Case Report Esophageal Rupture as a Primary Manifestation in Eosinophilic Esophagitis

Eosinophilic esophagitis (EoE) is a chronic inflammatory process characterized by symptoms of esophageal dysfunction and, histologically, by eosinophilic infiltration of the esophagus. In adults, it commonly presents with dysphagia, food impaction, and chest or abdominal pain. Chronic inflammation can lead to diffuse narrowing of the esophageal lumen which may cause food impaction. Endoscopic procedures to relieve food impaction may lead to complications such as esophageal perforation due to the friability of the esophageal mucosa. Spontaneous transmural esophageal rupture, also known as Boerhaave's syndrome, as a primary manifestation of EoE is rare. In this paper, we present two adult patients who presented with esophageal perforation as the initial manifestation of EoE. This rare complication of EoE has been documented in 13 other reports (11 adults, 2 children) and only 1 of the patients had been previously diagnosed with EoE. A history of dysphagia was present in 1 of our patients and in the majority of previously documented patients. Esophageal perforation is a potentially severe complication of EoE. Patients with a history of dysphagia and patients with spontaneous esophageal perforation should warrant an evaluation for EoE

The perceived obstacles in performing patch test to detect allergic contact dermatitis: a comparison between community allergists and directors of allergy training

Background: Patch testing (PT) is the gold standard test to detect allergic contact dermatitis. Despite its usefulness, it is not universally performed in allergy practices. Objectives: To determine obstacles in performing PT and to evaluate the differences in perception of these obstacles between community allergists and directors of training programs. Methods: Two anonymous Web-based questionnaires were distributed to 65 program directors (PDs) across the United States and to 200 community allergists in the mid-Atlantic region. Program directors and community allergists were categorized based on PT performance. Community allergists were categorized based on type of practice. Comparisons between categories were made using the ?2 test. Results: Perceived obstacles among community allergists and PDs were similar; this remained unchanged regardless of performing PT. When community allergists in solo practice were compared with those in group private practice, the difference in perception was significant (P < .01). Previous training in PT among PDs and community allergists was associated with a higher likelihood of performing PT. Conclusions: Previous training in PT among PDs and community allergists was associated with a higher likelihood of performing PT. We recommend that training in the application and interpretation of PT for diagnosis of allergic contact dermatitis should be an integral part of the curriculum of allergy fellowship training programs and should also be incorporated into the continuing medical education program of practicing allergists. Furthermore, although allergists may continue to rely on dermatologists for more comprehensive PT, use of the thin-layer rapid use epicutaneous test is a simple screening tool that should be available to all allergy practices

Novel STAT3 Gene Mutation Related Hyper-IgE Syndrome Misdiagnosed as Hidradenitis Suppurativa.

Although Hyper-IgE Syndrome (HIES) is a rare immunodeficiency disorder, presenting symptoms may be as common as lung and skin infections. Symptoms are usually nonspecific such as recurrent abscesses, folliculitis, and pneumonias along with skeletal abnormalities. Careful history of susceptibility to skin and lung infections, thorough family history, and findings on physical exam can guide towards the diagnosis of this often-eluded condition. Early optimization of therapy with prophylactic antibiotics can prevent recurrent infections and future complications and improve quality of life and longevity of survival. We present a case of a young female with Hyper-IgE Syndrome with a novel mutation in STAT 3 gene who initially presented with long standing history of intractable skin abscesses being managed as Hidradenitis Suppurativa

International Survey on Evaluation and Management of Eosinophilic Esophagitis

Background Recommendations regarding evaluation and management of eosinophilic esophagitis (EoE) remain incompletely defined. This survey assesses: how providers across the world diagnose, evaluate, and treat EoE and how educational activities affect management.Methods A web-based survey was sent to the members of World Allergy Organization, American College of Allergy, Asthma, and Immunology, and American Academy of Allergy, Asthma, and Immunology. A χ2 analysis compared responses based on personal and practice demographics and participation in educational activities.Results Of the 200 respondents, 68.5% were from the United States. The majority were allergists, who require biopsy to diagnose EoE, perform allergy testing, and obtain follow-up biopsy after treatment. The following variables had significant differences: (1) US practitioners were more likely to test for immediate-type hypersensitivity to foods and obtain follow-up endoscopic biopsies after the initial treatment; (2) Practitioners encountering patients with EoE more frequently were more likely to ask about personal and family history of atopy, test for immediate-type hypersensitivity to aeroallergens and foods, and recommend follow-up biopsy after treatment; and (3) Practitioners who participate more often in EoE workshops were more likely to perform patch testing for foods, while attendance at EoE lectures increased EoE management confidence.Conclusions Diagnostic and management strategies differ based on practice location, EoE patient load, and participation in educational activities. Practitioners who attend more EoE lectures are more confident managing EoE. Keywords: eosinophilic esophagitis, evaluation, treatment, educational activities, international, surve

Esophageal Rupture as a Primary Manifestation in Eosinophilic Esophagitis

Eosinophilic esophagitis (EoE) is a chronic inflammatory process characterized by symptoms of esophageal dysfunction and, histologically, by eosinophilic infiltration of the esophagus. In adults, it commonly presents with dysphagia, food impaction, and chest or abdominal pain. Chronic inflammation can lead to diffuse narrowing of the esophageal lumen which may cause food impaction. Endoscopic procedures to relieve food impaction may lead to complications such as esophageal perforation due to the friability of the esophageal mucosa. Spontaneous transmural esophageal rupture, also known as Boerhaave’s syndrome, as a primary manifestation of EoE is rare. In this paper, we present two adult patients who presented with esophageal perforation as the initial manifestation of EoE. This rare complication of EoE has been documented in 13 other reports (11 adults, 2 children) and only 1 of the patients had been previously diagnosed with EoE. A history of dysphagia was present in 1 of our patients and in the majority of previously documented patients. Esophageal perforation is a potentially severe complication of EoE. Patients with a history of dysphagia and patients with spontaneous esophageal perforation should warrant an evaluation for EoE

Effect of Topical Beclomethasone on Inflammatory Markers in Adults with Eosinophilic Esophagitis: A Pilot Study

Background Topical corticosteroids have proven efficacy in the treatment of eosinophilic esophagitis (EoE) and are considered the cornerstone of therapy. Objective To evaluate the effect of topical beclomethasone dipropionate (BDP) therapy on clinical outcomes, esophageal eosinophilia, and other markers of inflammation in patients with EoE. Methods Nine subjects with a biopsy-proven diagnosis of EoE were enrolled. In a cross-over design, the subjects were randomly assigned to a sequence of BDP and placebo. Treatment periods were 8 weeks, with a 4-week washout period. The subjects had endoscopic biopsies and blood tests at baseline and after each treatment period. They were instructed to maintain a diary of symptoms. Immuno-histochemical studies were performed for interleukins IL-4, IL-5, IL-13, granulocyte-macrophage colony-stimulating factor (GM-CSF), and transforming growth factor (TGF) beta. Reverse transcription polymerase chain reaction was performed for IL-3, IL-4, IL-5, IL-10, IL-13, IL-17F, IL-25, IL-33, chemokine ligands (CCL)2, CCL5, CCL11, GM-CSF, and TGF-beta levels. The mast cell tryptase (MCT) level was measured in esophageal tissues. Results BDP led to a significantly larger decrease in esophageal eosinophilia compared with placebo, but there was no significant change in peripheral eosinophilia and high-sensitivity C-reactive protein between the two groups. The study was not powered enough for us to report a significant improvement in clinical symptoms. There was a significant decrease in tissue IL-13 and MCT levels from baseline to the end of treatment between the treatment and placebo groups. Mean fold decreases in cytokine expression between the baseline and treatment groups were observed for IL-17F, IL-25, CCL2, and CCL5. Conclusion Treatment with topical BDP was associated with significant decrease in esophageal eosinophilia, MCT and IL-13. BDP is a potential alternative to fluticasone propionate and budesonide for treatment of EoE. Larger studies are needed to validate these findings

Development of a Core Outcome Set for Therapeutic Studies in Eosinophilic Esophagitis (COREOS).

BACKGROUND Endpoints used to determine treatment efficacy in eosinophilic esophagitis (EoE) have evolved over time. With multiple novel therapies in development for EoE, harmonization of outcomes measures will facilitate evidence synthesis and appraisal when comparing different treatments. OBJECTIVE To develop a core outcome set (COS) for controlled and observational studies of pharmacologic and diet interventions in adult and pediatric patients with EoE. METHODS Candidate outcomes were generated from systematic literature reviews and patient engagement interviews and surveys. Consensus was established using an iterative Delphi process, with items voted on using a 9-point Likert scale and with feedback from other participants to allow score refinement. Consensus meetings were held to ratify the outcome domains of importance and the core outcome measures. Stakeholders were recruited internationally and included adult and pediatric gastroenterologists, allergists, dieticians, pathologists, psychologists, researchers, and methodologists. RESULTS The COS consists of four outcome domains for controlled and observational studies: histopathology, endoscopy, patient-reported symptoms, and EoE-specific quality of life (QoL). A total of 69 stakeholders (response rate 95.8%) prioritized 42 outcomes in a two-round Delphi process and the final ratification meeting generated consensus on 33 outcome measures. These included measurement of the peak eosinophil count, EoE Histology Scoring System, EoE Endoscopic Reference Score, and patient-reported measures of dysphagia and QoL. CONCLUSIONS This interdisciplinary collaboration involving global stakeholders has produced a COS that can be applied to adult and pediatric studies of pharmacologic and diet therapies for EoE, which will facilitate meaningful treatment comparisons and improve the quality of data synthesis