Intermittent Soft-Tissue Tumor Enlargement in Episodic Angioedema With Eosinophilia Syndrome.

ABSTRACT: Episodic hypereosinophilia and angioedema syndrome, also known as Gleich syndrome, is a rare entity characterized by recurrent episodes of eosinophilia, angioedema, urticaria, fever and weight gain with spontaneous resolution. It is classified as an idiopathic hypereosinophilic syndrome. Unlike other hypereosinophilic syndromes, it has a low risk for internal organ damage. We report the case of a 42-year-old male with a 28-year history of recurrent erythematous wheals and plaques and persistent hypereosinophilia. Physical examination revealed a well-defined subcutaneous nodule on his right lower limb that increased in size with each episode of angioedema. Histopathology evidenced a lipoma with intense eosinophil infiltration within the mature adipose tissue, while the specimen of the wheal revealed scarce perivascular and interstitial eosinophilic inflammatory infiltrate. Diagnosis of episodic angioedema with eosinophilia syndrome was made based on clinical and laboratory findings.

Disseminated Coccidioidomycosis as the First Presentation of a C-Terminal NFKB2 Pathogenic Variant: A Case Report and Review of the Literature.

INTRODUCTION: Although most cases of coccidioidomycosis are subclinical or self-limited respiratory disease, 1% lead to extrathoracic dissemination and become fatal, especially in patients with an associated immunodeficiency. Up to 30%-50% of patients with defects in cell-mediated immunity, those with AIDS and recipients of solid-organ transplants, may develop disseminated coccidioidomycosis (DC). Within the primary immunodeficiencies, an uncommon group is caused by C-terminal NFKB2 pathogenic variants. MATERIALS AND METHODS: We performed a literature search of core databases. Written informed consent for the study and for publication was obtained. CASE PRESENTATION: A 7-year-old Mexican girl, eldest of 3 sisters, no relevant family history, and a history of recurrent upper respiratory infections and alopecia totalis was admitted with DC involving pulmonary, soft tissue, skin, bone and joint compromise. The immunodeficiency assessment showed low IgM and NK cells. We found an NFKB2 de novo heterozygous nonsense mutation of c.2611C>T (p.Gln871*). She was treated with liposomal amphotericin B and itraconazole with surgical debridement. The clinical phenotype of this primary immunodeficiency is characterized by antibody deficiency and associated broncho-pulmonary predisposition to infection, but moreover also opportunistic infections and autoimmunity, most recognizable alopecia and adrenocorticotropic hormone-deficiency. After 1 year of her discharge, she continues under surveillance with antifungal therapy with itraconazole and replacement intravenous immunoglobulin until today. CONCLUSION: This is the first case report of DC in a patient with an NFKB2 pathogenic variant and it illustrates the importance of screening for primary immunodeficiencies in patients with disseminated fungal infections.

[Mexican clinical practice guidelines of immunotherapy 2011]. / Guía Mexicana de Práctica Clínica de Inmunoterapia 2011.

BACKGROUND: Several international guidelines on immunotherapy exist, but they only apply partially in Mexico. The Mexican guideline of immunotherapy dates from 1998. OBJECTIVES: To establish clinical recommendations and suggestions for Allergy residents and specialists for skin testing and allergen immunotherapy based on evidence and Mexican expert opinion, according to the GRADE system. METHODS: The guidelines were developed following the methodology of a guideline for clinical practice starting with the formulation of clinical questions, in the context of Mexican environmental conditions and morbidity, with the participation of allergists from all regions of the country. External validation was obtained. Its development followed three steps: 1. formulation of 24 clinical questions. 2. Search for consensus on the answers among members of the Regional chapters of both Mexican Colleges of Allergists (CMICA and COMPEDIA) during regional meetings. 3. Literature search for articles related to the questions and grading of its quality according to GRADE. RESULTS: Based on the regional consensus, 116 articles and the safety, patient acceptance/ comfort and cost clinical recommendations and suggestions were developed on basic aspects of skin testing, subcutaneous and sublingual immunotherapy (patient preparation, vial preparation and application schedules) and the treatment of eventual adverse reactions. CONCLUSIONS: A clinical guideline was developed respecting particular methodology, validated by CMICA and COMPEDIA for its implementation among Mexican allergists. Several aspects deserve further study to improve scientific evidence. KEYWORDS: Allergen immunotherapy, subcutaneous immunotherapy, sublingual immunotherapy, skin testing, allergy diagnosis, rhinitis, asthma, atopic dermatitis, house dust mite, pollens, anaphylaxis, adrenaline, Mexico.

[Prevalence of hypersensitivity to Cupressus pollen in allergic patients in Monterey, Mexico]. / Prevalencia de hipersensibilidad al polen de Cupressus en pacientes alérgicos en Monterrey, México.

BACKGROUND: Cupressus pollen is one of the main causes of allergic respiratory disease in North America. In a previous study of pollen concentration in Monterrey, Cupressus occupied the 3rd place in frequency. OBJECTIVE: To know the sensitization to Cupressus in patients treated at our center. METHODS: We conducted an observational, cross-sectional and prospective study, with a questionnaire to assess potential exposure and allergic symptoms to pollen, skin tests with allergen extract of Cupressus 1:20 dilution, and skin tests with others 36 aeroallergens in our hospital. RESULTS: We did Cupressus skin test in 256 patients (136 women), 130 younger than 18 years old and 126 adults. The skin test was positive in 39 patients (15.2%). Of the 36 aeroallergens tested, Cupressus occupied the 7th place in frequency. Of the 130 under 18 years, only 10 (7.7%) had a positive skin test. Among the other 129 adults, 29 (22.5%) had a positive skin test (p = 0.001). Patients with a positive skin test Cupressus had at least one positive skin test to other aeroallergen in 97% of cases (p = 0.001). CONCLUSIONS: Cupressus sensitization in our study group was high (15.2%). The routine use of allergen extract of Cupressus should be considered in patients with respiratory allergy. However, it is necessary to determine precisely the prevalence of sensitization and its possible clinical implications.

[Atypical presentation of hereditary angioedema. A report of a case and literature review]. / Manifestación atípica de angioedema hereditario. Comunicación de un caso y revisión de la literatura.

Hereditary angioedema is an uncommon disorder mainly caused by defects of the gene for C1 inhibitor. These patients present recurrent edema episodes in the different regions of the body, including larynx edema in some cases. Low plasma levels of C1 inhibitor confirm the diagnosis. It is important to establish an early diagnosis and prompt treatment to this disorder due to its potential of fatal consequences and multiple alterations in the quality of life that have been associated with. The indicated treatment is substitutive therapy of C1 inhibitor concentrate. We present the case of a patient a 27 year-old female with a history of seven years of evolution, with daily periorbital, upper and lower extremities and labial edema episodes who was classified as C1 inhibitor deficiency type II. She began treatment with attenuated androgens in progressive increased doses with poor response, appearing torpid evolution without a favorable response. This case corresponds to a very atypical presentation of C1 inhibitor deficiency with daily symptoms, unlike the typical intermittent course of the classic disease.