Systemic autoinflammatory diseases in pediatric population.

Background: Systemic autoinflammatory diseases (SAID) are monogenic and polygenic inherited conditions characterized by dysregulation of the innate immune system. Objective: We aimed to characterize the clinical features of patients with SAID. Methods: This study was a retrospective chart review on the clinical and genetic features of the pediatric population with SAID observed from 1998 to 2020 in our center. Results: A total of 54 patients were evaluated: 18 with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome; 16 with Behçet disease; 13 with systemic juvenile idiopathic arthritis; 4 with syndrome of undifferentiated recurrent fever; 1 with cryopyrin associated periodic syndrome; 1 with chronic nonbacterial osteomyelitis; and 1 with Muckle-Wells syndrome. Conclusion: The analysis of clinical features of our patients are similar to other studies. Our goal was to aware the medical community to early recognize and treat SAID to improve quality of life of pediatric patients.

Association between self-reported drug hypersensitivity reactions and psychological disorders.

Background: Medically unexplained physical symptoms are a well-recognized problem and, in some cases, there is a well-established relationship between behavior and psychopathological disturbances. However, the association between drug hypersensitivity reactions and psychoactive disorders stills under discussion. Objective: Our main goal was to establish if there is an association between self-reported drug hypersensitivity reaction and psychopathology with need for psychoactive drug consumption. Methods: Retrospective study of adult patients evaluated in a first Immunoallergology appointment because of self-reported drug hypersensitivity over 1 year and register of data concerning psychoactive drugs use. Compare the study group with patients observed for allergic respiratory disease along the same year. Results: The study group included 70 patients that referred a total of 92 self-reported drug hypersensitivity reactions. Twenty-nine (41.4%) were under treatment with psychoactive drugs: 20 (70%) were treated with anxiolytics, 13 (18.6%) with antidepressants, 15 (21.4%) with sedatives, and 1 (1.4%) with antipsychotics. The control group included 160 patients and 38 patients (23.8%) were under treatment with psychoactive drugs: 31 (19.4%) where treated with antidepressants, 29 (18.1%) with anxiolytics, and 3 (1.9%) with sedatives. The use of psychoactive drugs in the study group is higher than in the control group (p = 0.007), the difference is especially important for sedative drugs (p < 0.001). Besides a higher use of psychoactive drugs, the study group also has a higher frequency of use of several psychoactive drug (p = 0.002). Conclusion: Patients with a self-report drug hypersensitivity have more tendency to be under treatment with psychoactive drugs and could have more tendency to somatization. Personality traces and psychopathology must be taken into account during an allergy workup.

Recurrent neck swelling after iodinated contrast media administration.

A 74-year-old male with an abdominal aortic aneurism reported 2 episodes of neck swelling 16 hours after an abdominal CT with iodinated contrast media that reappeared in a subsequent CT, although the use of pre-medication. The tests used to exclude the hypersensitivity hypothesis where negative and once a new CT where needed we decided to do it with close monitoring. We performed a facial picture and cervical ultrasound exam before and 24 hours after the exam. The neck swelling and the ultrasound findings 24 hours after the CT made the diagnosis of contrast induced sialoadenitis possible. Contrast induced sialadentis is an uncommon adverse reaction to iodinated contrast media. As it presents with neck swelling it is important to exclude a hypersensitivity reaction because it's further limitations and make a differential diagnosis.

Anaphylaxis to Agaricus bisporus ingestion.

A 33-year-old male with house dust mite allergic rhinitis and asthma reported an episode of facial and lip angioedema, dyspnea, cough and dysphagia at the age of 25, minutes after eating a mushroom ( Agaricus bisporus ) pizza. He denied any drug intake, hymenoptera stings or other possible triggers, and no identifiable cofactors were present. Since then he avoided all types of mushrooms, however an accidental contact occurred with mushroom sauce that resulted in angioedema of the lip within minutes. The allergy workup included measurements of total IgE and specific IgE to mushroom, and skin prick test to aeroallergens sources, possible food allergen sources and mushroom extract, a prick to prick test with raw and cooked A. bisporus , in addition to a SDS-PAGE and immunoblotting assay. The study revealed a specific IgE to mushroom of 0.76kUA/L positive skin prick test to mushroom extract, and prick to prick test positive to white and brown A. bisporus (raw and cooked). The immunoblotting identified two IgE binding proteins with 10kDa and 27kDa. We report a case of A. bisporus anaphylaxis probably due to primary mushroom sensitization. We detected two IgE-reactive proteins with 10kDa and 27kDa as possible culprit allergens.

Anaphylaxis to Agaricus bisporus ingestion / Anafilaxia por ingestão de Agaricus bisporus

ABSTRACT A 33-year-old male with house dust mite allergic rhinitis and asthma reported an episode of facial and lip angioedema, dyspnea, cough and dysphagia at the age of 25, minutes after eating a mushroom ( Agaricus bisporus ) pizza. He denied any drug intake, hymenoptera stings or other possible triggers, and no identifiable cofactors were present. Since then he avoided all types of mushrooms, however an accidental contact occurred with mushroom sauce that resulted in angioedema of the lip within minutes. The allergy workup included measurements of total IgE and specific IgE to mushroom, and skin prick test to aeroallergens sources, possible food allergen sources and mushroom extract, a prick to prick test with raw and cooked A. bisporus , in addition to a SDS-PAGE and immunoblotting assay. The study revealed a specific IgE to mushroom of 0.76kUA/L positive skin prick test to mushroom extract, and prick to prick test positive to white and brown A. bisporus (raw and cooked). The immunoblotting identified two IgE binding proteins with 10kDa and 27kDa. We report a case of A. bisporus anaphylaxis probably due to primary mushroom sensitization. We detected two IgE-reactive proteins with 10kDa and 27kDa as possible culprit allergens.

RESUMO Paciente do sexo masculino, 33 anos, com asma e rinite alérgica desencadeadas por ácaros, relatou episódio de angioedema facial e labial, dispneia, tosse e disfagia aos 25 anos, minutos após a ingestão de uma pizza de cogumelo ( Agaricus bisporus ). O paciente negou consumo de medicamentos, picadas de himenópteros, ou quaisquer outros possíveis desencadeadores ou cofatores que pudessem estar presentes. Desde então, evita todos os tipos de cogumelos, até a ocorrência de um contato acidental com molho de cogumelo, que resultou em angioedema labial minutos após. O estudo imunoalergológico incluiu doseamento de IgE total e específica para cogumelos, testes cutâneos para aeroalérgenos, possíveis alérgenos alimentares e extrato de cogumelos, teste prick to prick com A. bisporus cru e cozido e teste de SDS-PAGE immunoblotting . O estudo revelou IgE específica para cogumelos de 0,76kUA/L, teste cutâneo positivo para extrato de cogumelos e teste prick to prick positivo para A. bisporus branco e castanho (cru e cozido). O immunoblotting identificou duas proteínas de ligação de IgE, de 10kDa e 27kDa. Relatamos, assim, um caso de anafilaxia por ingestão de A. bisporus , provavelmente explicado por sensibilização primária a cogumelos. Detectamos duas proteínas IgE-reativas de 10kDa e 27kDa como os possíveis alérgenos responsáveis.