Leguminosas como causa infrecuente de alergia alimentaria en Colombia: reporte de dos casos y revisión de la literatura / Legumes as an infrequent cause of food allergy in Colombia: report of two cases and review of the literature

La alergia alimentaria se ha venido incrementando a nivel mundial, afectando alrededor del 1,5 % a 2,5 % de los adultos y 6 % de los niños, y tiene un gran impacto en la calidad de vida de los pacientes y sus cuidadores, debido a las dietas de restricción. Los alérgenos más prevalentes son la leche, el huevo, el trigo, la soja, los frutos secos, el maní, el pescado y los mariscos. Las leguminosas mejor estudiadas son el maní y la soja; otras leguminosas como las lentejas, garbanzos y arvejas representan la quinta causa de alergia alimentaria en el área mediterránea, en Turquía y en la India, siendo menos prevalentes en otras áreas geográficas. La alergia a las leguminosas es una entidad infrecuente en Colombia, se desconoce la prevalencia en el país. Describimos los primeros dos casos de anafilaxia por lentejas reportados en el país. Ambos pacientes menores de 18 años, con reacciones adversas tras la ingesta de leguminosas, en las cuales se demuestra alergia mediada por IgE a las lentejas y además sensibilización en el primer caso a las arvejas y garbanzos, y en el segundo caso a los frijoles. Diferentes datos sobre la prevalencia se han descrito en varias áreas geográficas, siendo mayor en países con dietas mediterráneas. Las reacciones mediadas por IgE suelen aparecer incluso con el alimento altamente cocido, debido a la termo-estabilidad de las proteínas. La reactividad cruzada más frecuente se relaciona con los garbanzos y las arvejas

Food allergy has been increasing worldwide. Affects around 1.5% to 2.5% of adults and 6% of children, and has a great impact on the quality of life of patients and their caregivers, due to restricted diets. The most prevalent allergens are milk, egg, wheat, soy, tree nuts, peanuts, fish and shellfish. The best studied legumes are peanuts and soybeans; other legumes such as lentils, chickpeas and peas represent the fifth cause of food allergy in the Mediterranean area, Turkey and India, being less prevalent in other geographical areas. Allergy to legumes is not common in Colombia, the prevalence in the country is unknown. We describe the first two cases of legumes anaphylaxis reported in the country. Both patients were under 18 years of age, with adverse reactions after ingesting legumes, in which IgE-mediated allergy was demonstrated; in the first case to lentils, peas and chickpeas, and in the second case, to lentils and beans. Different data on prevalence have been described in various geographical areas, being higher in countries with Mediterranean diets. IgE-mediated reactions usually appear even with highly cooked food, due to the thermo-stability of proteins. The most frequent cross-reactivity is related to chickpeas and peas

Anisakiasis gastro-alérgica, primera descripción de un caso en Colombia y revisión bibliográfica / Gastro-allergic anisakiasis: The first case reported in Colombia and a literature review

Resumen La anisakiasis es una enfermedad parasitaria zoonótica causada por el consumo de pescados o mariscos crudos o poco cocidos infectados con nematodos de los géneros Anisakis, Pseudoterranova y Contracaecum. Se describe el primer caso de anisakiasis en Colombia y se resume la literatura médica disponible. Una mujer de 52 años de edad consultó por dolor epigástrico agudo de inicio abrupto, náuseas, vómitos, diarrea y urticaria después de consumir pescado. El examen físico reveló sensibilidad moderada en el epigastrio. El examen de laboratorio evidenció leucocitosis, en tanto que la radiografía simple y el electrocardiograma no reflejaron ninguna anormalidad. El diagnóstico se hizo mediante una endoscopia de vías digestivas altas, la cual reveló engrosamiento de la pared gástrica y un parásito en movimiento. Se encontró una larva de Anisakis y se la extrajo por endoscopia, lo que alivió el dolor de la paciente. Clínicamente, la anisakiasis puede presentarse como una enfermedad gástrica, intestinal, en otros sistemas o alérgica. El diagnóstico se hace con base en la elaboración del historial alimentario del paciente y la visualización directa de las larvas; el único tratamiento efectivo consiste en su extracción endoscópica.

Abstract Anisakiasis is a zoonotic parasitic disease caused by consumption of raw or undercooked fish or seafood infected with nematodes of the Anisakis, Pseudoterranova or Contracaecum genera. Here, we describe the first case of anisakiasis in Colombia and summarize the available literature. A 52-year-old female with a history of abrupt-onset sharp epigastric pain, nausea, vomit, diarrhea, and urticaria following fish consumption consulted the health service. The physical examination revealed moderate tenderness of the epigastric region; the laboratory evaluation showed leukocytosis and a simple X-ray and ECG showed no abnormalities. The diagnosis was made by endoscopic examination, which revealed a thickened gastric wall and a moving larval worm. An Anisakis larva was found and extracted endoscopically, which relieved the pain of the patient. Clinically, anisakiasis may present as a gastric, intestinal, extragastrointestinal or allergic disease. Diagnosis and treatment of anisakiasis are made by a dietary history, direct visualization and endoscopic extraction of possible larvae, which is the only effective therapy.

Incapacitating solar urticaria: successful treatment with omalizumab

Abstract: Solar urticaria is a rare form of physical urticaria mediated by immunoglobulin E. The lesions appear immediately after the sun exposure, interfering with the patient's normal daily life. Omalizumab, a monoclonal anti-IgE antibody, has been recently approved for the treatment of chronic spontaneous urticaria, and the latest reports support its role also in the treatment of solar urticaria. Hereby, we report a case of solar urticaria refractory to conventional treatment strategies, with an excellent response to treatment with omalizumab and phototesting normalization.

Urticaria papular y sus agentes causales en Colombia / Papular urticaria: A review of causal agents in Colombia

Resumen La urticaria papular es una enfermedad alérgica causada por la picadura de insectos, la cual predomina en el trópico. El objetivo de esta revisión fue profundizar en sus aspectos epidemiológicos e inmunológicos, particularmente con base en datos publicados en Latinoamérica. Se hizo una revisión no sistemática mediante la búsqueda electrónica de artículos sobre la epidemiología de la urticaria papular, las características entomológicas de los agentes causales y los mecanismos inmunológicos asociados. Según los diversos reportes de centros médicos de Latinoamérica la urticaria papular es frecuente; el único estudio de prevalencia publicado indica que afecta a una cuarta parte de los niños escolares de Bogotá. Hay información sobre la relación causal entre la exposición domiciliaria a la pulga, la pobreza y la urticaria papular en Bogotá, una ciudad representativa de las altitudes andinas. No hay estudios que indaguen directamente sobre los insectos causales en zonas cálidas, aunque se sospecha clínicamente de los mosquitos Aedes aegypti y Culex quinquefasciatus. En cuanto a su patogenia, se destaca la participación de mecanismos celulares que involucran las células colaboradoras Th2, lo cual explica que sea una condición de hipersensibilidad retardada. El papel de la inmunoglobulina E (IgE) en la urticaria papular no está tan claro. Se desconocen los antígenos derivados de los insectos que causan la enfermedad, aunque se plantea que existen moléculas comunes de reacción cruzada entre los insectos, tales como el alérgeno Cte f 2 en la pulga, y sus homólogos en los mosquitos. La urticaria papular es una condición frecuente en Latinoamérica que debe investigarse en profundidad. La caracterización inmunológica de los componentes moleculares que causan esta condición puede resolver interrogantes sobre su etiología y su patogenia.

Abstract Papular urticaria is a chronic allergic reaction induced by insect bites, which is common in the tropics. The objective of this review was to deepen on epidemiological and immunological aspects of this disease, focused on data published in Latin American countries. We conducted a non-systematic review of the literature through electronic search on the epidemiology of papular urticaria, the entomological characteristics of the causative agents and associated immunological mechanisms. Several reports from medical centers suggest that papular urticaria is common in Latin America. Only one epidemiological survey designed to estimate prevalence of papular urticaria has been published, reporting that about a quarter of children under six years of age is affected by this condition in Bogotá. There is evidence on the causal relationship among exposure to indoor fleas, poverty and papular urticaria in Bogotá, a representative city of the Andean altitudes. Information about causal insects in tropical warmer areas is scarce, although from clinical reports Aedes aegypti and Culex quienquefasciatus appear to be the most common. Th2 cellular-mediated mechanisms are involved in its pathogenesis, which explains its delayed hypersensitivity. The role of immunoglobulin E is not clear in this disease. Insect-derived antigens directly involved in papular urticaria etiology are unknown. However, it is possible that common molecules among causal insects mediate cross-reactive reactions, such as Cte f 2 allergen, found in cat fleas, and its counterparts in mosquitoes. Papular urticaria is a frequent disease in Latin America that should be further investigated. Immunological characterization of the molecular components that cause this condition may solve questions about its pathogenesis.

Follicular traction urticaria

Abstract Inducible urticaria is a heterogeneous subgroup of chronic urticarias caused by a wide variety of environmental stimuli, such as exercise, cold, heat, pressure, sunlight, vibration, and water. A new term, follicular traction urticaria, was suggested as an unusual form of inducible urticarias. We report a patient who was diagnosed with follicular traction urticaria.

Reacción sistémica tras la ingesta de una oruga procesionaria del pino: ¿Manejo conservador? / Systemic reaction after pine processionary caterpillar ingestion: Conservative management?

La oruga procesionaria del pino, forma larvaria de la mariposa nocturna Thaumetopoea pityocampa, es una de las principales plagas forestales del sur de Europa. Con frecuencia, estas orugas producen reacciones locales en humanos debidas a los pelillos urticantes microscópicos que las recubren. La mayoría de los síntomas ocasionados son cutáneos, como urticaria aguda, y son mucho más infrecuentes las reacciones sistémicas. Se presenta un caso de reacción sistémica, con edema orofacial, babeo y urticaria tras la ingesta de una oruga procesionaria del pino en un lactante de 11 meses, en el cual el tratamiento farmacológico fue suficiente para la resolución de los síntomas. No tuvo, en ningún momento, compromiso de la vía aérea ni otros datos de anafilaxia, por lo que se desestimaron actuaciones más agresivas, como intubación orotraqueal, endoscopía o laringoscopía.

The larval form of the moth Thaumetopoea pityocampa, known as pine processionary caterpillar, is one of the main forest pests in southern Europe. Often, these caterpillars cause local reactions in humans, due to their microscopic stinging hairs. Most symptoms affect skin, in form of acute urticaria. Systemic reactions are rare. An eleven month old infant with a systemic reaction after ingestion of a pine processionary caterpillar is presented. The boy reached the Emergency Room with orofacial edema, drooling and urticaria. Drug treatment was enough to resolve the symptoms. The patient did not present airway commitment, nor anaphylaxis data at any moment, so more aggressive actions, such as endotracheal intubation, endoscopy or laryngoscopy, were rejected.

Urticaria inducible: serie de casos y revisión de la literatura

Las urticarias inducibles constituyen un grupo heterogéneo de trastornos cutáneos caracterizados por la aparición de habones, prurito o angioedema, que en ocasiones se acompañan de síntomas sistémicos causados por estímulos inocuos para la mayoría de la población, como el frío, el calor, la presión, etc., y que comprometen la calidad de vida de los pacientes. La mayor parte de la literatura médica pertinente proviene de reportes y series de casos, ya que su epidemiología se ha estudiado poco. El objetivo de esta revisión es ofrecer una visión actualizada de la información disponible sobre varios tipos de urticaria inducida, mediante la presentación de un caso clínico ilustrativo y la descripción de los mecanismos fisiopatológicos, las manifestaciones clínicas y el tratamiento de cada condición.

Inducible urticaria is a heterogeneous group of skin disorders characterized by the appearance of wheals, pruritus and/or angioedema, sometimes accompanied by systemic symptoms caused by innocuous stimuli (cold, heat, pressure, etc.). This group of disorders compromises people´s quality of life and most of the literature in this regard comes from case reports and case series since its epidemiology has been poorly studied and some cases are very rare. The aim of this review is to show an up-to-date overview of the available literature for various types of inducible urticarias, always beginning with an illustrative case and then describing their pathophysiological mechanisms, clinical manifestations, and treatment.

Chronic urticaria in adults: state-of-the-art in the new millennium

Chronic urticaria has been explored in several investigative aspects in the new millennium, either as to its pathogenesis, its stand as an autoimmune or auto-reactive disease, the correlation with HLA-linked genetic factors, especially with class II or its interrelation with the coagulation and fibrinolysis systems. New second-generation antihistamines, which act as good symptomatic drugs, emerged and were commercialized over the last decade. Old and new drugs that may interfere with the pathophysiology of the disease, such as cyclosporine and omalizumab have been developed and used as treatments. The purpose of this article is to describe the current state of knowledge on aspects of chronic urticaria such as, pathophysiology, diagnosis and the current therapeutic approach proposed in the literature.

Correlación entre urticaria crónica y coagulación / Correlation between chronic urticaria and coagulation

Chronic urticaria (UC), defined as recurrence of wheals with or without angioedema for more than 6 weeks, is a quite common disease that may severely worsen the quality of life. Although the actual athophysiological mechanisms are still unknown, what we do know is that the main cells involved in their pathology are mast cells and eosinophils. The present article reviews recent findings showing an additional pathogenic mechanisms in UC patients: activation of the coagulation cascade resulting in thrombin production. Several investigators have demonstrated the activation of coagulation that is due to the involvement of eosinophils and a tissue factor pathway with generation of thrombin potentially contributing to an increased vascular permeability. CU patients often present with elevation of coagulation and fibrinolysis markers, such as prothrombin fragment F1+2 and D-dimer, which correlate with the disease severity. Preliminary data indicate that anticoagulant treatment with heparin and warfarin may be effective in reducing the symptoms of this disorder...

Guía Argentina de urticaria y angioedema

Se actualiza el diagnóstico de la urticaria crónica (UC) y los conceptos, definiciones y sugerencias basados en la evidencia para su tratamiento. La urticaria ocurre en al menos 20% de la población en algún momento de la vida. Su etiología difiere en la forma aguda (menos de 6 semanas), y en la crónica. No es posible pronosticar si las formas agudas evolucionarán a UC, ya que todas son agudas al comienzo. La UC ocurre como espontánea (UCE) o inducible (UCI). El diagnóstico es sencillo, pero incluye un minucioso estudio para descartar diagnósticos diferenciales; para UCI son útiles las pruebas de provocación en la caracterización y manejo. Los estudios complementarios se deben limitar y orientar según sospecha clínica. El tratamiento se divide en tres enfoques: evitación, eliminación o tratamiento del estímulo desencadenante o de la causa, y tratamiento farmacológico. Recientemente éste se modificó, con empleo de antihistamínicos de segunda generación como primera línea y aumento de dosis de antihistamínicos H1 no sedantes, hasta 4 veces, como segunda línea. Los antihistamínicos son fundamentales para tratar la UC; sin embargo, un 40% de los pacientes no logra un buen control pese al aumento de dosis y requiere otro medicamento adicional. La evidencia más reciente considera que un grupo de fármacos puede utilizarse como tercera línea en estos casos, para mejorar la calidad de vida y limitar la toxicidad por el uso frecuente o crónico de esteroides sistémicos. Se recomiendan para esta tercera línea solo 3 fármacos: omalizumab, ciclosporina A o antileucotrienos.

This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20% of the population at some point in their lives. Acute urticaria (less than 6 weeks' duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU´s diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40% of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.

Guía argentina de urticaria y angioedema / Argentine guidelines for urticaria and angioedema

This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20

of the population at some point in their lives. Acute urticaria (less than 6 weeks’ duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU’s diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40

of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.

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.

Urticaria crónica / Chronic urticaria

La urticaria crónica (VC), es una variante común de la urticaria que se define como un síndrome reactivo de la piel y mucosas caracterizado por la presencia de placas eritematosas, habones o ronchas edematosas y pruriginosas que se prolonga durante más de seis semanas. La etiología de la VC no es muy clara en la mayoría de los casos, multitud de causas se han implicado. La patogenia es compleja. El conocimiento y estudio de cada uno de los factores celulares y solubles, su interacción dinámica, vías efectoras y trama de citoquinas conducirá al logro de una terapéutica efectiva y racional de la urticaria crónica.

Chronic urticaria (VC) is a common variant of urticaria which is defined as a reactive skin and mucous membranes syndrome characterized by the presence of plaques erythematous, wheals, or swollen and itchy hives that lasts more than six weeks. The etiology of the VC is not very clear in the majority of cases, multitude of causes have been implicated. The pathogenesis is complex. Knowledge and study of each cell and soluble factors, their dynamic interaction, effector pathways and cytokines plot will lead to the achievement of an effective and rational therapy of chronic urticaria.

Picadura de medusa Olindias sambaquiensis: Análisis de 49 casos

La picadura de la medusa o agua viva Olindia sambaquiensis es de ocurrencia frecuente en las playas de la costa de la provincia de Buenos Aires. Se notifican entre quinientas y mil picaduras de medusas por temporada en la localidad balnearia de Monte Hermoso, al sur de la provincia de Buenos Aires. El daño cutáneo que provoca por su elevada acción urticante plantea un serio inconveniente para el desarrollo del turismo. Durante el mes de enero de 1998, en la localidad de Monte Hermoso se reunieron un total de 49 casos que fueron examinados dentro de la hora posterior a la picadura. Veintiocho eran de sexo masculino (57.1%). La edad promedio fue de 16 ± 4.1 (rango 5-80). El 54% presentó lesiones eritemato-edematosas de configuración lineal, el 28% presentó lesiones predominantemente eritematosas y el 18% presentó placas eritemato-edematosas. En el 73% de los casos las lesiones se localizaron en miembros inferiores. Hemos redefinido la lesión cutánea producida por la medusa O. sambaquiensis, su evolución, sus dimensiones y localizaciones más frecuentes. Además, se ha tipificado y cuantificado el dolor que produce, y otros signos y síntomas que acompañan a la picadura en la hora posterior, a las primeras 24 horas y a los 30 días. Describimos las conductas terapéuticas utilizadas en nuestra costa, evaluamos la efectividad de algunas de ellas en el control del dolor y finalmente proponemos un esquema terapéutico para dicha picadura.

Olindias sambaquiensis jellyfish sting occurs frequently in Buenos Aires province coast beaches. Among five hundred and one thousand stings by jellyfish are reported each season at Monte Hermoso, a beach village in the South of Buenos Aires province. The skin damage provoked because of its highly irritant effect poses a serious issue related to tourism development. A total number of 49 cases that were examined during the first hour after the sting were enrolled in Monte Hermoso during January 1998. Twenty eight were males (57.1%). The average age was 16 ± 4.1 (range 5-80). Of them, 54% showed linear erythema-edematous lesions, 28% showed predominantly erythematous lesions, and in 18% the injuries were erythema-edematous plaques. In 73% of the cases the lesions were located in lower limbs. We had hereby redefined cutaneous lesions produced by O. sambaquiensis, its evolution, its dimensions and most frequent localizations. Besides, it has been typified and quantified the pain it provokes and other signs and symptoms that go with the sting during the posterior hour, during the first 24 hours, and after 30 days. We described the therapeutic conducts used in our coasts and we assessed the effectiveness of some of them in pain control, and finally we propose a therapeutic scheme for this sting.

Urticaria aguda / Acute urticaria

La urticaria es una reacción de la piel caracterizada clínicamente por la aparición de elevaciones cutáneas eritematosas o blanquecinas, edematosas, pruriginosas y transitorias, que pueden localizarse en cualquier parte de la superficie cutáneao en las mucosas. Su etiología es variada y sus mecanismos etiopatogénicos también suelen ser múltiples. La lesión típica es el habón o roncha, el cuál cambia de lugar en horas, su forma y tamaño son variables, puede persistir minutos, horas o semanas. La mayoría de las urticarias no presenta sintomatología sistémica. Suele clasificarse regularmente en urticaria aguda, cuando lleva menos de 6 semanas de aparición, y en urticaria crónica, cuando lleva más de 6 semanas. El diagnóstico es fundamentalmente clínico. Es una entidad frecuente que por su gran expresividad motivan constantes y reiteradas consultas a los servicios de urgencia. En este artículo se examinarán los mecanismos que llevan a la urticaria y angioedema, y se revisará la patogénesis, los aspectos clínicos, los estudios de laboratorio necesarios y el tratamiento de la urticaria aguda.

The urticaria is a skin reaction characterized clinically by the on set of erythematous skin elevations or whitish, oedematous,pruritic and transient, you can locate anywhere on the surface of the skin or the mucous membranes. Its etiology is varied and its mechanisms etiopathogenic also tend to be multiple. The typical lesion is the wheal or bump, which changes from place with inhours, its size and shape are variable, may persist for minutes, hours or weeks. The majority of the hives does not pose systemic symptoms. Usually qualify regularly in acute urticaria, when it takes less than 6 weeks of appearance, and chronic urticaria, when it takes over 6 weeks. The diagnosis is primarily clinical. Itis a common entity that by their expressivity motivate constants and repeated consultations to emergency services. This article will xamine the mechanisms that lead to angioedema and urticaria, he pathogenesis, clinical aspects, necessary laboratory studies and eatment.

Urticária aquagênica familiar: relato de dois casos e revisão da literatura / Familial aquagenic urticaria: report of two cases and literature review

Urticária aquagênica é forma rara de urticária física caracterizada por aparecimento de urticas após o contato com água, independente da temperatura. Há poucos casos descritos de urticária aquagênica e, destes, somente cinco da forma familiar. Apresentamos o primeiro relato de urticária aquagênica familiar no Brasil, acometendo mãe e filha. Ambas apresentavam urticas, principalmente após banho de chuveiro, independentemente da temperatura da água. A mãe referia ter o quadro há quatro anos, e a filha, desde o nascimento. Para diagnóstico, foram realizados testes de provocação com água, com aparecimento de lesões em ambas, e testes com dermografômetro, com cubo de gelo envolvido em plástico e de provocação para urticária colinérgica, sem o aparecimento de lesões, excluindo assim outras formas de urticária física.

Aquagenic urticaria is a rare form of physical urticaria, characterized by pruritic wheals that appear following contact with water, independently of its temperature. There are few reports of cases of aquagenic urticaria, and only five include the familial form. We present the first case of familial aquagenic urticaria in Brazil (mother and daughter). Both patients presented wheals following contact with water, especially when showering, regardless of its temperature. The mother reported onset of urticaria four years before and the daughter presented wheals since birth. For diagnostic purposes, they were submitted to a challenge test with water, and both subjects presented wheals, as well as to tests using ice cubes in plastic bag with dermographometer and challenge tests for cholinergic urticaria, with no appearance of lesions, excluding other forms of physical urticaria.