Eosinophilic granuloma of the sternum in a child treated with closed biopsy

Langerhans cell histiocytosis is a rare neoplastic proliferative disorder of the Langerhans cells. The clinical course is variable, ranging from a low symptomatic single bone lesion to fatal multiple organ involvement. Rarely, the sternum can be the first and single location of the disease. We report on a 12‐year‐old boy who presented with an aggressive lytic lesion of the proximal sternum associated with local pain and afternoon fever. Histopathological analysis of the closed biopsy specimen indicated eosinophilic granuloma of bone/Langerhans cell histiocytosis. Soon after the biopsy procedure the pain and fever subsided. Computed tomography at 2 months showed healing of the lytic lesion. The patient received no other type of treatment. At 2 year follow up he was symptom and disease free.     

颌面部骨嗜酸性肉芽肿18例临床分析

目的:分析颌面部骨嗜酸性肉芽肿的临床及X线特点。方法:对18例颌面部骨嗜酸性肉芽肿资料,分别从患者性别、发病年龄、临床表现、发病部位、X线表现、误诊情况、治疗等方面进行回顾性分析。结果:18例颌面部骨嗜酸性肉芽肿患者,男11例,女7例;平均发病年龄16.62岁,平均病程5.5个月;11例为单发,7例为多发,共计29个部位,其中累计下颌骨21处(72.8%),上颌骨4处(13.8%),颅骨3处(10.3%),颧骨1处。主要临床表现为牙齿松动,牙龈肿胀、溃烂、疼痛、面部肿胀伴压痛。X线表现为颌骨中央不规则、溶解样破坏,破坏区呈地图状、网状、虫蚀样改变,骨破坏边缘不清晰。易误诊为骨肉瘤(3例),牙源性粘液瘤(2例),恶性淋巴瘤(1例),浆细胞骨髓瘤(1例),低分化癌(1例),其他恶性肿瘤(4例)。治疗方法6例采取单纯手术,7例采取手术+放疗,3例采取手术+化疗,1例采取手术+放疗+化疗。平均随访5年,14例无复发。结论:该病变特点显著,应正确诊断和治疗。     

Multiple Cerebral Infarctions Accompanied by Subcortical and Subarachnoid Hemorrhaging in Bilateral Border Zone Areas in a Patient with Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is often associated with peripheral neuropathy, but reports of central nervous system involvement are quite rare. We herein report a patient with EGPA first identified as having hypereosinophilia who later developed asthma, eosinophilic otitis media, sinusitis, and hemorrhagic colitis. She subsequently developed hemiparesis. Head magnetic resonance imaging revealed multiple cerebral infarctions with subcortical and subarachnoid hemorrhaging colocalized at the bilateral border zone areas. She was diagnosed with EGPA-induced stroke and successfully treated with oral prednisolone. Inflammation in the small cerebral arteries in EGPA may induce bilateral border zone infarction with colocalizing subcortical and subarachnoid hemorrhaging.     

Emerging roles of basophils in allergic inflammation

Basophils have long been neglected in immunological studies because they were regarded as only minor relatives of mast cells. However, recent advances in analytical tools for basophils have clarified the non-redundant roles of basophils in allergic inflammation. Basophils play crucial roles in both IgE-dependent and -independent allergic inflammation, through their migration to the site of inflammation and secretion of various mediators, including cytokines, chemokines, and proteases. Basophils are known to produce large amounts of IL-4 in response to various stimuli. Basophil-derived IL-4 has recently been shown to play versatile roles in allergic inflammation by acting on various cell types, including macrophages, innate lymphoid cells, fibroblasts, and endothelial cells. Basophil-derived serine proteases are also crucial for the aggravation of allergic inflammation. Moreover, recent reports suggest the roles of basophils in modulating adaptive immune responses, particularly in the induction of Th2 differentiation and enhancement of humoral memory responses. In this review, we will discuss recent advances in understanding the roles of basophils in allergic inflammation.     

Eosinophilic gastroenteritis and graft‐versus‐host disease induced by transmission of Norovirus with fecal microbiota transplant

Fecal microbiota transplantation (FMT) was performed to decolonize gastrointestinal tract from antibiotic‐resistant bacteria before allogeneic hematopoietic cells transplantation (alloHCT). AlloHCT was complicated by norovirus gastroenteritis, acute graft‐versus‐host disease, and eosinophilic pancolitis. Norovirus was identified in samples from FMT material. Symptoms resolved after steroids course and second norovirus‐free FMT from another donor.     

An update on pityrosporum folliculitis in Singapore from a single tertiary care dermatological centre

INTRODUCTIONPityrosporum folliculitis (PF) is a common skin condition that can be easily misdiagnosed, especially by non-dermatologists. While the initial diagnosis is often made clinically, skin microscopy may help to confirm the same. However, there is scant literature regarding the clinical epidemiology of PF. To our knowledge, in Singapore, only one prior epidemiological study was performed in 1987. Through the present study, we aimed to provide an update regarding the epidemiology, diagnosis and treatment of patients with PF in Singapore.METHODSWe performed a retrospective review of patients with clinical presentations compatible with PF who presented to the dermatology clinic at the National University Hospital, Singapore, between 1 January 2011 and 31 December 2015. The medical records of patients identified as having clinical presentations that resembled PF were reviewed via written and electronic databases. Information regarding the demographics and clinical presentation of the patients was collected.RESULTSOf the 375 patients identified, 214 (57.1%) were confirmed as having PF based on Gram-stain microscopy. Most (35.0%) of these 214 patients were aged between 21 and 30 years, with a male-to-female ratio of 3:1. The lesions predominantly occurred on the trunk and the back. The majority of patients presented with symptoms that lasted more than one month. 128 (59.8%) patients received oral antifungal treatment, whereas 82 (38.3%) patients were treated with topical antifungal treatment alone.CONCLUSIONA typical Singapore patient with PF is a young man aged 21–30 years, with erythematous follicular papules or pustules over the trunk and the back.     

Reliability and Validity Study of Turkish Version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0) Led to Development of a New Pediatric Eosinophilic Esophagitis Scale: GaziESAS

BackgroundThere has been no valid and reliable Turkish scale that measures symptoms in children with eosinophilic esophagitis (EoE). The aim of the study is to test the validity and reliability of the Turkish version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0).Methods Relevant forms of Tr-PEESS v2.0 were applied to 2-18 years old children with EoE and to their parents. KINDL QoL patient and parent questionnaires and the GaziESAS scale developed in this study were used to test the convergent validity of Tr-PEESS v2.0. Discriminant validity was evaluated among 3 EoE treatment groups: under treatment, off treatment due to remission, and uncompliant with treatment. Reliability was evaluated by internal consistency, test–retest reliability, and item analysis.Results Fifty-two children/teens (mean age 130.2 ± 60.3 months) and 84 parents were interviewed twice one week apart. The mean duration of EoE was 47.2 ± 35.6 months. Tr-PEESS v2.0 reports correlated with GaziESAS (range 0.361-0.855) and KINDL QoL questionnaires (range −0.316 to 0.413). Parent report of Tr-PEESS v2.0 discriminated children uncompliant with treatment from the ones off treatment and undertreatment. Cronbach’s α values and intraclass correlation coefficients (ICC) values of Tr-PEESS v2.0 ranged from 0.614-0. 895 and 0.646-0.910, respectively.Conclusion Tr-PEESS v2.0 is a valid and reliable tool to use in Turkish children. GaziESAS is a new parent-proxy pediatric EoE scale with an additional adaptive behavior domain that passed scale developmental stages successfully for Turkish children with EoE.