Alergia a la leche de vaca: ¿se pueden evitar las pruebas de provocación oral? Un análisis probabilístico basado en datos clínicos / Cow's milk allergy: Can oral food challenges be avoided? A probabilistic analysis based on clinical data

Introducción. La prueba de provocación oral (PPO) para el diagnóstico de alergia a las proteínas de la leche de la vaca (APLV) presenta riesgos y requiere de recursos. Nuestro objetivo fue evaluar condiciones y pruebas complementarias para identificar una alta probabilidad de APLV. Población y métodos. Análisis secundario sobre estudio de pacientes atendidos en una unidad de alergia entre 2015 y 2018. Se determinaron las probabilidades prepruebas asociadas a los síntomas y sus combinaciones, y las probabilidades pospruebas luego de realizadas pruebas cutáneas y determinación de inmunoglobulina E (IgE) sérica. Resultados. Se evaluó la información de 239 pacientes. Se observaron probabilidades mayores al 95 % en pacientes con angioedema y combinación de urticaria y vómitos. Usando puntos de corte propuestos por Calvani et al., la combinación de vómitos con rinitis, sin angioedema, también superó el 95 %. Conclusión. Se ofrece una metodología para identificar pacientes en los que puede diagnosticarse APLV sin realización de PPO.

Introduction. The oral food challenge (OFC) for the diagnosis of cow's milk protein allergy (CMPA) poses risks and requires resources. Our objective was to assess conditions and complementary tests used to identify a high probability of CMPA. Population and methods. Secondary analysis of a study of patients seen at a unit of allergy between 2015 and 2018. Pre-testing probabilities associated with symptoms and their combinations and post-testing probabilities after skin prick testing and serum immunoglobulin E (IgE) levels were determined. Results. The data from 239 patients were assessed. A probability greater than 95% was observed for angioedema and a combination of urticaria and vomiting. Based on the cut-off points proposed by Calvani et al., the combination of vomiting with rhinitis, without angioedema, also exceeded 95%. Conclusion. A methodology is provided to identify patients in whom CMPA may be diagnosed without an OFC.

Episodic angioedema with eosinophilia (Gleich's syndrome) associated with urticarial vasculitis: a coincidence or a novel clinical entity?

Episodic angioedema with eosinophilia (EAE) is a rare condition characterized by recurrent attacks of angioedema and urticaria accompanied by a marked elevation of peripheral eosinophil count. We report the case of a young female patient diagnosed with EAE associated with urticarial vasculitis. A 40-year-old female patient was admitted to our institution due to recurrent episodes of cheek and eyelid angioedema in the previous year. Episodes of facial angioedema lasted for two months with spontaneous remission afterwards. In addition, she presented pruritic and painful skin eruptions of erythematous circles, which persisted for longer than 24 h, that were palpable, somewhat purplish, and more pronounced on the face, arms, and trunk. Laboratory investigation showed a sustained elevation of white cell counts with marked eosinophilia. Serum IgM, IgE, and IgA were normal; IgG was slightly elevated. C1-esterase inhibitor and tryptase test were normal. Reverse transcriptase-polymerase chain reaction was performed for detection of FIP1L1-PDGFRA and BCR-ABL rearrangements. None of these alterations were found. Skin biopsies were suggestive of urticarial vasculitis. The patient was submitted to esophagogastroduodenoscopy, which showed mild chronic gastritis, with no eosinophilic infiltration. Cardiac dimensions and function were normal. Abdominal ultrasound and total body CT-scan failed to show lymphadenopathy, organomegaly, and tumors. We report the first case of association between episodic angioedema with eosinophilia and urticarial vasculitis. It is possible that both conditions share a physiopathological mechanism, suggesting that it is not just a chance association.

Queilitis granulomatosa, a propósito de dos casos / Granulomatous cheilitis, regarding two cases

La queilitis granulomatosa es una entidad granulomatosa no infecciosa, poco frecuente, que se presenta como un aumento de volumen persistente de la región orofacial. El estudio histológico, junto con la exclusión de otras patologías granulomatosas son necesarios para su diagnóstico, especialmente cuando no se presenta con la triada clásica del Síndrome de Merkelsson Rosenthal. Presentamos dos casos de queilitis granulomatosa y una revisión de la literatura disponible.

Granulomatous cheilitis is a rare, non-infectious, granulomatous entity that presents as a persistent swelling of the orofacial region. Histological study together with the exclusion of other granulomatous diseases are necessary for the diagnosis, especially when the presentation is not the classic triad of Merkelsson Rosenthal Syndrome. We present two cases of granulomatous cheilitis and a review of the available literature.

Angioedème bradykinique d'évolution fatale suite à une prise d'énalapril. A propos d'un cas

L'angiœdème bradykinique est une maladie rare et grave qui constitue une complication exceptionnelle du traitement aux Inhibiteurs de l'Enzyme de Conversion (IEC). Elle engage le pronostic vital des patients dans 75% des cas et représente une urgence médicale majeure. L'évolution de cette affection est totalement imprévisible avec un risque de décès par asphyxie. Les auteurs rapportent l'observation d'une patiente de 70 ans hypertendue qui a présenté un angiœdème bradykinique d'évolution fatale deux jours après l'initiation de son traitement antihypertenseur par les IEC. Au travers cette observation, les auteurs voudraient mettre en lumière cette affection mortelle, de diagnostic difficile souvent méconnue

Episodic angioedema associated with eosinophilia

Abstract: We report a 12-year-old girl who presented with recurrent angioedema on the face, trunk, and extremities, and concomitant marked weight gain for 5 years. During the episode, her white blood cell count increased to 47.7×109/L with 89.9% eosinophils, followed by elevated serum level of IL-5, IgE, IgM, and LDH. Histopathology showed perivascular eosinophilic infiltration and diffuse eosinophilic infiltration throughout the dermis. Possible causes of hypereosinophilia and eosinophilic infiltration of vital organs were ruled out. We also tested the FIP1L1/PDGFRa and ETV6/PDGFRb fusion gene to exclude the possibility of myeloid and lymphatic vessel neoplasms. The patient was treated with methylprednisolone and discharged with an oral prednisolone taper, which resulted in complete remission of the edema and normalization of peripheral blood eosinophil count, serum IL-5 level, IgE, IgM, and LDH.

Angioedema por enalapril: caso clínico / Enalapril angioedema: clinical case

La prescripción de inhibidores de la enzima convertidora de angiotensina (IECA), en el manejo de la hipertensión e insuficiencia cardíaca se ha incrementado fuertemente desde su introducción en 1980. Estas drogas son consideradas seguras, pero se sabe que pueden producir angioedema severo como efecto secundarop en el 0.1 a 0.3% de los pacientes tratados. Caso clínico: paciente de sexo masculino de 57 años de edad, con diagnóstico de hipertensión arterial, tratado con Enalapril 20mg/día, desde hacía 7 días. Ingresa por guardia presentando angioedema en zona bipalpebral, bilateral; labio superior e inferior y regiones malares. No representa compromiso respiratorio. Dentro de sus antecedentes niega alergia a medicamentos y/o alimentos. Se indica como tratamiento Hidrocortisona y Difenhindramina (EV), evolucionando favorablemente, por lo que es externado indicándose Prednisona y Fexofenadina (OV). Dentro de las 48 hs. siguientes concurre a control, encontrandosé asintomático. Discusión: El mecanismo por el cual los inhibidores de la angiotensina producen angioedema no es claro pero probablemente sería por una acumulación tisular de bradiquinina. El angioedema generalmente afecta cabeza y cuello, por lo tanto la vía aérea está en riesgo. Ante un paciente con angioedema tratado con IECA, debería considerarse como primera causa a la administración del medicamento ya que la misma puede presentarse a los días, meses e incluso años de comenzado el tratamiento. Conclusión: El desafío para futuras investigaciones es identificar los subgrupos de pacientes con mayor riesgo de presentar angioedema, en quienes el riesgo de recibir terapia con IECA es mayor que el beneficio.

Prescription of angiotensin convertidara inhibitors (ACE) in the management of hypertension and heart failure has increased rapidly since its introduction in 1980. These drugs are considered to be safe, but it is known that can produce severe angioedema as side effect in 0.1 to 0.3% of treated patients. Clinical Case: A 57 years old male patient was admitted to the emergency department because angioedema in the bipalpebral and bilateral areas as well as in the upper and cheek regions. He had a diagnosis of hypertension and had been taking enalapril 20mg/day, for the previous 7 days. There was no respiratory compromise. He denied history of drug and / or food allergies. Hydrocortisone and Difenhindramina (EV) were prescribed with a favourable outcome. The patient was discharged with indications of prednisone and fexofenadine (OV). At a follow-up visit, 48 hours later, he presented no symptoms. Discussion: The mechanism by which inhibitors of angiotensin produced angioedema is unclear but would probably be by a tissue accumulation of bradykinin. Angioedema usually affects the head and neck so the airway is at risk. In patients with angioedema and ACE inhibitors treatment, IECA should be considered as first cause. It can occur days, months and even years of after the beginning of treatment. Conclusion: The challenge for future research is to identify subgroups of patients at increased risk of angioedema, in whom the risk of receiving ACE inhibitor therapy is greater than the benefit.

Recurrent abdominal pain due to hereditary angioedema.

Recurrent abdominal pain is a common problem in children that may need invasive procedures for diagnosis. Hereditary angioedema (HAE) is rarely considered in the differential diagnosis. Here it is reported a girl with HAE, who presented initially as recurrent abdominal pain without cutaneous manifestations. Each episode was managed elsewhere as an acute surgical emergency and an exploratory laparotomy was planned. Diagnosis was confirmed by quantitative assay of C1 inhibitor. On detailed evaluation, many members of her family were affected.

A case report of nonepisodic angioedema with eosinophilia in a Korean patient and a review of the Korean literature

Episodic angioedema with eosinophilia (EAE) is characterized by recurrent angioedema, peripheral eosinophilia, elevated serum IgM, fever, weight gain, and a benign course lacking any internal organ involvement. Dozens of cases of the nonepisodic variant (NEAE), which is limited to a single attack, have been reported in Japan. These NEAE cases normally have been less severe than the episodic type. In this paper, we describe the case of a Korean patient whose clinical and laboratory findings were consistent with NEAE, and review five other cases of EAE and NEAE reported in the Korean literature. The Korean NEAE cases outlined in this paper demonstrate that, as in Japan, NEAE is not uncommon in Korea, and also suggest that this disease exhibits a cultural predilection for Asian populations.

A case of Hereditary Angioedema Associated with Idiopathic Hypoparathyroidism

Hereditary angioedema is a rare autosomal dominant disease characterized by the edema of subcutaneous tissues, respiratory tract and bowel. It is caused by the deficiency of C1 esterase inhibitor. Hereditary angioedema may be associated with autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, autoimmune thyroiditis and glomerulonephritis. We report a 34-year-old male patient with hereditary angioedema who developed idiopathic hypoparathyroidism. Autoimmunity seems to be an important basis of this association and it might be caused by the immune dysfunction due to decreased level of complements; nevertheless, a casual association could not be excluded. To our knowledge, this is the first report of hereditary angioedema in association with idiopathic hypoparathyroidism in the medical literature.

Angioedema y urticaria asociadas con infección activa del virus de Epstein-Barr en un adulto / Angioedema and urticaria associated with active Epstein-Barr virus infection in an adult patient

Un paciente de 43 años de edad consultó por una historia de dos años con episodios recurrentes de urticaria en miembros y tronco, y edema de labios y párpados. Se le realizaron varios estudios de diagnóstico, incluyendo mediciones del C1 inhibidor y análisis de marcadores para infecciones virales, entre otras cosas. Los resultados fueron dentro de los parámetros normales salvo los títulos de IgG anti-EBV que resultaron positivos en dilucione de 1/80. Se efectuó la técnica de transcripción reversa y PCR (RT-PCR) para determinar la presencia de EBV en forma replicativa, y se determinó la presencia de ARN con secuencias del gen BLLF1. El paciente recibió un tratamiento durante 40 días con acyclovir 200 mg 4 veces al día. Al final de dicho período, los síntomas (urticaria y angioedema) desaparecieron por completo, y la técnica de RT-PCR resultó negativa para el gen BLLF1 del virus de Epstein-Barr. Sobre las bases del seguimiento clínico y los resultados de la técnica de RT-PCR, concluimos que la infección activa por el EBV puede jugar un rol en la expresión de urticaria y angioedema en pacientes susceptibles

Edema angioneurótico hereditario / Hereditary angioneurotic edema

Se realiza una revisión del tema edema angioneurótico hereditario. Después de una introducción histórica se considera el sistema del complemento, el sistema de las cininas, los inhibidores de las proteasas hemáticas derivadas del plasminógeno y la vinculación entre ambos. Se consideran también los avances últimos en lo que a citogenética molecular se refiere. Se presentan las diferentes formas clínicas de los edemas angioneuróticos hereditarios, así como sus diferencias clínico-laboratoriales. Se hace una valoración de los tratamientos sustitutivos y profilácticos. Se expone el estudio de tres casos clínicos observados en el lapso de 30 años de práctica dermatológica.

Urticaria in Thai children.

In this study, 142 patients under 12 years of age with the diagnosis of urticaria accompanied or not by angioedema were examined. In all 72.6% of the patients were under 6 years of age. Boys and girls were equally affected, 13.4% of the cases had chronic urticaria, 56.3% had a previous history of urticaria, 88.0% had generalized urticaria, and about half of the cases had associated angioedema. The causes of the urticaria were identified or suspected in 32.4% of the cases. Drugs, foods, insect bites and stings, infections and cold were the most common or associated precipitating agents in that order. Histories revealed 27.5% of the cases had records of other allergic diseases, and 76.1% had allergic diseases in the family. Dermographisms were found in 16.2% of the cases, and 22.5% of cases had eosinophilia. The means of IgE levels in both acute and chronic urticaria were within normal limits, and there was no statistically significant difference between the two groups. Skin testing was of little practical value in evaluating the etiology of the urticaria. Clinical trials of drugs for symptomatic treatment revealed that clemastine, ketotifen and hydroxyzine gave approximately equally high response rates. All these three medications gave significantly greater response rates than chlorpheniramine.

Urticaria vasculítica: angioedema adquirido y enfermedad tiroidea autoinmune / Vasculitic urticaria: acquired angioedema and autoimmune thyroid disease

Se estudia un caso de urticaria vasculítica y deficiente adquirida del inhibidor de C1 en una paciente con historia personal y familiar de enfermedad de Graves que presenta poliquistosis ovárica. La deficiencia no es confirmada en sus familiares, es transitoria al igual que la disminución de C4 y presencia de complejos inmunes circulantes y se halla FAN + 1/250. Se cree que ambos cuadros se vinculan a la alteración inmune de la paciente, ya que la función tiroidea estaba normalizada al comenzar los brotes, y se menciona la probable acción beneficiosa de la indometacina y el antiestrógeno en la remisión clínica de ambas afecciones sin descartar la remisión espontánea