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A 63-year-old caucasian male with history of tonsil cancer, under induction chemotherapy, reported food intolerance and vomiting with duration of one month. Symptoms had increased over the last days and were associated with a weight loss of 10 Kg during the past three months. The patient lived all of is life in an urban environment. General physical examination revealed cachexia and dehydration. Gastrointestinal symptoms persisted despite intravenous pantoprazole, prokinetic drugs and nasogastric tube insertion. On investigation, patient presented normocytic and normochromic anemia (9.2 g/dL), lymphocytosis (11.78 x109/L) with neutrophilia (70.7%) and eosinophilia (7.7%), hypoalbuminemia (2.8 g/dL) and elevated C-reactive protein (25.5 mg/dL). Upper endoscopy revealed deformation of bulb and second part of the duodenum with mucosal edema, superficial ulceration and friability (Figure 1a). Biopsies were taken from the bulb and second portion of the duodenum. Computer tomography demonstrated gastric distention, duodenal wall thickening and lumen narrowing in the second and third portion of the duodenum (Figure 2). These findings were indicative of a functionally relevant duodenum stenosis. Histopathologic evaluation of biopsy specimens from the duodenum revealed moderate accumulation of eosinophilic granulocytes and nematode larvae within mucosal crypts (Figure 1b). What is the diagnosis?, Competing Interests: The authors declare that they have no conflict of interest, (© Acta Gastro-Enterologica Belgica.)

Objective: The objective of this study was to analyze the histopathologic changes in recurrent nasal polyps (NPs) in terms of tissue inflammatory cells infiltration and mucosal remodeling., Methods: Thirty-five patients with primary NPs requiring a revision surgery during follow-up and a matched control group of 35 primary NP patients without recurrence were retrospectively enrolled. Histopathologic examination was performed of tissue inflammatory cells, subepithelial edema, epithelial cell hyperplasia, basement membrane thickness, and fibrosis., Results: The mean eosinophil and neutrophil counts, proportions of eosinophil and neutrophil, as well as total inflammatory cell count were significantly higher in the recurrent group than those in the controls. Higher subepithelial edema was seen in the recurrent group. Within the recurrent group, the mean tissue eosinophil count and relative eosinophilia reduced from the first operation to revision surgery, whereas neutrophil count and proportion, and total inflammatory cell counts remained unchanged. Subepithelial edema and epithelial cell hyperplasia were significantly reduced after the first surgical procedure., Conclusion: NP recurrence is associated with higher inflammatory grade (especially in eosinophilia and neutrophilia). Surgical and corticoid treatments may have an impact on mucosal histopathologic changes but need further certification., (© 2021 S. Karger AG, Basel.)

Background: Aim of the study was to evaluate the association between celiac disease and eosinophilic oesophagitis/oesophageal eosinophilia in children., Methods: A total of 278 patients with celiac disease (mean age: 7.12 ± 4.64 years, M/F: 0.77) were involved in the study. The patients were evaluated retrospectively in terms of clinical, endoscopic and histopathological findings. The association between celiac disease and eosinophilic oesophagitis/oesophageal eosinophilia was determined., Results: According to Marsh classification system 6 (2.1%) of the patients were graded type 3A, 10 (3.5%) were type 3B, 262 (94.4%) were type 3C. The histopathological examination of oesophageal biopsy specimens of the patients revealed <15 eosinophils per high power field in only 4 (1.4%) patients. Two of these patients were positive for HLA DQ8, one was DQ2, and the other one was both DQ8 and DQ2. Tissue transglutaminase IgA level was above 300 U/mL in these patients. None of them had elevated serum total IgE levels, peripheral eosinophilia and history of atopic diseases. The gastrointestinal symptoms resolved and tissue transglutaminase IgA level of the patients were declined after 3 months of gluten-free diet., Conclusion: Although an association between celiac disease and eosinophilic oesophagitis/oesophageal eosinophilia have been postulated in recent years, no exact relationship was established in this study. This is the first study reporting the performance of follow-up GI endoscopy with biopsies revealing the resolution of oesophageal eosinophilia., (© 2021 John Wiley & Sons Ltd.)

Eosinophilic otitis media (EOM) is a difficult-to-treat otitis media (OM) characterized by eosinophilic accumulation in the middle ear mucosa and secretion. Associated sensorineural hearing loss can eventually lead to (functional) deafness. EOM is strongly associated with type 2 inflammation driven respiratory disease, i.e. asthma and chronic rhinosinusitis with nasal polyps (CRSwNP), for which biological treatment is available. This case report discusses a patient suffering from EOM with severe mixed hearing loss, nearing functional deafness. Dupilumab treatment resulted in complete and enduring remission of the EOM, enabling adequate hearing rehabilitation. Concurrent control of the comorbid asthma and CRSwNP was obtained. Laryngoscope, 131:2649-2651, 2021., (© 2021 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Competing Interests: The authors declare no conflict of interest.

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel vasculitis characterized by asthma, hyper-eosinophilia, and progressive multiorgan involvement. EGPA is traditionally treated using glucocorticoids, either alone or in combination with conventional immunosuppressants. Mepolizumab, a novel anti-interleukin (IL)-5 agent, has been approved as an add-on therapy for adult patients with EGPA. The recommended dose of mepolizumab is 300 mg (subcutaneous [SC]) every 4 weeks., Case Study: The present report discusses three cases of refractory and/or relapsing EGPA in patients regularly taking a stable dose of prednisolone, all of whom were successfully treated with a lower-than-recommended dose of mepolizumab (100 mg SC, every 4 weeks)., Results: Treatment with a low dose of mepolizumab enabled us to gradually reduce glucocorticoid doses. In addition, patients treated with low doses of mepolizumab exhibited better asthma control and experienced sustained relapse-free periods. Responses to 100 mg of mepolizumab were comparable to those previously observed in patients taking the recommended dose of 300 mg., Conclusion: Our findings suggest that mepolizumab at a third of the recommended dose can achieve reasonable clinical efficacy in the long-term treatment of EGPA in some patients. Initiation of mepolizumab therapy in the early, eosinophilic phase of EGPA-which is characterized by asthma, marked blood eosinophilia, pulmonary infiltrates, and sinonasal abnormalities-may help to prevent the deleterious side-effects of long-term glucocorticoid use while reducing the cost of EGPA treatment.

Background: There is limited information about peripheral blood eosinophilia (PBE) and airway obstruction in sarcoidosis. Since pulmonary sarcoidosis affects the airways, it is often confused with asthma. The aims of the study are to investigate airway obstruction and PBE in sarcoidosis patients and to examine the similarity of clinical presentation with asthma., Methods: The patients matching the ATS/ERS/WASOG diagnosis criteria and were between 18 and 80 years of age were included consecutively between 2018 and 2020. Other diseases causing granulomas were excluded., Results: A total of 84 patients were included of which 26 (31%) had a PBE level of ≥300 µL with no significant difference seen between sarcoidosis stage and PBE (p > 0.05). A significant (p < 0.05) decrease was only seen in FEV1 as the stage of sarcoidosis progressed. Respectively 31 (36.9%), 12 (14.3%) and 4 (4.8%) patients had an obstructive, restrictive and mixed respiratory function disorder. Twenty-four (28.6%) subjects with sarcoidosis had history of asthma. Spring fever, eczema, and skin/nose allergy were noticed in 17 (20.2%) of the patients., Discussion: Mild PBE may be seen in sarcoidosis. Patients applying with PBE, airway obstruction, bronchial hyperreactivity along with spring fever, eczema, skin/nose allergy, wheezing, chest tightness, shortness of breath and cough may be also evaluated in terms of sarcoidosis.

Background: Growing evidence suggest that type 2 immune effectors play a role in solid organ transplantation. The aim of this study was to evaluate the impact of blood count eosinophils (BCEo) on immunological outcomes in kidney transplant recipients with stable graft function after 3 months post-transplant., Method: We performed cause-specific Cox model considering BCEo, the use of calcineurin inhibitors and systemic corticoids as time-dependent explicative variables on a prospective cohort of 1013 kidney transplant patients who experienced kidney allograft rejection and/or the appearance of de novo donor specific antibodies after excluding common causes of increased BCEo.., Findings: BCEo ≥ 0.3 G/L was associated with a 3-fold increased risk of rejection independent of immunosuppressive regimen after 3 months post-transplant in patients without pre-transplant DSAs and with CNI-based immunosuppression. No association between BCEo either with donor specific antibodies or graft survival was noticed., Interpretation: These observations in this large cohort support the hypothesis of eosinophils in allo-immunity in human and claim for further mechanistic research., Funding: This study was supported by the French National Research Agency, The "Institut de Recherche en Santé Respiratoire des Pays de la Loire" and the University hospital of Nantes., Competing Interests: Declaration of Competing Interest The authors of this manuscript have no conflicts of interest to disclose as described by EBioMedicine., (Copyright © 2021 The Author(s). Published by Elsevier B.V. All rights reserved.)

Background: Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome is a form of autoimmune hepatitis with cholestatic features and is characterized by negative anti-mitochondrial antibody and cholangiographic changes on magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. Peripheral blood hypereosinophilia in conjunction with autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome has not been reported yet. Here we present a case of autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome with hypereosinophilia., Case Presentation: A 33-year-old Iranian man with the fatigue, jaundice, elevated liver enzymes and alkaline phosphatase, and hypereosinophilia was referred to our hospital. Viral and autoimmune hepatitis were excluded, and secondary workups for hypereosinophilia were all negative. Magnetic resonance cholangiopancreatography showed beaded appearance of intra- and extrahepatic biliary tree, and liver biopsy revealed interface hepatitis. Therefore, the diagnosis of autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome was made, and prednisolone, azathioprine, and ursodeoxycholic acid was initiated. His jaundice and peripheral blood eosinophilia resolved after 2 weeks, and he became completely asymptomatic., Conclusion: Eosinophils might contribute to the clinical presentation and disease complications., (© 2021. The Author(s).)

Objectives: This study aims to investigate the prevalence of persistent eosinophilia and associated organ complications in Thai patients with systemic sclerosis (SSc)., Patients and Methods: This post-hoc study included 107 adult patients (23 males, 84 females; mean age: 50.4±11.6 years; range, 18 to 79 years) diagnosed with SSc between November 2013 and June 2017. Eosinophilia was defined as an absolute eosinophil count of >500/μL or a percentage count of >7%. Eosinophil levels collected at every visit over one year were categorized as persistently high (PH), persistently low (PL), high-to-low (HL), low-to-high (LH), or variable levels (VL). The study compared variables between PH and non-PH (PL+HL+LH+VL) groups. The patients with baseline eosinophilia were also identified and compared with the non-eosinophilia group., Results: The median disease duration was 3.2 years. Of the patients, 79.4% had diffuse cutaneous SSc and 76.7% had anti-Scl-70 positivity. A total of 11.2%, 66.4%, 1.9%, 8.4%, and 12.1% of the patients were categorized into the PH, PL, HL, LH, and VL groups, respectively. Compared to non-PH groups, the PH group had a higher prevalence of anti-centromere antibody (ACA), higher baseline percent predicted total lung capacity, and lower baseline C-reactive protein and creatine phosphokinase (p<0.05 for all). The ACA positivity (odds ratio [OR]: 18.5; 95% confidence interval [CI]: 1.64-208.46) was associated with PH. The patients with baseline eosinophilia (17.8%) had a higher prevalence of non-specific interstitial pneumonia with periodic eosinophilia at the time of diagnosis (100% vs. 6.5%, p<0.0001; OR: 4.667; 95% CI: 1.712-12.724)., Conclusion: The PH was seldom (11%) in patients with SSc compared to periodic eosinophilia, which was more prevalent (18%). It may be related to ACA positivity and better pulmonary outcomes, whereas periodic eosinophilia may involve interstitial lung disease., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2021, Turkish League Against Rheumatism.)

Introduction: Studies on eosinophilic gastroenteritis have identified broad spectrums of disease. We aimed to characterize subtypes of disease and ascertain outcomes of each group., Methods: This is a retrospective cohort study from a large tertiary medical center including 35 patients diagnosed with eosinophilic gastroenteritis from 2007 to 2018. We defined 2 groups of patients based on clinical and laboratory findings at presentation. Severe disease was defined as having weight loss at time of presentation, hypoalbuminemia at presentation, serosal disease involvement, or anemia at diagnosis. The remaining patients were labeled as mild disease group. We collected and compared demographic data, clinical features, laboratory findings, an allergy history, and disease course of both cohorts., Results: Among 35 patients with eosinophilic gastroenteritis, 18 patients met the criteria for severe disease and 17 patients for mild disease. Of the patients with severe eosinophilic gastroenteritis, 6 (38%) had remission without chronic symptoms, whereas 10 (63%) had chronic symptoms requiring chronic medical therapy. Of the mild group, 12 patients (80%) had disease remission without chronic medications. An allergy history was more common in the severe disease group (83%) compared with the mild disease group (45%). Prednisone and open capsule budesonide were the most commonly used treatment medications in both groups., Discussion: Patients with eosinophilic gastroenteritis may be characterized into 2 forms. Patients with weight loss at time of presentation, hypoalbuminemia at presentation, serosal disease involvement, or anemia at diagnosis were associated with a chronic disease course requiring chronic medications., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)

BACKGROUND Eosinophilic gastroenteritis is a broad classification of disease characterized by eosinophilic infiltration of the gastrointestinal tract in the absence of a stimulatory cause. Given the ability of eosinophilic gastroenteritis to affect the entire gastrointestinal tract, it can present in a variety of ways, from chronic intermittent pain to mechanical obstruction. We present a rare case in which eosinophilic gastroenteritis of the jejunum led to small bowel diverticulosis and volvulus, requiring surgery. CASE REPORT An 83-year-old woman with a history of chronic abdominal pain, nausea, and early satiety presented to our clinic after a thorough gastrointestinal workup and radiologic diagnosis of partial midgut volvulus. She underwent an exploratory laparotomy and was found to have normal rotational anatomy with prominent small bowel diverticulosis. A section of 70 cm of proximal jejunum was resected, encompassing all visible diverticula, and a primary anastomosis was performed. The patient recovered without complication. She was seen at follow-up with complete resolution of her presenting symptoms. CONCLUSIONS We propose that this patient's pathology was caused by chronic intermittent obstructions related to eosinophilic gastroenteritis, leading to repeated periods of increased intraluminal pressure and severe small bowel diverticulosis. This case highlights the importance of maintaining an index of suspicion for small bowel diverticulosis in the setting of chronic eosinophilic gastroenteritis.

Objective: To define the pattern of cardiac involvement in eosinophilic granulomatosis and polyangiitis (EGPA) and propose an algorithm for heart disease screening., Methods: This was a retrospective study of EGPA patients attending a specialized vasculitis clinic (1989-2016). Clinical characteristics and cardiovascular evaluation (CE) results (serum troponin, ECG, echocardiography and cardiac magnetic resonance) were collected and compared according to symptoms and inflammatory cardiac disease (ICD)., Results: A total of 131 EGPA patients were included, of whom 96 (73%) had undergone CE. The median (interquartile range) age was 50 (38-58) years and 36% showed ANCA+. Asthma preceded diagnosis by a median of 97 (36-240) months. Among the 96 patients who underwent CE, 43% were symptomatic, with dyspnea (47%) and chest pain (29%) being the predominant symptoms. In asymptomatic patients, CE reported abnormalities in 45% of cases, with a subsequent earlier diagnosis (4 vs 11 months). Overall, 27 patients had EGPA-related ICD (EGPA-rICD) that was already present at diagnosis in 20 cases, preceded it in 2 cases and developed later in 5 cases. EGPA-rICD patients were younger (46 vs 50 years; P = 0.04), had more frequently abnormal ECG (30.8 vs 2.1%; P < 0.001), negative ANCA (85 vs 69%; NS), higher BVAS score (3 vs 1; P = 0.005), higher eosinophil count (5.60 vs 1.60 × 109/l; P = 0.029) and higher CRP (52 vs 15 mg/l; P = 0.017). Overall, 11% of cases with EGPA-rICD were asymptomatic., Conclusion: In our study, 45% of asymptomatic patients had an abnormal baseline cardiac evaluation, which allowed an earlier diagnosis of cardiac disease. We recommend prompt cardiac screening in all EGPA patients, instead of a symptoms-guided algorithm., (© Crown copyright 2021.)

Rationale: Eosinophilic enteritis (EE) is an immune-mediated antigen-driven disease that may lead to clinical symptoms and organ dysfunction and characterized by the presence of extensive eosinophilic infiltrates on histopathological examination of the intestinal mucosa., Patient Concerns: A 29-year-old man presented with a half-month duration of paroxysmal upper abdominal pain that gradually evolved into continuous pain accompanied by the urge to defecate., Diagnoses: Pathological findings of enteroscopy showed acute and chronic inflammation accompanied by eosinophilic infiltration (>20/ high-power field)., Interventions: The patient was initially treated with IV infusion of dexamethasone 10 mg per day for 3 days, which was reduced to 7.5 mg per day for 2 days once pain relief was achieved. Upon discharged from our hospital, the patient was prescribed with oral prednisolone 30 mg per day, which was reduced by 5 mg per week for 6 weeks until discontinuation., Outcomes: The patient was relieved from the pain after receiving dexamethasone for 5 days, and he was maintained on oral prednisolone 30 mg per day upon discharge from the hospital. On the day of discharge, the eosinophil count and derived ratios were normal., Lessons: In patients with EE, the dynamic changes of the eosinophil count should be monitored. Clinicians must be aware that not all patients with EE have a history of allergies. In the management and treatment of the disease, multisite biopsies should be carried out if EE is suspected, and EE is responsive to steroid therapy., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Eosinophilia can occur due to a plethora of allergic, infective, neoplastic and idiopathic conditions. Hypereosinophilic syndrome (HES) is characterised by sustained eosinophilia and multiorgan dysfunction in the absence of an identifiable cause. It may range from a self-limiting condition to a rapidly progressive life-threatening disorder, of which ischaemic stroke is a rare presentation. Such episodes can rarely be the presenting manifestation, and may develop before any other laboratory abnormality or organ involvement. We report a case of HES presented with multiorgan (neurological and renal) involvement, managed successfully with steroids and cytoreductive therapy. High initial absolute eosinophil count can be a clue to the diagnosis and early treatment should be initiated in such patients, to prevent fatal outcomes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Severe eosinophilic asthma is a high-burden disease. Mepolizumab has been effective in several randomized clinical trials. However, such success might not be applicable to patients treated in usual clinical practice. The objectives of this article are to evaluate the efficacy of mepolizumab in severe uncontrolled eosinophilic asthma under usual clinical practice, and to determine characteristics associated with the response to this treatment., Methods: We have conducted a retrospective, multicentre study, including all adult patients with severe uncontrolled eosinophilic asthma in Galicia, Spain, on whom mepolizumab treatment was started before June 2020, at least 6 months before the time of inclusion, and had received at least one dose of the drug. Patient characteristics, clinical data, respiratory function and comorbidities were collected at baseline and at the 6-month-follow-up. Responders and super-responders were defined according to clinical response and requirement of systemic corticosteroids., Results: 122 patients (mean age 58 years old) were included. In the follow-up treatment 6 months later, 75.4% of the patients were well-controlled, displaying a significant reduction in blood eosinophil counts (p < 0.001), hospital admissions and disease exacerbations (p < 0.001), and had their systemic glucocorticosteroid dose significantly reduced (p < 0.001). The inhaled corticosteroid dose was also lowered (p < 0.01) after 6 months of treatment. Around two-thirds had a clinically significant increase in FEV1, 95% of the patients were considered responders and 43% super-responders., Conclusion: In routine clinical practice, mepolizumab is effective in patients with severe eosinophilic asthma and it has a good safety profile., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rising morbidity amongst hospitalized patients. Whilst clinical protocols for the management of individual DRESS cases have been well established, determination of potential prevention of these cases by utilizing novel "avoidability" tools has remained unexplored. This retrospective study reviewed records of patients who presented to the emergency department of Weill Cornell Medicine-affiliated Hamad General Hospital, Doha Qatar with suspected DRESS syndrome. These cases were independently adjudicated (utilizing the RegiSCAR, and JSCAR tools) as DRESS-drug pairs by a team of two clinical pharmacists and two General Physicians. They were then rated for potential avoidability with the Liverpool adverse drug reactions avoidability tool (LAAT) by the same team of raters. A total of 16 patients satisfied RegiSCAR criteria for DRESS syndrome. The mean age of the study population was 41.5 years (SD ± 13.3). The study population was predominantly male (n = 12; [75%]). The median latent period from drug ingestion to clinical presentation was 14 days (interquartile range [IQR] 6.5, 29). The median RegiSCAR and J-SCAR scores were 6 (IQR 5, 6.8), 5 (IQR 4, 5.8) respectively. Utilizing the LAAT, about 60% of the DRESS syndrome-drug pairs were rated as "avoidable" ("probable" or "definite"). The overall Krippendorf's alpha with the LAAT was 0.81 (SE 0.10, CI 0.59-1.00); with an intraclass correlation coefficient (ICC) of 0.90 (CI 0.77, 0.96.). In a randomly selected cohort of DRESS syndrome-drug pairs, a significant proportion was potentially avoidable ("possibly" and "definitely") utilizing the LAAT. This will need validation by larger sample-sized prospective studies utilizing the updated LAAT proposed by this study., (© 2021. The Author(s).)

We describe a Caucasian man in his late 60s who was admitted to the intensive care unit (ICU) with a history of cardiogenic shock secondary to an acute myocardial infarction. The patient's baseline serum creatinine levels were 0.9-1 mg/dL. On day 7 of the admission treatment with quetiapine was initiated due to a delirium episode. The next day the patient developed an erythematous-maculopapular rash and fever, with eosinophilia in the blood count. Over the following days the patient experienced an acute deterioration of kidney function requiring continuous renal replacement therapy. The skin lesions and eosinophilia resolved after withdrawal of quetiapine and systemic steroid therapy was administered. The patient was discharged from the ICU with a serum creatinine level of 2.6 mg/dL. Three months later, blood tests showed no recovery of the kidney function. According to the Naranjo adverse drug reaction probability scale, this event would be classified as 'probable' DRESS syndrome and, based on the RegiSCAR scoring system, was classified as 'definite' DRESS syndrome., Competing Interests: Competing interests: None declared., (© European Association of Hospital Pharmacists 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

The objectives were to evaluate drug rash with eosinophilia and systemic symptoms syndrome due to sulfasalazine and to carry out the pharmacoeconomic assessment associated with this adverse drug reaction (ADR). A 37-year woman was presented with rashes, fever, cough, and dyspnea. In the past 3 months, she was on sulfasalazine for inflammatory polyarthritis and seronegative spondyloarthritis. The diagnosis was based on raised eosinophils count, breathing difficulty, and typical pattern of rashes. Significant improvement was seen after discontinuation of sulfasalazine and with the initiation of parenteral corticosteroids. The casualty of this ADR was "probable" based on RegiSCAR, WHO, and Naranjo casualty assessment scales. Preventability, severity was assessed and total cost for management of the ADR was found to be ' 12,126. Thus, ADRs not only adds to patient sufferings but also increase the economic burden. Health-care providers need to be made aware of potentially fatal ADRs associated with sulfa drugs and should be keen to report such ADRs to drug safety authorities., Competing Interests: None

Sclerosing mucoepidermoid carcinoma (SMC) is described as a "sclerosing variant" of mucoepidermoid carcinoma, and it is characterized by dense fibrosis and sclerosis of the stroma. SMC with eosinophilia (SMCE) is another and more rare subtype characterized by eosinophilia in addition to the sclerotic stroma common to SMC. However, unlike SMC, SMCE is not listed in the current 4th edition of WHO classification. Here, we describe three cases: one SMC in the parotid gland, one SMCE in the submandibular gland and one SMCE in the minor salivary gland of the oral cavity. The patients included a 71-year-old Japanese male, a 74-year-old Japanese female, and an 81-year-old Japanese female. They each complained of mass formation and underwent surgical resection. Histologically, the tumors mainly consisted of squamous cells with scarce keratinization that formed irregular large and small nests along with cystic structures containing mucous cells against the background of sclerotic stroma. One oral SMCE showed fine nesting and trabecular invasion. The two SMCEs included dense aggregates of eosinophils as well as more prominent lymphoid infiltration. Fluorescence in situ hybridization for MAML2 confirmed split signals in SMC, but not in SMCE., (© 2021. The Japanese Society for Clinical Molecular Morphology.)

Eosinophilic angiocentric fibrosis (EAF) is a rare and slowly progressive disease, which usually involves the sino-nasal structures and upper respiratory tract. It is a fibroinflammatory lesion with an unclear etiology. Recent literature suggests a relation to rheumatic or immunological disorders. Therefore, immunophenotypic workup is critical when suspected. We report a case of a 32-year-old man complaining of nasal obstruction lasting more than 2 years. Nasal endoscopy and computed tomography showed a deviated septum with bilateral soft tissue swelling. During the septoturbinoplasty, a submucosal mass with severe adhesion was observed beneath the septal flap. The mass was completely removed. Dense stromal fibrosis with eosinophil-rich inflammatory cell infiltration was found on histologic examination and the patient was diagnosed with EAF. In addition, we reviewed the pathologic diagnostic criteria, differential diagnosis, and management of EAF.

Background: Eosinophilic cystitis is a rare inflammatory disease of the bladder characterized by eosinophilic infiltration of the bladder wall. Most Eosinophilic cystitis cases present with mucosal lesions of the urinary bladder. We present a very rare case of large mass-forming eosinophilic cystitis, involving the inside and outside of the bladder associated with an infected urachal cyst., Case Presentation: A 59-year-old man presented with gross hematuria, fever, dysuria, and suprapubic pain. Computed tomography showed a heterogeneously enhancing mass that measured 7.6 cm × 4 cm located on the anterosuperior portion of the bladder with an internal fluid collection. Cystoscopy revealed a raspberry-like mass lesion on the bladder dome. Transurethral resection of the bladder was initially performed. The mass lesion protruding from inside the bladder was removed, and pus-like fluid was drained. The pathologic diagnosis was eosinophilic cystitis. Follow-up computed tomography showed a remnant mass outside the bladder and urachal cyst. To eliminate the remnant lesion, robot-assisted partial cystectomy was performed. The patient showed no evidence of recurrent disease on follow-up cystoscopy and computed tomography for up to 2 years., Conclusions: Clinicians should consider the possibility of eosinophilic cystitis in patients who present with hematuria, fever, and suprapubic pain and have both intravesical and extravesical masses., (© 2021. The Author(s).)

Eosinophilic fasciitis (EF) and generalized morphea (GM) are rare and difficult-to-treat sclerosing skin diseases which may occur in association with hematologic disorders. We present a 66-year-old man with EF and associated Waldenström macroglobulinemia who received combination therapy with rituximab (375mg/m2 every other week, gradually extended to every eight weeks), prednisolone (1.25-30mg/d), and methotrexate (7.5-15mg/w). Three months after rituximab initiation, his skin condition improved steadily accompanied by a significant improvement in joint mobility with only mild and transitory flares (observation period: 59 months under treatment with rituximab). To date, there are five case reports on rituximab treatment of EF/GM with an association to hypergammaglobulinemia in three of those cases. Therapy effected significant improvement in four patients. Our case adds to the hitherto limited evidence that rituximab may be a promising therapeutic strategy for EF/GM in association with hypergammaglobulinemia.

Late-onset circulatory collapse (LCC) in preterm infants is presumably caused by relative adrenal insufficiency. Because eosinophilia is known to be associated with adrenal insufficiency, we attempted to clarify the relation-ship between eosinophilia and LCC in preterm infants. We divided the cases of the infants (born at < 28 weeks' gestation) admitted to our neonatal intensive care unit in 2008-2010 into 2 groups: those diagnosed with LCC that received glucocorticoids (LCC group), and those who did not receive glucocorticoids (control group). We compared eosinophil counts between the 2 groups and between before and after glucocorticoid treatment in the LCC group. A total of 28 infants were examined: LCC group (n = 12); control group (n = 16). The peak eosin-ophil counts of the LCC group were significantly higher than those of the control group (median: 1.392 × 109/L vs. 1.033 × 109/L, respectively; p = 0.02). Additionally, in the LCC group, the eosinophil counts declined significantly after glucocorticoid treatment (0.877 × 109/L vs. 0.271 × 109/L, p = 0.003). Eosinophil counts in the LCC group were significantly higher than in the control group and decreased rapidly after gluco-corticoid treatment. These results indicate that eosinophilia may be a factor associated with LCC caused by adrenal insufficiency., Competing Interests: No potential conflict of interest relevant to this article was reported.

Background: The association of bronchiectasis (BE) in patients with severe eosinophilic asthma (SEA) is quite frequent. Mepolizumab is a well-recognized treatment for SEA; we aim to evaluate its effectiveness in SEA patients with and without BE in real-life., Methods: We performed a single-center retrospective pilot study, including patients with SEA treated with mepolizumab for one year. Asthma control test (ACT), lung function, annual exacerbations rate, oral corticosteroid dosage, FeNO, chronic mucous secretions, blood and sputum eosinophils were recorded at baseline and after 6 and 12 months., Results: we included 32 patients (mean age: 52.3 ± 10, 59% female). 50% showed co-presence of bronchiectasis, (SEA + BE). Significant improvements were found in ACT [(13.8 ± 4.6 to 20.7 ± 4.1, p = 0.0009) and (13 ± 4.8 to 20.7 ± 4.6, p = 0.0003)], annual exacerbations rate [from 7 (4-12) to 0 (0.00-0.75) and from 8 (4-12) to 0 (0-1), p < 0.0001], and blood eosinophils count [748 cells/μL (400-1250) vs. 84 cells/μL (52.5-100), and from 691 cells/μL (405-798) vs. 60 cells/μL (41-105), p < 0.0001] in SEA and SEA + BE group respectively, already after 6 months of treatment. A reduction in daily oral corticosteroids intake at 12 months was shown [from 15 mg (0-25) to 0 mg (0-0), p = 0.003 and from 8.8 mg (0-25) to 0 mg (0-0) (p = 0.01)] in both SEA and SEA + BE, respectively. Similar results were found, comparing SEA + BE patients based on the severity of bronchiectasis., Conclusions: Mepolizumab effectively improves asthma symptoms control, reducing annual exacerbations and corticosteroid intake in all patients with SEA, even in the subgroup with coexisting bronchiectasis, independently of their severity., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid in a 46-year-old woman., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Acute myocarditis is an inflammatory disease of the heart mostly diagnosed in young people, which can present as sudden death. The etiology includes infectious agents (mostly viruses), systemic diseases and toxins. We aim to characterize infants and children with myocarditis at post-mortem presenting as sudden deaths., Methods: Retrospective evaluation of 813 post-mortems in infants and children dying suddenly and unexpectedly between 2009-2019. Data retrieved included histological features, microbiology and clinical history., Results: 23 of 813 post-mortems reviewed corresponded to acute myocarditis and 1 to dilated cardiomyopathy related to remote Parvovirus infection. PCR identified enterovirus (7), parvovirus (7 cases, 2 also with HHV6 and 1 case with EVB), Influenza A (1), Parainfluenza type 3 (1). Two cases corresponded to hypersensitivity myocarditis, 1 was Group A Streptococcus and 5 idiopathic myocarditis. Enterovirus was frequent in infants (7/10), and in newborns was associated with meningoencephalitis or congenital myocarditis. More than 50% were less than 2 years of age and all remained clinically unsuspected., Conclusion: Myocarditis represents almost 3% of all sudden pediatric deaths. Enterovirus and parvovirus were the most common viruses. This retrospective analysis showed that patients experienced viral symptoms but remained unsuspected, highlighting the need for more clinical awareness of myocarditis.

A 28-year-old woman was referred to our hospital for liver dysfunction and neck pain. Blood tests revealed elevated liver enzymes and eosinophilia. Ultrasonography, computed tomography, and magnetic resonance imaging showed a mass lesion near the hepatic hilus. The tumor was considered to be an inflammatory pseudo-tumor or malignancy. A liver-mass biopsy was performed and led to a diagnosis of inflammatory pseudo-tumor. In the present case, a markedly elevated eosinophil count was a characteristic clinical feature, and the patient underwent steroid therapy. Treatment resulted in a reduced eosinophil count, improved neck symptoms, and disappearance of the inflammatory pseudo-tumor.

This is a short report describing the clinical presentation, investigations, treatment and outcome of a patient living in the Jamaican countryside who was diagnosed with a live, motile subfoveal helminth identified as Angiostrongylus cantonensis on optical coherence tomography at the University Hospital of the West Indies Eye Clinic. This is the second documented case in Jamaica. Early recognition of angiostrongyliasis is important as it may manifest as eosinophilic meningitis which can be fatal without prompt treatment.

Eosinophilic fasciitis (EF) is a rare connective tissue disease that causes inflammation and fibrosis of the fascia, inducing pain and motor dysfunction. Characteristic skin manifestations, such as edema, erythema, induration, peau d'orange appearance, and the groove sign, are of diagnostic significance and observed in the majority of patients with EF. We herein report a case of EF without these characteristic skin manifestations. A 66-year-old Japanese woman developed progressive limb pain and motor dysfunction. No skin changes were observed. We diagnosed the patient with EF based on the clinical course, magnetic resonance imaging, and en bloc biopsy containing fascia and muscle. Oral prednisolone therapy markedly attenuated limb pain and motor dysfunctions. Through a systemic search of the medical literature, we retrieved 4 juvenile cases and 8 adult cases of EF without characteristic skin manifestations during the clinical course. We herein present a systemic review on EF without skin manifestations and discuss differences between the two proposed sets of diagnostic criteria of EF.

Objective: To determine whether myringoplasty can be an effective tool to control eosinophilic otitis media and improve hearing., Methods: Severity scores for patients with eosinophilic otitis media with or without myringoplasty were retrospectively analyzed. Surgical outcomes were assessed and compared to those of patients with control: chronic otitis media with tympanic membrane perforation., Results: The average eosinophilic otitis media severity score for the patients with myringoplasty was significantly lower after than before surgery (P = 0.004), although the score for the group without myringoplasty remained stable during the study period. The success rate for graft uptake was 87.5%, and a statistically significant improvement was observed in the postoperative air conduction hearing level within the speech range (mean ± standard deviation; 41.5 ± 12.3 to 34.6 ± 9.22 dB; P = 0.0026) for patients with eosinophilic otitis media., Conclusion: Myringoplasty contributed to decreased postoperative eosinophilic otitis media severity scores and should be considered to protect the patients from recurrent bacterial infections and pathogenic antigen parasitism in the middle ear., (Copyright © 2020. Published by Elsevier B.V.)

We present the case of a 56-year-old woman diagnosed with primary biliary cholangitis (PBC). She has continuously taken 600 mg/day of ursodeoxycholic acid. Edema of the lower limbs manifested on July 20, 20XX; after 2 weeks, she manifested rapid weight gain and nettle rash on the limbs and trunk. She was admitted to our hospital on August 22. She had marked eosinophilia, hypoalbuminemia, anemia, non-pitting lower limbs edema, and nettle rash of the limbs and the trunk. We ruled out other diseases that may have caused the edema and suspected her with episodic angioedema with eosinophilia (EAE). The peripheral blood eosinophil count rapidly decreased after the administration of 30 mg prednisolone. The edema and nettle rash improved on the 7th day of admission, and the hypoalbuminemia and anemia improved on the 14th day. Prednisolone was tapered and discontinued, and there was no relapse of edema. We revised our diagnosis to non-recurrent EAE. She was diagnosed with asymptomatic PBC; therefore, anemia and hypoalbuminemia were considered not PBC but chronic inflammation and decrease in appetite. In this case, elevation of serum IgG4 was observed at onset and at remission. This suggests that IgG4 may be involved in the development of EAE in patients with chronic liver disease.

Background: Eosinophilic Otitis Media (EOM) is a relatively newly defined entity of recurrent and resistant otitis media., Objective: To perform a systematic review of otologic manifestations, diagnosis and management of eosinophilic otitis media (EOM)., Methods: 393 patients diagnosed with EOM of 26 studies met inclusion criteria and were assessed for demographics, otologic manifestations, diagnostic criteria fulfilled, and medical and surgical treatments., Results: Most common otologic manifestations were hearing loss (65%), otitis media with effusion (16%), tympanic membrane perforation (13%), and otorrhea (13%). 93% had a predominantly eosinophilic middle ear effusion, 95% had asthma, 85% had a highly viscous middle ear effusion, 71% had nasal polyposis, and 58% had resistance to conventional treatment. For treatment, 39% received intratympanic steroid injections, 33% received systemic steroids, 17% received steroid ear drops and 13% received a biological agent. 39% of patients underwent a surgical intervention with 26% receiving functional endoscopic sinus surgery and 18% receiving myringotomy with tube insertion. Success rates were highest with use of intratympanic steroids (45%), systemic steroids (26%), and biological agents (58%)., Conclusion: Intratympanic steroids show the most efficacy in treating EOM, and aggressive optimization of asthma may be beneficial in resolving otologic symptoms. Surgery should be reserved for refractory cases and complications.

Objectives: Eosinophilic otitis media (EOM) is an intractable otitis media mostly associated with bronchial asthma. Dupilumab, an anti-interleukin (IL)-4 receptor (R)α, is effective and has been approved for use in patients with moderate to severe bronchial asthma, atopic dermatitis and chronic rhinosinusitis with nasal polyposis, whose diseases are not controlled by previous treatments including other molecular targeted drugs. We aimed to assess efficacy of dupilumab in three EOM patients with associated bronchial asthma, who were poor responders to previous topical and systemic corticosteroid therapy and molecular targeted therapies., Patients and Methods: Three patients with severe, refractory EOM (two with a granulation type) associated with bronchial asthma received dupilumab as add-on therapy for at least 6 months. The efficacy of dupilumab therapy was evaluated using severity scores, symptom scores, hearing acuities, temporal bone computed tomography (CT) scores, and surrogate markers before and after therapy., Results: Severity scores in all patients were dramatically reduced to 2 points or less (full score: 16 points) after initiation of therapy. Air conduction hearing levels were improved in all patients. Temporal bone CT scores in two patients were reduced, and serum IgE levels in all three patients also decreased following therapy., Conclusion: We provide the first report that add-on dupilumab therapy was effective in patients with severe, refractory EOM who did not respond to the treatments including other molecular targeted therapy. Patients with severe middle ear mucosal change may benefit particularly from dupilumab therapy., Competing Interests: Declaration of Competing Interest We declare that we have no conflicts of interest to disclose., (Copyright © 2020. Published by Elsevier B.V.)

BACKGROUND Toxocariasis is an infection due to ingestion of the helminth parasite larvae found in dogs (Toxocara canis) or cats (Toxocara cati). Symptoms vary from being asymptomatic to shock, depending on the organ invaded by the parasite. However, cardiac involvement with shock in toxocariasis is very rare. CASE REPORT A 21-year-old woman without any history of underlying conditions visited the Emergency Department because of epigastric pain, vomiting, headache, and dizziness. Her blood pressure was 80/60 mmHg. Computed tomography (CT) of the brain showed no abnormal lesions. The abdominal-pelvic CT with contrast showed right pleural effusion, pericardial effusion, and focal ascites in the pelvic cavity. Laboratory tests revealed an elevation of eosinophils (40%) and cardiac enzymes (creatinine kinase-MB 27.6 ng/mL, high-sensitive cardiac troponin T 1.21 ng/mL). The transthoracic echocardiogram showed left ventricular systolic dysfunction (ejection fraction 44%) and moderate pericardial effusion. She was presumptively diagnosed with hypereosinophilic perimyocarditis and admitted to the Intensive Care Unit for shock. The pericardial effusion increased during treatment; therefore, pericardiocentesis was performed. Analysis of the pericardial effusion showed eosinophilia (eosinophils 90%) and the serologic test for parasites was positive for Toxocara and Sparganum. A combination therapy of albendazole, praziquantel, and corticosteroid resolved the pericardial effusion and the peripheral blood eosinophil count normalized. She was discharged without any other complications. At Outpatient Clinic follow-ups and observations over the next 2 years there were no abnormal findings, including pericardial effusion or eosinophilia. CONCLUSIONS Toxocariasis rarely causes perimyocarditis with cardiogenic shock. Patients who present with pericardial effusion and eosinophilia need to be evaluated for parasitic infection.

Eosinophilic annular erythema is an idiopathic acute eosinophilic dermatosis. It is a rare condition, with approximately 30 cases reported in the English literature. It features annular, figurate urticarial edematous plaques primarily affecting the trunk and proximal limbs. During evaluation of a patient, secondary causes of eosinophilic inflammation such as allergy-related conditions (eczema, drug, urticaria, contact dermatitis), parasitic infestations, and autoimmune dermatoses will need to be excluded. We present an unusual case of a 47-year-old patient who developed this condition.