Urticarial Vasculitis.

Urticarial vasculitis is a rare autoimmune disorder characterized by persistent edematous papules and plaques on the skin that last longer than 24 hours, often accompanied by systemic symptoms such as joint pain and fever. Unlike common urticaria, this condition involves inflammation of small blood vessels, leading to more severe and long-lasting skin lesions with a tendency to leave a bruiselike appearance. Diagnosis is challenging and may require a skin biopsy. Associated with underlying autoimmune diseases, treatment involves managing symptoms with medications such as antihistamines and corticosteroids, addressing the immune system's dysfunction, and treating any concurrent autoimmune conditions.

Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study.

OBJECTIVE: Single-organ cutaneous small-vessel vasculitis (SoCSVV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. Pathogenetically, idiopathic forms are described, as well as the induction from different triggers, such as infections, drugs, and vaccines. Following the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic outbreak, cases of cutaneous vasculitis induced by both COVID-19 and COVID-19 vaccinations have been reported in literature. The aim of this study is to provide the most recent evidence on new etiological factors, clinical features, and management of the SoCSVV. PATIENTS AND METHODS: We included 42 patients (22 women, 20 men) with SoCSVV and no systemic involvement in the study. The mean age of the patients was 57.3 years. Palpable purpura was the most frequent clinical manifestation (38 cases-90.4%). All patients were diagnosed with leukocytoclastic vasculitis by skin biopsy. RESULTS: The etiological factors were as follows: idiopathic in 9 (21%) patients, drug-related in 19 (45%) patients, COVID-19 infection-related in 5 (12%) patients, post-COVID-19 vaccination in 5 (12%) patients, paraneoplastic in 2 (5%) patients, and drug and infection and sepsis in 1 patient each. Among the drug-related cases, 16 (84%) were antibiotic-related, and most of them were beta-lactam antibiotics. Eosinophilia was present in skin biopsy in the cases related to vaccination and drugs, while intense necrosis and vascular damage in the skin were observed in the cases related to COVID-19 infection, unlike the others. A rapid resolution was observed with the cessation of drugs and short-term steroid treatment for the precipitating factors. CONCLUSIONS: SoCSVV is usually associated with drugs, preceding infections, and vaccines. COVID-19 infection and COVID-19 vaccinations have been reported as new etiological factors. SoCSVV indicates that the disease seems to be a mild, self-limiting illness with a good clinical result.

Cuadros cutáneo-mucosos asociados al consumo de cocaína / Cutaneous and Mucosal Conditions Associated With Cocaine Use

El consumo de cocaína, junto con algunos de sus adulterantes más frecuentes como el levamisol, puede provocar múltiples procesos cutáneos y mucosos, ya sean de índole isquémico, dermatosis neutrofílicas, lesiones destructivas de la línea media y vasculitis asociadas a ANCA, entre otros. Generalmente no se asocia clínica sistémica llamativa.Todos estos cuadros pueden presentar anticuerpos antinucleares, antifosfolípido y contra distintos antígenos de los neutrófilos, en ocasiones con un patrón característico. El estudio histológico suele mostrar cambios vasculares como vasculitis leucocitoclástica, necrosis de la pared y trombos. En este artículo revisamos las características clínicas, serológicas e histológicas de estas entidades, junto con los mecanismos fisiopatológicos implicados, el diagnóstico diferencial y su tratamiento. (AU)

Cocaine and some of its main adulterants, such as levamisole, can cause multiple cutaneous and mucosal manifestations, including ischemic complications, neutrophilic dermatoses, midline destructive lesions, and vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). Striking systemic symptoms are generally not seen.In all these conditions, positive test results may be observed for antinuclear antibodies, antiphospholipid antibodies, and various ANCAs, sometimes with characteristic staining patterns. Histology typically shows vascular changes, such as leukocytoclastic vasculitis, necrotizing vasculitis, and thrombi. We review the clinical, serologic, and histologic features of cutaneous and mucosal conditions associated with the use of cocaine and also look at pathophysiologic mechanisms, differential diagnoses, and treatments. (AU)

[Translated article] Cutaneous and Mucosal Conditions Associated With Cocaine Use / Cuadros cutáneo-mucosos asociados al consumo de cocaína

Cocaine and some of its main adulterants, such as levamisole, can cause multiple cutaneous and mucosal manifestations, including ischemic complications, neutrophilic dermatoses, midline destructive lesions, and vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). Striking systemic symptoms are generally not seen. In all these conditions, positive test results may be observed for antinuclear antibodies, antiphospholipid antibodies, and various ANCAs, sometimes with characteristic staining patterns. Histology typically shows vascular changes, such as leukocytoclastic vasculitis, necrotizing vasculitis, and thrombi. We review the clinical, serologic, and histologic features of cutaneous and mucosal conditions associated with the use of cocaine and also look at pathophysiologic mechanisms, differential diagnoses, and treatments. (AU)

El consumo de cocaína, junto con algunos de sus adulterantes más frecuentes como el levamisol, puede provocar múltiples procesos cutáneos y mucosos, ya sean de índole isquémico, dermatosis neutrofílicas, lesiones destructivas de la línea media y vasculitis asociadas a ANCA, entre otros. Generalmente no se asocia clínica sistémica llamativa. Todos estos cuadros pueden presentar anticuerpos antinucleares, antifosfolípido y contra distintos antígenos de los neutrófilos, en ocasiones con un patrón característico. El estudio histológico suele mostrar cambios vasculares, como vasculitis leucocitoclástica, necrosis de la pared y trombos. En este artículo revisamos las características clínicas, serológicas e histológicas de estas entidades, junto con los mecanismos fisiopatológicos implicados, el diagnóstico diferencial y su tratamiento. (AU)

Vasculitis tras la monitorización de presión arterial / Vasculitis after blood pressure monitoring

Fundamento. Descripción de aparición de una equimosis en brazo simultáneamente con vasculitis de origen leucocitoclástica, propuesta de usos alternativos a la toma de presión arterial, y el estudio de efectos secundarios a dicha medida. Paciente. Varón de 80 años acude a urgencias con disnea, insuficiencia cardíaca, insuficiencia renal prediálisis con hiperpotasemia e inestabilidad hemodinámica. Durante su estancia desarrolló una lesión cutánea con aspecto de púrpura palpable, desde el límite inferior del manguito de presión arterial hasta la zona distal de la mano y que no desaparecía a la vitropresión, y prurito. Durante su ingreso, la lesión del brazo se extendió a todos los miembros, tanto superiores como inferiores. Resultados. El estudio de la lesión concluyó con diagnóstico de vasculitis leucocitoclástica por presencia de eosinófilos, lo que sugiere probable etiología medicamentosa a la toma de antibiótico desde 7 días antes de la entrada a urgencias. Discusión. Se plantea la necesidad de monitorización seriada de la presión arterial y la duración de dicha monitorización en pacientes inestables frente a los efectos secundarios de esta técnica. También el estudio de otras utilidades de la toma de presión arterial (AU)

Introduction. Description of appearance of ecchymosis on an arm, simultaneously with a classical Leukocytoclastic Vasculitis, the proposal of alternative utilities of measuring blood pressure, and the study of side effects to that measure. Patient. Case 80-year-old male came to ER with dyspnea, heart failure, predialysis renal failure with hyperkalemia and hemodynamic instability. During his stay he developed a skin lesion that looks like palpable purpura, from the lower limit of the blood pressure cuff to the distal area of the hand that not disappeared with vitropression, and pruritus. During admission the arm injury was extended to all members, both upper and lower. Results. The study concluded with diagnosis of Leukocytoclastic Vasculitis given the presence of eosinophils, that which suggested probable drug etiology to an antibiotic that had been taken since seven days prior to admission to ER. Discusion. The need for serial monitoring of blood pressure, and the duration of such monitoring in unstable patients considering the side effects of those techniques was questioned. In addition, the study of other utilities of measuring blood pressure (AU)

Vasculitis del sábado noche / Saturday night vasculitis

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Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia / Lesoes cutaneas e baqueteamento digital reveladores de vasculite urticariforme hipocomplementemica e hepatite C com crioglobulinemia mista

Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

A vasculite urticariforme é uma entidade clinico-patológica rara caracterizada por lesões urticariformes com duração superior a 24 horas e uma vasculite leucocitoclásica na histologia. É dividida em normo e hipocomplementêmica. Os autores relatam o caso de uma mulher saudável de 49 anos, com lesões cutâneas intensamente pruriginosas e baqueteamento digital com 1 ano de evolução. O estudo efectuado permitiu efectuar os diagnósticos de vasculite urticariforme hipocomplementêmica, hepatite C crônica e crioglobulinêmia mista tipo II. A doente iniciou tratamento sintomático e foi referenciada para a Gastroenterologia para orientação da hepatite, com melhoria progressiva das lesões cutâneas. O desenvolvimento de vasculite urticariforme hipocomplementêmica no contexto de hepatite C crónica é raro e os possíveis mecanismos patogênicos são discutidos.

Vasculitis cutánea leucocitoclástica y glomeruesclerosis focal y segmentaria con formación de semi lunas en una joven consumidora de cocaína/crack cortada con levamisol V / Cutaneous leukocytoclastic vasculitis and focal segmental glomeruesclerosis with crescent formation in a young consumer of cocaine / crack cut with levamisole V

Se describen los hallazgos anatomopatológicos encontrados en la autopsia de una joven de 19 años de edad, indigente, trabajadora del sexo, adicta al crack desde los 12 años de edad, quien los últimos 4 meses de su vida tuvo tres ingresos hospitalarios al Hospital México de San José‚ de Costa Rica con diagnóstico de vasculitis cutánea por crack e insuficiencia renal aguda. Los hallazgos más relevantes en la autopsia fueron: vasculitis aguda leucocitoclástica con trombosis y paniculitis glomeruloesclerosis focal y segmentaria con formación de semilunas...

Edema agudo hemorrágico da infância: relato de três casos / Acute hemorrhagic edema of infancy: report of three cases

O Edema Agudo Hemorrágico da Infância é uma vasculite leucocitoclástica pouco frequente, que ocorre, quase exclusivamente, em crianças entre 4 meses e 2 anos de idade. Caracteriza-se, clinicamente, pela tríade febre, lesões purpúricas na face, pavilhões auriculares e extremidades e edema. Embora os achados cutâneos sejam dramáticos e de surgimento rápido, o prognóstico é favorável, com resolução espontânea dentro de 1 a 3 semanas. Descrevem-se três casos cujos achados clínicos e histopatológicos são característicos de edema agudo hemorrágico da infância.

Acute Hemorrhagic Edema of Infancy is an infrequent leukocytoclastic vasculitis which occurs almost exclusively in children between 4 months and 2 years of age. It is clinically characterized by the triad fever, purpuric lesions on the face, auricular pinna and extremities, and edema. Although the cutaneous findings are dramatic and of rapid onset, the prognosis is favorable, with spontaneous resolution within 1 to 3 weeks. Three cases are described in which clinical and histopathological findings are characteristic of acute hemorrhagic edema of infancy.

Vasculite leucocitoclástica cutânea associada à tuberculose pulmonar / Cutaneous leukocytoclastic vasculitis accompanied by pulmonary tuberculosis

Relatamos o caso de um homem de 50 anos com uma rara associação: tuberculose pulmonar e vasculite leucocitoclástica cutânea. O paciente procurou o pronto atendimento em razão do quadro de cefaléia, artralgia, rash cutâneo e perda ponderal (4 kg) nos últimos 20 dias. A radiografia de tórax, solicitada em consulta ambulatorial prévia, demonstrava cavitação nos lobos médio e superior do pulmão direito, confirmada por tomografia computadorizada. Apresentou baciloscopia de escarro (coloração de Ziehl-Neelsen) positiva em três amostras consecutivas e biópsia da lesão de pele compatível com vasculite leucocitoclástica cutânea. Foi, então, realizado o diagnóstico de vaculite leucocitoclástica cutânea associada à tuberculose pulmonar. Nosso objetivo é descrever uma associação pouco relatada na literatura médica e discutir seus possíveis mecanismos patogênicos.

We report the case of a 50-year-old male with a rare combination: pulmonary tuberculosis and cutaneous leukocytoclastic vasculitis. The patient sought emergency treatment presenting with headache, arthralgia, cutaneous rash, and weight loss (4 kg) in the last 20 days. A chest X-ray, performed in a previous outpatient visit, revealed cavitation in the middle and upper lobes of the right lung, as confirmed by computed tomography. Sputum smear microscopy (Ziehl-Neelsen staining) was positive in three consecutive samples, and the result of the skin lesion biopsy was consistent with cutaneous leukocytoclastic vasculitis. The patient was therefore diagnosed with cutaneous leukocytoclastic vasculitis accompanied by pulmonary tuberculosis. Our objective was to describe a combination rarely reported in the medical literature and to discuss the possible pathogenic mechanisms of this combination.

Vasculitis de vaso pequeño asociada a un producto oral usado para la caída del pelo / Small vessels vasculitis associated with a oral medication for loss hair

Se presenta el caso de una mujer de 36 años que consultó por lesiones vasculíticas en extremidades inferiores que aparecieron con relación al uso de un producto oral comercializado para la caída del pelo que contiene prosilium, vitaminas B6, B5, B8, hierro, zinc y vitamina E. Algunos elementos evolucionaron hacia la necrosis, especialmente en la pierna derecha. Los análisis de laboratorio permitieron excluir otras patologías asociadas con vasculitis, diagnosticándose una vasculitis cutánea de vaso pequeño posiblemente asociado a alguno de los componentes de dicho producto.

A 36 year old woman went for a consultation because of vasculitic lesions in her lower extremities that appeared after the use of a commercial oral product for hair loss which contains prosilium, vitamin B6, B5, B8, E, iron, and zinc. Some elements evolved towards necrosis, especially in the right leg. Laboratory analysis excluded other causes of vasculitis. The final diagnosis was a cutaneous vasculitis possible associated with some of the components of the hair loss product.

Erythema elevatum diutinum as a first clinical manifestation for diagnosing HIV infection: case history

CONTEXTO: O eritema elevatum diutinum é uma dermatose crônica, rara, variante clínica da vasculite leucocitoclástica, provavelmente mediada por imunocomplexos. Está sendo incluído no grupo das dermatoses específicas associadas à infecção pelo HIV. Em geral, associa-se a outros processos infecciosos, auto-imunes e neoplásicos. RELATO DE CASO: Relatamos o caso de um paciente em que a manifestação cutânea de eritema elevatum diutinum foi a primeira evidência clínica para diagnóstico da infecção pelo HIV. O tratamento foi feito com dapsona e se obteve regressão parcial das lesões após 15 dias, mesmo antes de o esquema anti-retroviral ser prescrito. CONCLUSÃO: Frente ao diagnóstico de eritema elevatum diutinum, deve-se investigar a infecção pelo HIV, principalmente nas apresentações clínicas atípicas e exacerbadas.

Vasculite leucocitoclástica: associação com infecção pelo vírus da hepatite C / Leucocitoclastic vasculitis: association with hepatitis C virus infection

A vasculite leucocitoclástica pode estar associada a infecções, drogas ou neoplasias malignas. Os autores relatam o caso clínico de um paciente que apresentou quadro de lesões petequiais e púrpura palpável em membros inferiores, sendo diagnosticado histologicamente como vasculite leucocitoclástica. Na investigação etiológica foi evidenciada infecção pelo vírus da hepatite C, já com hepatopatia crônica (AU)

Leucocitoclastic Vasculitis may be associated with infections, drugs, or malignant neoplasms. Here the authors report the case of a patient with palpable, petechial purple lesions in the lower limbs, which were histologically diagnosed as leucocitoclastic vasculitis. An etiological investigation evidenced hepatitis c virus infection with chronic hepatopathy (AU)

Vasculopatía séptica / Septic Vasculopathy

La sepsis es una de las principales causas de mortalidad en todo el mundo. Se desconoce la frecuencia con la que aparecen lesiones cutáneas en el contexto de una sepsis, pero en la mayoría de los casos constituyen una de las manifestaciones iniciales, que permitirán un diagnóstico precoz, de esta grave enfermedad que puede comprometer la vida. Se han descrito 4 mecanismos fisiopatológicos fundamentales por los que se producen lesiones cutáneas en el contexto de una sepsis: a) coagulación intravascular diseminada; b) invasión directa del microorganismo de la pared vascular; c) vasculitis de mecanismo inmune y d) embolismo séptico. Se sabe que estos mecanismos no son excluyentes y en un mismo paciente se pueden solapar varios de ellos. En esta revisión analizaremos cada uno de estos mecanismos, las manifestaciones clínicas que inducen y los hallazgos anatomopatológicos que se pueden encontrar en la biopsia cutánea (AU)

Sepsis is one of the commonest causes of death around the world. The real frequency of cutaneous lesions in the setting of sepsis is unknown, but when they appear, they are usually one of the earliest signs of sepsis, thus allowing a rapid diagnosis of this potentially life-threatening condition. Four are the main physiopathologic mechanisms that can induce cutaneous lesions in sepsis: a) disseminated intravascular coagulation; b) direct vessel wall invasion by the microorganism; c) immune-mediated vasculitis, and d) septic embolism. We know that more than one of these mechanisms can appear in one single patient. In this review, we analyse these four mechanisms, their clinical presentation, and the histological findings that can be found in the cutaneous biopsy (AU)

Edema hemorrágico agudo del lactante: reporte de dos casos / Acute infantile hemorrhagic edema: two case report

El Edema Hemorrágico Agudo del Lactante (EHAL) es una vasculitis leucocitoclásica de curso benigno, generalmente sin compromiso sistémico, que se presenta entre los 4 y 24 meses de edad. Clínicamente se caracteriza por la súbita aparición de placas o pápulas purpúricas, edematosas, que forman lesiones en escarapela, localizadas principalmente en mejillas, pabellones auriculares y porción distal de extremidades. Su patogenia es desconocida: se postula que el mecanismo sería mediado por inmunocomplejos, probablemente precipitado por infecciones virales, ingesta de medicamentos y vacunas recientes. En la histopatología se encuentra una vasculitis leucocitoclástica. Algunos plantean que se trataría de una variante del púrpura de Schõnlein-Henoch. El cuadro se resuelve espontáneamente en una a tres semanas, por lo que no requiere tratamiento específico. Se presentan dos casos clínicos característicos, destacando en uno de ellos la presencia de PCR positiva para parvovirus B19, cuya asociación es relativamente infrecuente.

Vasculitis during immunotherapy treatment in a patient with allergy to Cupressus arizonica

Allergen immunotherapy dates back to 1911 and has been used successfully to treat large numbers of patients throughout the last century. Case report: a 66-year-old woman presented with symptoms of allergic rhinitis and asthma due to sensitization to Cupressus arizonica. Specific immunotherapy was prescribed as a continuous 2-year treatment with a depot preparation of standarized and characterized allergen extracts of Cupressus arizonica pollen. Forty-eight hours after one maintenance dose of 0.8 cc, the patient presented palpable violaceous purpuric lesions and pruritus on both legs. We performed skin prick and intradermal tests with Cupressus arizonica. Twenty-four hours later, the 1/1 dilution intradermal skin test was positive. Biopsy showed leukocytoclastic vasculitis. Conclusions: A middle-aged woman experienced cutaneous non-necrotizing vasculitis after 2 years of maintenance immunotherapy. The interval between injections and the first appearance of cutaneous lesions suggests a type III hypersensitivity immune reaction. Skin biopsy of the positive intradermal test also supports this hypothesis

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Vasculitis necrotisante a medicamentos: Hospital Dr. Dario Contreras / Necrotizing vasculitis due to drugs: Hospital Dr Dario Contreras

Se estudiaron dos casos de niños internados por referimientos, en el Hospital Dr. Dario Contreras, con los diagnósticos de Meningitis Bacteriana y Cangrena Seca en ambas piernas. Estos niños llegaron de lugares diferentes, pero con igual historia de administración de medicamentos diferentes en cortos períodos de tiempo en cada uno de ellos. El primero llegó desde el Hospital Materno Infantil San Lorenzo de Los Minas, y el segundo desde el Hospital de San Juan de la Maguana, ambos pacientes antes de ser ingresados fueron medicados por sus madres, con antipiréticos y antipalúdidos, y llevados a los centros donde fueron tratados y luego referidos. Durante su ingreso en esos centros hospitalarios les fueron hechos varios estudios entre ellos de orina, hemáticos, L.C.R., etc. y les administraron analgésicos, antipiréticos y antibióticos sin lograr la mejoría deseada por lo que fueron referidos con fines quirúrgicos. El primer caso nos llegó referido en el mes de septiembre de 1984, en tan malas condiciones que aunque fue tratado quirúrgicamente no sobrevivió, porque presentó una septisemia tipo fulminante dos días después de la cirugía. No se obtuvo en estos casos el mismo resultado, ya que la administración de los medicamentos específicos dependía del resultado de laboratorio y de la biopsia, cultivos y antibiogramas y de la rapidez con que ellos fueran reportados

Vasculitis linfomonocitica: informe de nueve casos / Lymphomonocitic vasculitis: report of 9 cases

Las vasculitis cutaneas constituyen un grupo heterogeneo de cuadros clinicos etiologicamente inespecificos, la mayoria sin manifestaciones sistemicas. En los ultimos tres anos, nuestro protocolo de estudio sobre vasculitis cutanea se ha basado en el estudio del tipo de celula comprometida. Para ello estudiamos prospectivamente cincuenta biopsias de piel y encontramos nueve pacientes con inflitrado de tipo mononuclear donde predominan los linfocitos y monocitos a nivel de dermis papilar y reticular. Despues de estudiarlos por mas de dos anos, no hemps encontrado asociacion con enfermedades del tejido conjuntivo, linfoma de celulas T, parapsoriasis, granuloma anular, papulosis linfomatoide, reticulosis pagetoide, reaccion toxica o erupcion polimorfa a la luz. Encontramos la vasculitis monolinfocitica asociada a medicamentos en cuatro pacientes, y a urticaria cronica en tres, de los cuales dos tenian infeccion urinaria y uno bursitis septica. Ademas, este tipo de vasculitis se puede asociar a enfermedades del tejido conjuntivo, por lo que pensamos que sea un sindrome y no simplemente un proceso reactivo de la piel a multiples antigenos. Es posible que para la expresion de los dos subtipos de la enfermedad vascular inflamatoria, el antigeno desencadene un trastorno en la inmunorregulacion en aquellos individuos con cierta susceptibilidad genetica.