Vasculitis por IgA del adulto. Caso clínico

La vasculitis por IgA, es la vasculitis más frecuente en pediatría. Puede presentarse en adultos, con una clínica y evolución diferente y un pronóstico más grave que en los niños, incluida la progresión a enfermedad renal terminal. La historia natural de la enfermedad y de la nefritis, ha sido poco estudiada en adultos; no se dispone de criterios diagnósticos universalmente aceptados y el tratamiento es controvertido, dada la ausencia de estudios controlados, randomizados que lo avalen. Se reporta el caso de un paciente que presentó un síndrome purpúrico petequial, microhematuria, proteinuria y una evolución rápida a la insuficiencia renal, de cuyo estudio etiológico surge el diagnóstico de vasculitis por IgA del adulto.

The IgA vasculitis is the most common vasculitis in Pediatrics. It can also present in adults but with a different clinical course and a worse prognosis, including the possibility of progression to end stage renal disease. The natural history of the disease and its nephritis have been scarcely studied in adults. There is no universal agreement in diagnostic criteria and the treatment is controversial given the absence of controlled randomized trials. We report the case of a patient who presented clinically with a petechial purpuric rash, microhematuria, proteinuria and rapid progression to renal failure that was diagnosed with IgA vasculitis in adult.

A vasculite por IgA é a vasculite mais comum em pediatria. Pode ocorrer em adultos, com apresentação e evolução clínica diferentes e prognóstico mais grave do que em crianças, incluindo progressão para doença renal terminal. A história natural da doença e da nefrite tem sido pouco estudada em adultos; Não existem critérios diagnósticos universalmente aceitos e o tratamento é controverso, dada a ausência de estudos controlados e randomizados que o apoiem. É relatado o caso de um paciente que apresentou síndrome purpúrica petequial, microhematúria, proteinúria e rápida evolução para insuficiência renal, de cujo estudo etiológico surge o diagnóstico de vasculite por IgA do adulto.

Vasculite associada à IgA em criança: relato de caso / IgA vasculitis in a child: case report

A Vasculite associada à imunoglobulina A (VIgA), também conhecida como púrpura de Henoch-Schonlein, púrpura anafilactóide ou púrpura reumática é uma vasculite de pequenos vasos associada a deposição de imunocomplexos IgA, de etiologia ainda desconhecida e que acomete principalmente crianças. Em grande parte dos casos pediátricos, é uma doença autolimitada com manifestações cutâneas, articulares, gastrintestinais e renais. O diagnóstico diferencial inclui outras vasculites, como lúpus eritematoso sistêmico, meningococcemia, coagulação intravascular disseminada e síndrome hemolítica urêmica. Neste artigo abordam-se os principais aspectos da VIgA nas crianças, salientando-se a importância do diagnóstico diferencial precoce. É apresentado o caso clínico de uma paciente do sexo feminino de 5 anos com lesões purpúricas tratada numa primeira abordagem como infecção bacteriana grave. Após reavaliação médica houve alteração terapêutica com uso de glicocorticóides resultando em melhora expressiva dos sintomas. [au]

Vasculitis associated with immunoglobulin A (VIgA), also known as Henoch-Schonlein purpura, anaphylactoid purpura or rheumatic purpura is a small vessel vasculitis associated with deposition of IgA immune complexes, of unknown etiology and affecting mainly children. In most pediatric cases, it is a self-limited disease with cutaneous, joint, gastrointestinal and renal manifestations. The differential diagnosis includes other vasculitis, such as systemic lupus erythematosus, meningococcemia, disseminated intravascular coagulation and uremic hemolytic syndrome. In this article, the main aspects of HSP in children are addressed, highlighting the importance of early differential diagnosis. The clinical case of a 5-year-old female patient with purpuric lesions treated in a first approach as a severe bacterial infection is presented. After medical re-evaluation, there was a therapeutic change with the use of glucocorticoids resulting in a significant improvement of symptoms. [au]

IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship? / Vasculite por IgA (púrpura de Henoch-Schönlein), nefrite e psoríase em uma criança: existe relação?

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

Resumo Histórico A psoríase é uma doença crônica imunomediada que afeta principalmente a pele tanto em adultos quanto em crianças, mas também pode ter envolvimento sistêmico, particularmente com artrite e lesão renal. A nefropatia por IgA é o distúrbio renal mais frequentemente associado à psoríase. Aproximadamente um terço de todos os casos de psoríase começam na infância, mas a associação entre psoríase e distúrbios renais tem sido pouco relatada em pacientes pediátricos. A Púrpura de Henoch-Schönlein (PHS) é uma vasculite sistêmica caracterizada por depósitos de IgA nas paredes dos vasos de órgãos afetados e no mesângio do rim. A histopatologia da nefrite da PHS é idêntica à da nefropatia por IgA. Relato de caso Um menino de 6 anos de idade com início recente de psoríase desenvolveu PHS com envolvimento renal, clinicamente manifestado por proteinúria nefrótica e hematúria. A biópsia renal revelou crescentes fibrocelulares glomerulares e depósitos mesangiais de IgA compatíveis com a nefropatia por IgA. O tratamento com corticosteróides sistêmicos levou ao controle da hematúria, mas como a proteinúria nefrótica persistiu, a ciclofosfamida foi adicionada, levando a uma diminuição gradual da proteinúria. Conclusões Propomos um mecanismo comum subjacente na patogênese tanto da PHS quanto da psoríase, envolvendo uma desregulação da resposta imune mediada por IgA, que poderia predispor a ambas as entidades, bem como a danos renais e nefropatia por IgA nesses pacientes.

Función predictiva del índice de neutrófilos/linfocitos y del volumen plaquetario medio en la púrpura de Henoch-Schönlein relacionada con compromiso renal y gastrointestinal / Predictive role of neutrophil to lymphocyte ratio and mean platelet volume in Henoch-Schönlein purpura related gastrointestinal and renal involvement

Antecedentes: La púrpura de Henoch-Schönlein (PHS) es una vasculitis sistémica de vasos pequeños. El objetivo fue evaluar el índice de neutrófilos/linfocitos (INL) en sangre y el volumen plaquetario medio (VPM) en la PHS e investigar la relación con el compromiso renal y gastrointestinal.Métodos: Se incluyeron niños con PHS y controles sanos. Se evaluaron concentración de hemoglobina, recuento de leucocitos, recuento de trombocitos, INL, VPM, velocidad de sedimentación globular y proteína C-reactiva.Resultados: El INL fue significativamente mayor en los pacientes con PHS con hemorragia gastrointestinal (p < 0,001). El valor ideal de corte del INL para predecir la hemorragia gastrointestinal fue 2,05, con 93 % de sensibilidad y 62 % de especificidad. El VPM fue significativamente mayor en los pacientes con PHS con compromiso renal (p = 0,027).Conclusiones: El INL en sangre y el VPM podrían ser útiles para identificar el compromiso renal y gastrointestinal en la PHS

Background: Henoch-Schönlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. The aim was to evaluate the blood neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) in patients with HSP and to investigate the relationship with gastrointestinal and renal involvement.Methods: Children with HSP and healthy individuals as controls were included. Hemoglobin level, white blood cell count, platelet count, NLR, MPV erythrocyte sedimentation rate and C-reactive protein were evaluated.Results: There were 71 HSP children and 74 controls. NLR was significantly higher in HSP patients with gastrointestinal bleeding than without gastrointestinal bleeding (p < 0,001). The optimal cutoff value of NLR for predicting gastrointestinal bleeding was 2.05, with 93 % sensitivity and 62 % specificity. MPV was significantly higher in HSP patients with renal involvement than without renal involvement (p = 0,027).Conclusions:Blood NLR and MPV may be useful markers to identify gastrointestinal and renal involvement in HSP patients.

Púrpura de Henoch-Schönlein como manifestación inicial de mieloma múltiple / Henoch-Schonlein purpura as initial manifestation of multiple myeloma

Resumen La púrpura de Henoch-Schönlein como vasculitis paraneoplásica de tumores sólidos se encuentra en 9 a 11% de los casos reportados en adultos con carcinoma de estómago, mama, pulmón, próstata o riñón; es poco frecuente como paraneoplásico de mieloma múltiple. Se comunica el caso de una paciente que padeció púrpura de Henoch-Schönlein como manifestación inicial de mieloma múltiple.

Abstract Henoch-Schonlein purpura as paraneoplasic vasculitis of solid tumors is found in 9-11% of cases reported in adults with stomach, breast, lung, prostate and kidney carcinomas; it is little frequent as paraneoplastic of multiple myeloma. This paper reports the case of a patient that suffered from Henoch-Schonlein purpura as initial manifestation of multiple myeloma.

Púrpura de Henoch Schönlein en niños: casuistica del Hospital de Gandia, Valencia. España / Henoch Schonlein purpura in children: Casuistic from the Hospital of Gandia. Valencia. Spain

La Purpura de Henoch Schonlein (PHS) es la vasculitis mas frecuente en pediatría. Su análisis epidemiológico, clínico y evolución, son importantes en el manejo de los pacientes. Objetivo general: Revisar unas serie de pacientes con PHS de un hospital de referencia comarcal. Método: Se recogió información de las historias clínicas y se analizaron los datos de acuerdo a parámetros definidos. Resultados: 17 pacientes menores de 15 años fueron evaluados; esta cifra corresponde a 29 casos/año/100.000 habitantes <15años edad promedio 6.12±4.9 años; predominio del sexo masculino; la mayoría de los casos se presentó en otoño e invierno. Se detectó antecedente infeccioso previo o concomitante en 47% de los casos, predominantemente infecciones del tracto respiratorio superior y, la forma de presentación mas frecuente fue la tríada: purpura cutánea palpable, manifestaciones gastrointestinales y articulares. Se encontró que 53% de los pacientes presentaron manifestación nefrológica inicial, predominando hematuria y proteinuria. Todos los casos mantuvieron función de filtración renal normal. Estreptococo beta hemolítico fue aislado en un tercio de los casos. Todos los pacientes han evolucionado satisfactoriamente después de 11±7 meses de seguimiento ambulatorio. Conclusiones: La casuística de PHS en este hospital muestra que la incidencia es mayor al promedio reportado en la mayoría de las casuísticas internacionales y es mayor en otoño, distinto a lo habitualmente reportado. Esto sugiere la probable existencia de factores ambientales, microbiológicos, o de otra índole, no precisados, lo cual ameritaría estudios pertinentes en materia de salud publica.

Background: Henoch Schonlein Purpura (HSP) is the commonest vasculitis in children. To perform epidemiological analysis and to evaluate clinic features and results of follow up are important for the adequate management of patients. Objectives: To review epidemiologic characteristics of HSP in a series of patients attended in a referral hospital in Spain and to analyze clinical, laboratory results and evolution of patients. Methods: Data from the clinical records was collected and analyzed according to defined parameters. Results: 17 patients under 15 years of age were evaluated; it corresponds to 29 cases/year/100.000 IH <15 years. Mean age 6.12±4.9 years. The majority of cases presented in autumn and winter. Infectious antecedent or simultaneous infection with the onset was detected in 47% of cases mainly in upper respiratory tract. Clinical presentation was the classical triad of palpable cutaneous purpura, gastrointestinal and joints manifestations. Initial nephrologic manifestations were found in 53% of cases, predominantly hematuria and proteinuria, with normal glomerular filtration ratio. Beta hemolytic streptococcus was isolated in one third of cases. After ambulatory follow up of 11±7 months all patients have had uneventful recovery. Conclusions: this casuistic of PHS shows that the incidence in this geographic area is higher compared with the majority of international and national series and it is more frequent in autumn, which is different to the reported in other series. This suggests the probable presence of environmental, microbiologic or another features, no clearly known, which requires analysis from the public health point of view.

Prevalencia del compromiso renal durante el primer año de seguimiento en pacientes con púrpura de Henoch Schönlein en una institución pediátrica de Bogotá, Colombia / Prevalence of kidney involvement during the first year of follow-up in patients with Henoch-Schönlein purpura in a paediatric institution in Bogotá, Colombia

Resumen Introducción: La púrpura de Henoch-Schönlein es la vasculitis más frecuente en la infancia. La afectación renal continúa siendo la causa de morbimortalidad principal en niños. El espectro del compromiso renal va desde manifestaciones leves hasta el desarrollo de síndrome nefrótico o nefrítico, o insuficiencia renal. Objetivo: Determinar las características clínicas al comienzo y el compromiso renal de los pacientes con diagnóstico definitivo de púrpura de Henoch-Schönlein, durante el primer mes, a los 3 meses y al ario de seguimiento. Métodos: Estudio descriptivo, retrospectivo, de pacientes con diagnóstico definitivo de púrpura de Henoch-Schönlein, de la consulta de Reumatología Pediátrica, en una institución de Bogotá, Colombia, en el período comprendido de 2010 a 2016. Resultados: Ochenta y seis pacientes fueron incluidos en el estudio, 42 niñas y 44 niños, edad media de presentación 5,3 años, DE 2,4 años (1a14anos). Se presentó compromiso renal en 39 pacientes (45%). Se evidenció una tendencia mayor a compromiso renal entre los pacientes con artritis (p = 0,053). El hallazgo clínico más frecuente fue proteinuria aislada (49%), seguido de proteinuria/hematuria (28%) y hematuria aislada en el 15%. Había 8/39 pacientes con compromiso renal durante el año de seguimiento que fueron llevados a biopsia, 6 (75%) con compromiso renal al inicio y 25% al mes. El promedio de seguimiento fue de 26,8 meses con una desviación estándar de 17 meses (1 a 72 meses), no hubo disfunción renal a la última valoración en ninguno de los 39 pacientes con compromiso renal. Conclusión: El compromiso renal fue más frecuente en las primeras semanas de la enfermedad, así como en las formas severas.

Abstract Introduction: Henoch-Schönlein Purpura is the most common vasculitis in children, with Henoch-Schönlein Purpura kidney involvement remaining the main cause of morbidity and mortality. The spectrum of Kidney involvement of kidney involvement ranges from mild symptoms to the development of a nephrotic and/or nephritic syndrome or kidney failure. Objective: To determine the clinic features at onset, and kidney involvement of patients with a final diagnosis of Henoch-Schönlein Purpura during the first month, 3 months, and up to the first year of follow-up. Methods: A retrospective study conducted on patients with a final diagnosis of Henoch-Schönlein Purpura in a Paediatric Rheumatology Department in an institution of Bogota, Colombia, during the period between 2010 and 2016. Results: The study included 86 patients, 42 girls and 44 boys. The median age at disease onset was 5.3 years (SD 2.4 years: range1-14years). Kidney involvement was present in 39/86 (45%) patients. A trend to kidney involvement was observed in patients with abdominal symptoms (p=,053). The most frequent clinical finding was isolated proteinuria (49%), followed of proteinuria/haematuria (28%), and isolated hematuria (15%). Renal biopsy was performed on 8/39 patients with Henoch-Schönlein Purpura nephritis. The mean follow-up was 26.8 months (SD 17 months: range 1-72). There was no evidence of kidney damage in the last assessment in any of the 39 patients with kidney involvement. Conclusion: In this group of patients, kidney involvement was more severe and common in the first weeks of the disease onset.

Prevalencia y manifestaciones clínicas más frecuentes de la Púrpura de Henoch Schõnlein en la población pediátrica de la Clínica Universitaria Colombia y Clínica Reina Sofía entre los años 2009-2015 / Prevalence and most frequent clinical manifestations of the Henoch-Schõnlein Purpura in the pediatric population of the Clínica Universitaria Colombia and Clinica Reina Sofia between the years 2009-2015

Objetivo: estimar la prevalencia y determinar las manifestaciones clínicas más frecuentes de la Púrpura de Henoch-Schõnlein teniendo en cuenta los criterios EULAR-PRINTO-PReS en la población pediátrica que ingresó a la Clínica Universitaria Colombia y Clínica Reina Sofía a los servicios de urgencias y hospitalización entre los años 2009 al 2015. Método: estudio descriptivo, cohorte histórica, que se realizó en la ciudad de Bogotá, se incluyeron 109 pacientes menores de 18 años con diagnóstico de Púrpura de Henoch-Schõnlein. Resultados: Se encontró una prevalencia en el ámbito hospitalario de 2,07 casos por cada 1.000 hospitalizaciones y en el escenario de urgencias 0,2 por cada 1.000 consultas, respecto a las manifestaciones clínicas se comportó así: Purpura palpable (100 por ciento), artritis/artralgia (80 por ciento), Dolor abdominal (33 por ciento), nefropatía (6 por ciento), Depósitos IgA (0 por ciento). Conclusiones: En nuestro estudio, esta enfermedad fue mucho más prevalente en niñas, respecto a las manifestaciones clínicas de acuerdo a los criterios diagnósticos EULAR/PRINTO/Pres se comportó de manera similar a otros estudios.

Objective: estimate the prevalence and determine the most frequent clinical manifestations of Henoch-Schõnlein Purpura, taking into account the EULAR-PRINTO-PReS criteria in the pediatric population that entered the emergency and Hospitalization services in the Clinica Universitaria Colombia and Clínica Reina Sofía between the years 2009 and 2015. Method: descriptive study, historical cohort, carried out in the city of Bogotá, included 109 patients under the age of 18 years with diagnosis of Henoch-Schõnlein Purpura. Results: A prevalence of 2.07 cases per 1000 hospitalizations was found in the hospital setting and in the emergency setting 0.2 per 1000 visits, in relation to the clinical manifestations, it was: Palpable purpura (100percent), arthritis / arthralgia 80 percent, Abdominal pain (33 percent), nephropathy (6 percent), IgA deposits (0 percent). Conclusions: In our study, this disease was much more prevalent in girls, on the other hand in regard to the clinical manifestations according to diagnostic criteria of EULAR / PRINTO / Pres, our population behaved in a similar way to other studies.

Púrpura de Henoch-Schönlein con compromiso ocular en un adulto: Reporte de caso y revisión de la literatura / Henoch-Schönlein purpura with ocular manifestations in adult: Case report and review of the literature

La púrpura de Henoch Schönlein es una vasculitis sistémica que afecta vasos pequeños, predominantemente en niños. Los eventos trombóticos son una rara complicación, y en la literatura solo algunos casos se han reportado. Entre éstos, un caso de oclusión de arteria central de la retina bilateral y un caso de oclusión de vena central de la retina, ambos pacientes con diagnóstico de púrpura de Henoch Schönlein, el segundo dos semanas después a iniciar hemodiálisis. Describimos el caso de un paciente adulto con diagnóstico de púrpura de Henoch Schönlein e hipertensión arterial, en insuficiencia renal crónica estadio V, en hemodiálisis hacía 20 días, quien se presentó con disminución de agudeza visual de 15 días de evolución en ojo derecho. Los hallazgos al examen ocular fueron edema del disco, edema macular, tortuosidad vascular, hemorragias en cuatro cuadrantes, se consideró una oclusión de vena central de la retina. Requirió terapia antiangiogénica y fotocoagulación laser con buena evolución.

Henoch Schönlein purpura is a systemic vasculitis that affects small vessels and mainly aff ecting children. Thrombotic events are a rare complication, and only a few cases have been reported. Among these a case of bilateral central retinal arterial occlusion in patients with Henoch Schönlein purpura, and a case of central retinal vein occlusion in a patient with Henoch Schönlein purpura over a two weeks period of hemodialysis. We describe the case of an adult patient diagnosed with Henoch Schönlein purpura and arterial hypertension, chronic renal failure in hemodialysis for 20 days; who presents with decreased visual acuity for 15 days in the right eye. The findings were disc edema, macular edema, vascular tortuosity, haemorrhages in four quadrants. Central retinal vein occlusion was considered and antiangiogenic therapy and laser photocoagulation was performed with good response.

Púrpura de Henoch-Schõnlein em adulto / Henoch-Schõnlein purpura in adults

A púrpura de Henoch-Schõnlein é uma vasculite de pequenos vasos, mais comum em crianças, com acometimento cutâneo, articular, gastrointestinal e renal. É raro o acometimento em adultos, conferindo nestes pior prognóstico. Relatamos o caso de um paciente de 54 anos com púrpura de Henoch-Schõnlein com acometimento de pele, articulação, abdominal e renal, sem resposta clínica com altas doses de prednisona, mantendo quadro de claudicação abdominal e proteinúria nefrótica.

Henoch-Schõnlein purpura is a vasculitis of small vessels, more common in children with involvement cutaneous, articular, gastrointestinal and renal. It rarely affects adults, giving these worse prognosis. We report a patient aged 54 with Henoch-Schõnlein purpura with involvement of skin, joint, abdominal and renal, without clinical response with high doses of prednisone keeping a lameness abdominal and nephrotic proteinuria.

Púrpura de Henoch-Schõnlein com acometimento incomum de face / Unusual face involvement in Henoch-Schõnlein purpura

A Púrpura de Henoch-Shõnlein é uma vasculite de causa idiopática, que se caracteriza pelo depósito predominante de IgA na parede dos pequenos vasos, envolvendo tipicamente pele, intestino, articulações e glomérulo renal. O acometimento cutâneo ocorre principalmente em membros inferiores e região glútea, sendo raramente encontrado em face e membros superiores. Relatamos o caso de uma paciente do sexo feminino, com seis anos de idade, que apresentava lesões púrpuricas em região auricular, periorbital, malar, mentoniana e membros superiores, além de artralgia e volvo intestinal. Após tratamento cirúrgico e pulsoterapia com glicocorticoide, houve regressão dos sintomas, sem maiores complicações.

Henoch-Schõnlein Purpura is an idiopathic vasculitis characterized by deposits of immunoglobulin, mainly IgA, on the walls of small vessels, typically involving the skin, gut, joints, and renal glomeruli. Cutaneous involvement affects specially the lower limbs and buttocks, and it is seldom found on the face and upper limbs. We report the case of a 6-year old girl with purpuric lesions over the auricular, periorbital, malar, and mentonian regions and the upper limbs, arthralgia, and intestinal torsion. After surgical treatment and pulse therapy with glucocorticoids, her symptoms subsided without further complications.

Edema agudo hemorrágico de la infancia en una niña mayor; una entidad diferente de la púrpura de Henoch-Shönlein / Infantile acute hemorrhagic edema in a female child: a different entity from Henoch-Shönlein purpura

Introducción. El edema agudo hemorrágico de la infancia (EAHI) es una entidad nosológica que se manifiesta en niños pequeños, es autolimitada y de curso benigno. Usualmente su manifestación es secundaria y existe el antecedente de infección de vías aéreas superiores, administración de antibióticos o aplicación de vacunas. Las dos manifestaciones cutáneas principales son lesiones purpúricas en roseta o cocarda y anulares o en tiro al blanco, que se encuentran de manera primaria en cara y extremidades superiores, además hay edema en cara y extremidades. Caso clínico. Se informa el caso de un preescolar femenino de 4 años y 11 meses de edad con manifestaciones clínicas clásicas de esta entidad, pero que se presentó en edad tardía. Conclusión. Se refuerza el concepto de que la púrpura de Henoch-Schönlein y el EAHI son entidades diferentes.

Introduction. Infantile acute hemorrhagic edema (IAHE) is an entity manifested in young children and has a self-limiting and benign course. It usually appears secondary to a history of upper respiratory illness, course of antibiotics or vaccination. The two primary cutaneous features include large "cockade" or rosette appearance or annular purpuric lesions found primarily on the face and upper extremities along with edema of the limbs and face. Case report. We report the case of a female patient (age 4 years and 11 months) who manifested all the classic clinical characteristics of this entity at an older age. Conclusion. The concept that Henoch-Schönlein purpura and acute hemorrhagic edema of infancy are different entities is reinforced.

Historia de las vasculitis primarias en Latinoamérica / History of primary vasculitis in Latinoamerican

En este artículo se hace una revisión extensa sobre las vasculitis primarias y se recopila la información latinoamericana que aparece referen-ciada en las más importantes bases de datos mundiales en inglés, español y portugués, desde el año 1945 hasta septiembre del año 2007. Igualmente se establecieron varios contactos con reumatólogos o médicos interesados en este tópico. Se realizaron búsquedas secundarias de los estudios que aparecieran citados en los artículos seleccionados y se revisaron manualmente abstracts de congresos.

In this paper an extensive review about primary vasculitis and Latino American information that appears referenced in the most important world wide data bases in English, Spanish and Portuguese from 1945 to september 2007, is compiled. Several contacts with rheumatologist or physician interested in this topic were made. Also secondary searches of the studies that appeared mentioned in selected articles were made and the abstracts of congresses were reviewed manually.