuevo SARS CoV-2 (Covid-19), que se ha dado a conocer por sus efectos de-vastadores, tanto por sus abundantes pérdidas humanas y su enorme golpe en las diferentes economías mundiales. Ya es conocida la rela-ción de otros coronavirus humanos que se han vinculado con la EK, sin embargo es de recien-te aparición, algunos reportes de casos en va-rios países, que vinculan pacientes pediátricos que cumplen criterios de EK con la infección confirmada de SARS CoV-2.En el presente es-crito, se describirá la posible vinculación de éste nuevo virus con la Enfermedad de Kawasaki...(AU)

Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis - part I

Abstract The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.

Livedoid Vasculopathy: diagnosis and treatment in pregnant women / Vasculopatia livedoide: diagnóstico e tratamento em mulher grávida

Abstract Livedoid Vasculopathy is a disease characterized by occlusion of the capillaries of the dermis, without inflammatory signs. It begins with purpuric papules or macules that develop into painful ulcers, mainly involving the ankles and feet. In this case report, we describe diagnosis and treatment in a young pregnant patient, with excellent clinical response.

Resumo A vasculopatia livedoide é uma doença caracterizada pela oclusão dos capilares da derme, sem sinais inflamatórios. Tem início com pápulas ou máculas purpúricas que evoluem para úlceras dolorosas, com predominância na topografia de tornozelos e pés. Neste relato de caso, descrevemos o diagnóstico e a terapêutica em uma paciente jovem gestante, com excelente evolução clínica.

Manifestaciones atípicas del síndrome de vasculitis-angeítis- endotelitis (SVAE) cutánea / Atypical manifestations of cutaneous vasculitis- angeitis-endotheliitis syndrome

Se reportan dos pacientes con diagnóstico histopatológico de vasculitis cutánea de presentación clínica atípica. Un joven de 13 años con lesiones bullosas y flictenulares generalizadas de 2-3 cm sin sobreinfección, que se iniciaron en la cavidad oral y se extendieron al tórax anterior y extremidades, asociadas con infección ventilo-respiratoria tipo neumonía adquirida en la comunidad (NAC), que requirió antibióticos y corticoides sistémicos, con resolución completa a los 17 días y diagnóstico definitivo de vasculitis por hipersensibilidad. El otro paciente corresponde a un adulto de 58 años con historia de 18 meses de lesiones maculares purpúricas en miembros superiores e inferiores, algunas confluyen generando placas violáceas no mayores de 5 cm. Cursó con diabetes, retinopatía e hipertensión arterial. La biopsia mostró vasculitis leucocitoclásica con "signo de promontorio", por lo cual se sospechó sarcoma de Kaposi que se descartó. El diagnóstico definitivo fue vasculitis linfocítica en pitiriasis liquenoide crónica (PLC).

Two patients with histopathological diagnosis of cutaneous vasculitis of atypical clinical course are presented. The first patient was a 13-year-old boy with bullous lesions and generalized phlyctenules measuring 2-3 cm with no superinfection, which began in the oral cavity and extended to the anterior chest and extremities, associated with ventilation-respiratory community acquired pneumonia (CAP), which required antibiotic therapy and systemic corticoids, with complete resolution at 17 days and definitive diagnosis of hypersensitivity vasculitis. The other patient corresponds to a 58-year-old man with purpuric macular lesions in upper and lower extremities of 18 months duration. Some of them were confluent purpuric plaques not greater than 5 cm. Patient had associated diabetes, retinopathy and hypertension. Biopsy showed leukocytoclastic vasculitis with the presence of "promontory sign" suggestive of Kaposi ́s sarcoma which was ruled out. The definitive diagnosis was lymphocytic vasculitis in a patient with pytriasis lichenoids chronica (PLC).

Algorithm for diagnosis of primary vasculitides / Algoritmo diagnóstico de vasculites primárias

Primary vasculitides are diseases with a wide variety of anatomical, clinical, radiological, and laboratory presentations. Primary vasculitides are difficult to diagnose because of the complexity of clinical presentation, which may lead to delayed treatment and increased financial costs of workup investigations involving non-essential tests. Our objective in the present study is to create an algorithm that helps diagnosis of Primary vasculitides. The algorithm presented in this article allows fast, simple and cost-effective diagnosis of primary vasculitides using just clinical concepts and a few laboratory tests

As vasculites primárias são doenças que possuem apresentações anatômicas, clínicas, radiológicas e laboratoriais muito distintas. Em virtude da complexidade dos quadros clínicos apresentados, as vasculites primárias são de difícil diagnóstico, o que pode promover um retardo no início da terapêutica, além de aumentar os custos financeiros da investigação propedêutica com exames não essenciais. O objetivo deste estudo foi criar um algoritmo que auxilie o diagnóstico das vasculites primárias. O algoritmo apresentado neste artigo permite a realização de diagnóstico rápido, simples e de baixo custo nas vasculites primárias, com a utilização de alguns conceitos clínicos e poucos exames laboratoriais

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

Cutaneous vasculitis and renal involvement in Mycoplasma pneumoniae infection

No abstract available.

Vasculitis paraneoplásica: presentación de 2 casos clínicos / Paraneoplastic vasculitis: presentation of two clinical case

Las vasculitis constituyen un grupo heterogéneo de enfermedades que se definen por un proceso inflamatorio de la pared vascular, con obstrucción y necrosis distal. Son poco frecuentes y pueden ser primarias o asociadas a infecciones, enfermedades sistémicas o excepcionalmente a neoplasias. La asociación de vasculitis y neoplasia es infrecuente; la vasculitis se presentaantes, de forma concomitante o posteriormente al diagnóstico de la neoplasia. Las más frecuentemente asociadas son las de origen hematooncológico y luego, en menor medida, los tumores sólidos. Las manifestaciones cutáneas por afectación de “pequeño vaso” son las más habituales, y dentro de ellas las leucocitoclásticas. Se presentan 2 casos clínicos excepcionales de vasculitis paraneoplásicas, el primer caso se trata de una paciente de 57 años con lesiones vasculíticas de pequeño vaso asociadas a cáncer broncopulmonar y el segundo caso se trata de una paciente de 79 años con diagnóstico previo de glioblastoma encefálico de alto grado y lesiones en miembros inferiores con características de vasculitis de pequeño vaso.

Vasculitis constitutes a heterogeneous group of diseases that are defined by an inflammatory process of the vascular wall, with occlusion and distal necrosis. They are rare and may be primary or secondary infections, systemic disease or exceptionally to neoplasic disease. The association of vasculitis and neoplasia is uncommon; vasculitis occurs before, concomitantly or subsequently to the diagnosis of neoplasia. The most frequently associated are the hemato-oncology and then to a lesser extent the solid tumors. Cutaneous manifestations by affectation of “small vessel” are the most common. We present 2 exceptional clinical cases of vasculitis associated with cancer, the first case is about a 57 years old patient with lesions of small vessel associated with bronchopulmonary cancer and the second case is a 79 years old patient diagnosed with brain glioblastoma of high-grade and injuries in lower limbs with characteristics of small vessel vasculitis.

Situações especiais nas vasculites pulmonares / Special situations in pulmonary vasculitis

A extensa rede vascular pulmonar e brônquica, associada à grande quantidade de células imunocompetentes, torna os pulmões importante alvo de vasculites imunomediadas. O envolvimento destes vasos sanguíneos pode provocar qualquer sintoma respiratório, dependendo do tipo, local e extensão da lesão no sistema vascular respiratório. Algumas situações ameaçadoras da vida podem resultar, abrindo ou acompanhando o quadro destas patologias e, se não prontamente identificadas e tratadas, podem resultar em risco elevado de morbimortalidade. Destacamos duas destas condições especiais - estenoses de grande vias aéreas e hemorragia alveolar disusa - e descrevemos seus aspectos fisiopatológicos, clínicos e terapêuticos

The extensive pulmonary and bronchial vascular system, associated to large amount of immunocompetent cells, makes the respiratory system an important target of immune mediated vasculitis. These blood vessels involvement can cause any respiratory symptoms, depending on the type, location, and degree of lesion in the pulmonary vascular system. Some life-thereatening conditions may ensue, opening or following the course of those deseases and, if not identified and treated promptly, result in increased risk of morbitidy and mortality. We highlight two of those special situations: large airway stenosis and diffuse alveolar hemorrhage, describing their pathophysiological clinical and therapeutic aspects

Avaliação radiológica das vasculites pulmonares / Radiological evaluation of pulmonary vasculitis

A vasculite pulmonar corresponde a um grupo heterogêneo de doenças e de diagnóstico difícil. Esta revisão aborda o papel da radiologia na avaliação dessas entidades. Os achados de imagem são variados e pouco específicos na vasculite pulmonar, mas uma análise padronizada das alterações radiológicas pode ajudar a estreitar o diagnóstico diferencial. Neste artigo, uma análise radiológica baseada no tamanho dos vasos acometidos é utilizada para classificar as vasculites primárias. A hemorragia alveolar difusa é uma das manifestações das vasculites pulmonares primárias, além de várias doenças, e também é revisada neste artigo. A integração das características clínicas, laboratoriais e radiológicas é importante para uma diagnóstico mais específico de várias formas de vasculite pulmonar

The pulmonary vasculitides encompass a heterogenous group of diseases and are usually hard to diagnose. This review addresses the role of imaging in the investigation of pulmonary vasculitis. Imaging are variable and poorly specific in pulmonary vasculitis, but a pattern approach based on radiologic findings may help to refine or narrow the diferential diagnosis. In this article, an approach based on the size of the affected vessels in used to classify the primary vasculitides. Diffuse alveolar hemorrhage is one of the manifestations of primary pulmonary vasculitis, among other entities, and is also revisited in this review. Integration of clinical, laboratory, and imaging findings is important for making a specific diagnosis of pulmonary vasculitis

Vasculites pulmonares para o pneumologista: uma "cartilha introdutória" / Pulmonary vasculitis for pulmonologists: a 'introductory primer

Vasculites pulmonares são doenças raras, mas com grande possibilidade de produzir morbimortalidade nos indivíduos acometidos, não somente pela sua potencial gravidade multisistêmica, mas também por seu diagnóstico geralmente tardio. A identificação de padrões de doença que remetem a estes diagnósticos e a utilização de novos meios de investigação - incluindo ANCA (anticorpo antineutrofílico citoplasmático), reconhecendo suas vantagens e limitações - são importantes, se quisermos utilizar o moderno arsenal terapêutico da forma adequada e com sucesso. Atenção às resultantes clínicas é fundamental no acompanhamento de longo curso destes pacientes

Pulmonary vasculitis are rare illnesses, although possessing substantial morbi-mortality in affected individuals, not only due to its multisystemic nature, but also because of commonly delayed diagnosis. Disease pattern recognition that remind us of that group of diseases, and the use of modern investigation methods - including ANCA (anti-neutrophil cytoplasmic antibodies), with the knowledge of its pros and cons, are key features if the state of the art therapeutic modalities are to be properly used with success. Attention to clinical outcomes is fundamental in the long range management of these patients

Diagnóstico diferencial de nódulos pulmonares cavitados: a propósito de un caso clínico / Differential diagnosis of cavitary pulmonary nodules: a propos of a clinical case

El diagnóstico diferencial de nódulos pulmonares cavitados constituye un gran desafo en el cual, la clínica, la imagenología y los exámenes de laboratorio permiten una orientación etiológica en cutro grandes grupos: infecciosa, neoplásica, reumatológica y miscelánea. Presentamos el caso de una paciente gran fumadora y portadora de una diabetes mellitus descompensada, con nódulos pulmonares cavitados, en la cual el contexto clínico obligaba a plantear ciertas etiologías como las infecciosas y neoplásicas, pero cuya biopsia fue compatible con Granulomatosis con Poliangeítis (ex Granulomatosis de Wegener), sin una clínica ni exámenes de laboratorio concordantes con dicho hallazgo.

The differential diagnosis of cavitary pulmonary nodules is a great challenge, where the clinical context in addition to the image studies and laboratory tests are part of the key tools to guide the diagnostic process through 4 major etiological groups: infectious, neoplastic, rheumatologic and miscellaneous. We describe a case of a heavy smoker and complicated diabetic patient with cavitary pulmonary nodules, with a clinical context that induces certain etiologies such as infectious and neoplastic, but whose biopsy was compatible with Granulomatosis with polyangiitis (ex Wegener 's granulomatosis), without a clinical exam nor laboratory tests suggesting this finding.

A propósito de un caso de urticaria vasculítica intermedia en un paciente pediátrico / A propos of a case of intermediate vasculitic urticaria in a pediatric patient

Se presenta un paciente de sexo masculino, de 2 años y siete meses de edad, con cuadro clínico compatible con urticaria vasculítica. La biopsia cutánea evidenció una vasculitis con infiltrado mononuclear. A diferencia del cuadro típico de urticaria vasculitis, en este paciente en la histopatología no se evidenció vasculitis leucicitoclástica, hecho que nos permite categorizarla como una forma intermedia de urticaria vasculitis.

We report a male patient, 2 years and seven months old, with clinical symptoms compatible with vasculitic urticaria. A skin biopsy showed vasculitis with mononuclear infiltrate. Unlike the typical picture of urticaria vasculitis in this patient, the histopathology did not reveal vasculitis leucicitoclástica, allowing us to categorize it as an intermediate form of urticaria vasculitis.

Edema hemorrágico agudo del lactante: reporte de un caso de presentación atípica / Acute hemorrhagic edema of infancy: case report

Acute infant hemorragic edema is a benign condition that affects children between 4 month and 2 years of age. Quick onset inflammatory edema and generalized ring shaped symmetric purpura are characteristic. Histology is of leucocitoclastic vasculitis. Clinical case: 9 month old child with a respiratory tract affection 2 weeks previous, consulting for left leg edema and fever, afterwards cutaneous and enteral mucosa purpura. Discussion: Acute hemorrhagic edema is a benign and self-limited illness. Lhe main differential diagnosis is Schonlein Henoch Purpura, and a careful evaluation and clinical follow-up is neccesary.

Introducción: El Edema Hemorrágico Agudo del Lactante (EHAL) es una condición benigna que afecta a niños entre 4 meses y 2 años, de inicio brusco, con edema inflamatorio y lesiones cutáneas del tipo purpúrico, anulares generalizadas y simétricas. A la histología los hallazgos son compatibles con una vasculitis leucocitoclástica. Caso clínico: Lactante de 9 meses de edad, que cursó con cuadro respiratorio 2 semanas previas y que consulta por aumento de volumen de la extremidad inferior izquierda y fiebre, agregándose en forma progresiva lesiones cutáneas purpúricas, compromiso de mucosas y gastrointestinal. Discusión: El EHAL es una condición benigna y autolimitada, cuyo principal diagnóstico diferencial es Púrpura de Schonlein-Henoch, la cual requiere una cuidadosa evaluación y segumiento clínico.