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In this article we represent an 18-year-old female with progressive back pain and neurologic symptoms due to a large heterogenous mass centered in posterior elements of lower thoracic vertebrae with prominent soft tissue extension into paravertebral and epidural space and subsequent central cord compression along with severe adjacent inflammatory changes. Considering patient age, lesion location and characteristics, aggressive Osteoblastoma, eosinophilic granuloma, and with lower probability of Ewing sarcoma were included in the differential list, which aggressive Osteoblastoma was confirmed after pathologic examination.

Objective: Eosinophilic granuloma is the most common type of Langerhans cell histiocytosis. Vertebral bone involvement of eosinophilic granuloma is a rare disease. There are few studies in the literature on patients with vertebral bone involvement diagnosed with eosinophilic granuloma. There is still no clear protocol for the treatment of patients with vertebral involvement with eosinophilic granuloma. This article is a summary of the clinical follow-ups of our 6 cases diagnosed with spinal eosinophilic granuloma. We changed the chemotherapy protocol or extended the duration of chemotherapy in patients with progressive disease.Methods: In this study, there are six patients (4 male and 2 female) with vertebral involvement of eosinophilic granuloma in our hospital between September 2017 and August 2022. The clinical findings, radiological images, treatments and follow-up results of the patients were analyzed retrospectively.Results: In the follow-ups, a new vertebral bone involvement was observed in two patients and a femoral bone involvement was observed in another patient. One patient with neurological deficit underwent surgical treatment. Chemotherapy treatment was given to four patients with multiple bone involvement or recurrence. No recurrence was observed in three patients who started chemotherapy treatment after recurrence.Conclusions: Surgical treatment should be considered in patients with neurological deficits and spinal instability. According to the results of our study, patients with multiple bone or systemic involvement and no neurological deficit should receive chemotherapy treatment. We recommend changing the chemotherapy protocol or extending the chemotherapy duration in patients with progressive disease., Цель. Эозинофильная гранулема является наиболее частым типом лангергансоклеточного гистиоцитоза. Поражение костей позвоночника эозинофильной гранулемой является редким заболеванием. В литературе имеется несколько исследований пациентов с поражением позвоночника, у которых диагностирована эозинофильная гранулема. До сих пор нет четкого протокола лечения пациентов с поражением позвоночника эозинофильной гранулемой. Эта статья представляет собой краткое изложение клинических наблюдений наших 6 случаев с диагнозом спинальной эозинофильной гранулемы. Мы изменили протокол химиотерапии или увеличили продолжительность химиотерапии у пациентов с прогрессирующим заболеванием. Методы. В этом исследовании участвовало шесть пациентов (4 мужчины и 2 женщины) с поражением позвоночника эозинофильной гранулемой в нашей больнице в период с сентября 2017 года по август 2022 года. Клинические данные, рентгенологические изображения, лечение и результаты наблюдения за пациентами были проанализированы ретроспективно. Результаты. При последующем наблюдении новое поражение позвоночника наблюдалось у двух пациентов и поражение бедренной кости у другого пациента. Одному больному с неврологическим дефицитом проведено оперативное лечение. Химиотерапевтическое лечение было назначено четырем пациентам с множественным поражением костей или рецидивом. У трех пациентов, начавших химиотерапию после рецидива, повторных рецидивов не наблюдали. Выводы. Хирургическое лечение следует рассматривать у пациентов с неврологическим дефицитом и нестабильностью позвоночника. По результатам нашего исследования больные с множественным костным или системным поражением и отсутствием неврологического дефицита должны получать химиотерапевтическое лечение. Мы рекомендуем изменить протокол химиотерапии или увеличить продолжительность химиотерапии у пациентов с прогрессирующим заболеванием., Мета. Еозинофільна гранульома є найбільш поширеним типом гістіоцитозу Лангерганса. Ураження кісток хребців еозинофільною гранульомою є рідкісним захворюванням. У літературі мало досліджень пацієнтів із ураженням кісток хребців з діагнозом еозинофільна гранульома. Досі немає чіткого протоколу лікування пацієнтів із ураженням хребців еозинофільною гранульомою. Ця стаття є коротким підсумком клінічного спостереження за нашими 6 випадками з діагнозом спінальна еозинофільна гранульома. Ми змінили протокол хіміотерапії або подовжили тривалість хіміотерапії у пацієнтів із прогресуючим захворюванням.Методи. У цьому дослідженні було шість пацієнтів (4 чоловіки та 2 жінки) з ураженням хребців еозинофільною гранульомою в нашій лікарні в період з вересня 2017 р. по серпень 2022 р. Клінічні дані, рентгенологічні зображення, лікування та результати подальшого спостереження пацієнтів були проаналізовано ретроспективно.Результати. Під час подальшого спостереження у двох пацієнтів спостерігалося нове ураження кісток хребців, а в іншого пацієнта – ураження стегнової кістки. Одному хворому з неврологічним дефіцитом проведено оперативне лікування. Хіміотерапію отримували чотири пацієнти з множинним ураженням кісток або рецидивом. У трьох пацієнтів, які почали хіміотерапію після рецидиву, повторних рецидивів не спостерігали.Висновки. Хірургічне лікування слід розглядати у пацієнтів з неврологічним дефіцитом і нестабільністю хребта. Згідно з результатами нашого дослідження, пацієнти з множинним кістковим або системним ураженням і відсутністю неврологічного дефіциту повинні отримувати хіміотерапію. Ми рекомендуємо змінити протокол хіміотерапії або збільшити тривалість хіміотерапії у пацієнтів з прогресуючим захворюванням.

Riga-Fede disease (RFD), also known as traumatic eosinophilic granuloma, is a benign inflammatory condition of the oral cavity that may mimic a malignant tumour. It’s a rare condition mainly reported in infants. We present a unique case report of a 57-year-old man who presented to our outpatient department with foreign body sensation in the throat and swelling over the dorsum of the tongue for a period of 1 month. He gave no history of trauma, especially due to sharp teeth. Video laryngoscopy done revealed an ulceroproliferative growth on the midline of the dorsum of the tongue. With clinical suspicion of malignancy, the patient underwent wide local excision of the tongue lesion. Histopathological examination was suggestive of RFD. The postoperative follow-up was uneventful with good wound healing. The patient was followed up with no evidence of recurrence.

Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocytic cells that rarely affects adults. Different therapeutic options have been used in the treatment of this condition depending on the severity of the disease. However, the management of LCH is often controversial when a resection with clean margins is not achieved. We present a 37-year-old male who was diagnosed with LCH affecting the middle third of the clavicle. The patient was subjected to a wide resection of the lesion creating a large bone defect. The reconstruction was then performed using a 3-D printed porous pseudo-prosthesis (Ti6AL4V) which was fixed using an anatomical clavicular plate (Depuy-Synthes, Raynham, MA, USA) and locking screws. The implant trabeculae were filled with bioactive bone graft substitute (GlassBONETM, Noraker, France). After two years of follow-up the patient was pain free, had a full range of movement, and scored 100 on the Constant Scale and 2.5 points on the disabilities of the arm, shoulder and hand score (DASH). Moreover, in this case, the implantation of the 3-D printed pseudo-prosthesis avoided the use of adjuvant therapies such as radiotherapy. The use of a 3-D printed pseudo-prosthesis achieved an excellent clinical and functional outcome in the treatment of a large bone defect, following a resection of a LCH of the clavicle. 3-D printed pseudo-prostheses could be useful instruments for the treatment of bone defects following large bone resections in musculoskeletal tumors.

Vertebra plana is a rare radiologic condition characterized by a uniform loss of height of a vertebral body that represents a diagnostic challenge for surgeons. The purpose of this study was to review all possible differential diagnoses that may present with a vertebra plana (VP) described in the current literature. For that purpose, we performed a narrative literature review in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, analyzing 602 articles. Patient demographics, clinical presentation, imaging characteristics and diagnoses were investigated. VP is not a pathognomonic feature of Langerhans cell histiocytosis, but other oncologic and non-oncologic conditions should be considered. The list of differential diagnoses, based on our literature review, can be recalled with the mnemonic HEIGHT OF HOMO: H—Histiocytosis; E—Ewing’s sarcoma; I—Infection; G—Giant cell tumor; H—Hematologic neoplasms; T—Tuberculosis; O—Osteogenesis imperfecta; F—Fracture; H—Hemangioma; O—Osteoblastoma; M—Metastasis; O—Osteomyelitis, chronic.

PURPOSE Ophthalmic histiocytic lesions comprise a heterogeneous rare group of disorders that are characterized by an abnormal proliferation of histiocytes and may affect all age groups of both sexes. The aim of this study was to highlight the basic demographic, clinical, and histopathological characteristics of this rare group of diseases in ophthalmic practice, which has not been previously studied in this area. Only individual cases have been previously reported. METHODS This was a retrospective study of all biopsied ocular and periocular histiocytic lesions from two centers, King Khaled Eye Specialist Hospital (KKESH) and King Abdulaziz University Hospital (KAUH) in Riyadh, Saudi Arabia, from January 1993 to December 2018. The histopathological diagnosis was confirmed, and the cases were re-classified by reviewing all histopathological slides. The corresponding demographic and clinical data were analyzed. A relevant literature review was also carried out for comparison of our collected analyzed data to published data and to draw our own conclusions. RESULTS A total of 34 ocular/periocular histiocytic lesions in 28 patients who were mostly Saudis (92.9%) were included. The male-to-female ratio was 4:3. The median age at presentation was 6.4 years (range: 2.8-35 years). Twenty-two patients had unilateral involvement, and six patients had bilateral lesions. In patients with Langerhans cell histiocytosis (LCH; L group), the most common presenting findings were eyelid swelling (75%), periocular tenderness (37.5%), proptosis/globe displacement (37.5%) eyelid erythema (25%), and orbital pain (12.5%). In patients with Rosai Dorfman disease (RDD; R group), proptosis/globe displacement occurred in all patients and 80% had decreased vision. Patients in the C group (Cutaneous non-LCH histiocytoses) had variable clinical features because of the different locations of the histiocytic lesions, with the majority involving the eyelids (66.7%). Diagnosis was accurately reached clinically in 38.8%, 33.7%, and 46.7% of patients in the L, C, and R groups, respectively. Overall, the clinical diagnosis was in concordance with the histopathologic diagnosis in 14 out of 34 lesions (41.2%). CONCLUSIONS Histiocytic disease is more likely to be overlooked clinically owing to its rarity. In the C group, juvenile xanthogranuloma (JXG) was the most commonly encountered histiocytic lesion and had a tendency to present at a later age with extremely rare intraocular involvement in contrast to previously published reports. The median age at presentation was higher in group R. All patients in group L had strictly unilateral disease, while RDD (group R) was most commonly bilateral. Future research on genetic aspects, management, and prognosis is necessary.

Athough few case reports of human fascioliasis have been reported from different parts of India, there is no case reported from Kashmir valley till date. Here in we report two cases of human fascioliasis. The both patients presented with fever, marked eosinophilia and liver leisions on imaging. Hepatobiliary imaging showed vague features like mild biliary dilatation and liver leisions representing burrows. Liver biopsy in one of the patients revealed eosinophilic granuloma. Both patients were diagnosed definitively with endoscopic retrograde cholangio-pancreatography (ERCP) by demonstrating live adult fasciola worms. Any patient presenting with fever, marked eosinophilia and liver leisions on imaging should be evaluated for fascioliasis.

Palisaded neutrophilic and granulomatous dermatitis (PNGD) represents a cutaneous histopathologic reaction spectrum associated with several underlying disorders. Few cases of PNGD have been associated with chronic myelomonocytic leukemia (CMML), a malignant hematopoietic disorder with features in between those of a myeloproliferative neoplasm and myelodysplastic syndrome. We present a patient with a generalized papular skin reaction involving the neck, chest, and shoulders with histomorphological features on the spectrum of PNGD. Subsequent laboratory workup demonstrated a persistent mild monocytosis, raising concern for CMML. The diagnosis was ultimately confirmed with a bone marrow biopsy and associated mutational analysis through next-generation sequencing which identified deleterious variants in SRSF2, IDH2, and ASXL1. The findings in this case strengthen the previously made association between PNGD and SRSF2-mutated CMML and may help better define a unique recognizable clinical-histopathological-molecular subtype for dermatopathologists.

Langerhans cell histiocytosis (LCH) is a rare enigmatic disease that pre-dominantly affects children under 5 years of age. We report an interesting case of a 5 month old female diagnosed with multisystem LCH. Her disease process included osseous, pulmonary, gastrointestinal, cutaneous, hematopoietic and neurologic involvement. This case highlights the varying clinical symptoms, risk factors, pathogenesis, and management of multisystem LCH. This case also emphasizes the role of diagnostic imaging in this multifaceted disease.

Histiocitosis; Granuloma eosinófilo; Inhibidores BRAF Histiocitosi; Granuloma eosinòfil; Inhibidors BRAF Histiocytosis; Eosinophilic granuloma; BRAF inhibitors Langerhans cell histiocytosis (LCH) is a type of myeloid neoplasia that can affect different organs or tissues and exhibits substantial variability in its clinical presentation and biological behaviour, so it may mimic different diseases. Performance of different clinical assessments and laboratory and imaging tests is recommended to determine the extent of involvement, which may be of a single location or multisystemic, and the presence or absence of dysfunction in risk organs, such as the haematopoietic system, liver and spleen. The diagnosis must be confirmed by histological examination of a biopsy sample. Molecular tests have identified mutations in the mitogen-activated protein kinase (MAPK) pathway, which has expanded treatment options. The diagnosis is complex and there is controversy regarding the management of certain cases. Treatment recommendations depend on the location of the lesions and the extent of involvement. International collaborative studies have demonstrated the effectiveness of prolonged combination therapies such as vinblastine and prednisone in severe or multisystemic forms, and anti-inflammatory drugs such as indomethacin and other cytostatic combinations have proven beneficial. Langerhans cell histiocytosis is a good example of the importance of precision medicine and the benefit of identifying molecular targets, common to different neoplasms, to develop new therapies. MAPK pathway inhibitors offer an alternative treatment option in refractory cases and neurodegenerative forms of LCH. Molecular testing can contribute to the prognosis, treatment and follow-up of LCH, especially in severe forms of disease. La histiocitosis de células de Langerhans es un tipo de neoplasia hematológica de origen mieloide, que puede afectar a diferentes órganos o tejidos, con gran variabilidad en la presentación clínica y comportamiento biológico, por lo que puede simular diferentes enfermedades. Se recomienda realizar diversas pruebas clínicas, analíticas y de imagen, para determinar la extensión de la afectación, que puede ser única o multisistémica, y la presencia o no de disfunción en órganos de riesgo como sistema hematopoyético, hígado y bazo. El diagnóstico se debe confirmar mediante biopsia y estudio histológico. Los estudios moleculares han permitido identificar mutaciones en la vía MAPK, lo que han ampliado las opciones terapéuticas. El diagnóstico es complejo y existe controversia en el manejo de ciertos casos. Las recomendaciones terapéuticas dependen de la localización de las lesiones y la extensión de la afectación. Los estudios colaborativos internacionales han demostrado la efectividad de terapias prolongadas combinadas como vinblastina y prednisona en formas graves o multisistémicas y destaca el papel beneficioso de fármacos antinflamatorios como indometacina y de otras combinaciones de citostáticos. HCL representa un buen ejemplo de la importancia de la medicina de precisión y del beneficio de la identificación de dianas moleculares, comunes a diferentes neoplasias, para desarrollar nuevas terapias dirigidas. Los inhibidores de la vía MAPK representan una alternativa terapéutica en casos refractarios y en las formas neurodegenerativas de HCL. Los estudios moleculares pueden contribuir en el pronóstico, tratamiento y seguimiento, especialmente en las formas graves. The study was partially funded by a grant allocated to the Histiocytosis Research Project of the Fundación Vasca de Innovación e Investigación Sanitaria BIOEF (BIO16/ER/020/BC) and the Asociación Española contra la Histiocitosis-ACHE (BC/A/15/012, BIOEF11/017, BIOEF09/047), whose principal investigator is Itziar Astigarraga.

Emergency service physicians have forensic physician duties in addition to providing treatment and protective services. Errors in forensic report writing could affect the judicial process negatively.A 16-year old male presented at the Emergency Department (ED) following an assault. With a diagnosis of epidural hematoma on the computed tomography taken because of head trauma, the patient was transferred to the brain surgery ward. In the forensic report written in ED, there was stated to be a danger to life. In the repeated evaluation, there was seen to be a mass in the calvarium and surgery was performed. In the histopatholgical evaluation, the diagnosis was made of Langerhans Cell Histiocytosis. It must not be forgotten that errors or ommissions in a forensic report constitute malpractice. The necessary care and attention must be paid to forensic reports prepared in the Emergency Department.

This paper presents two clinical cases of eosinophilic granuloma of bone diagnosed by CT, MRI, and 18F-FDG PET/CT. In both cases the patients were admitted to the clinic with suspected primary malignant bone tumor and the diagnosis of a solitary eosinophilic granuloma was made based on the results of comprehensive radiological diagnostic examination and histological verification. Solitary eosinophilic granuloma of bone is an infrequent condition, occurring in less than 1% of cases of skeletal tumor masses. The most common eosinophilic granuloma is found in the parietal and frontal bones of the skull and is an osteolytic volumetric mass that gradually increases in size. Although most bone tumors can be detected by radiography, computed tomography is preferred, primarily because of its superior ability to detect cortical bone destruction. The diagnostic accuracy of computed tomography and magnetic resonance imaging may be different. The combined use of radiological and radionuclide methods allows us to narrow the spectrum of differential diagnosis. Unfortunately, relatively low specificity of existing radiological diagnostic studies in most cases does not allow to establish a precise diagnosis, and biopsy with subsequent pathological examination remains the method of choice. These clinical observations demonstrate the need to include eosinophilic granuloma in the differential diagnosis when a solitary osteolytic focus is detected.

Introduction: Traumatic ulcerative granuloma with stromal eosinophilia is an uncommon condition of the oral mucosa with a chronic course, usually affecting the tongue. Case Report: Clinically it presents as a chronic ulcer, with raised and indurated borders, rarely presented as a tumor. Histologically it shows a diffuse mixed inflammatory infiltrate, rich in eosinophils. The etiology of this lesion is still unclear; however, chronic irritation from traumatic agents is considered a major initiating factor. In some cases, the presence of CD30+ mononuclear cells within the lesions suggest the possibility of a CD30+ lymphoproliferative disorder. This article presents a case of a traumatic ulcerative granuloma with stromal eosinophilia manifested in a 56-year-old female with a solitary ulcerated tumor inside the right cheek. Conclusion: It was diagnosed based on clinical data and histopathological features. In a brief literature review, the entity has been characterized, analyzing its etiology and nature.

京都府立医科大学附属北部医療センター 消化器内科, 京都府立医科大学 消化器内科, 京都府立医科大学附属北部医療センター 外科, Department of Gastroenterology, North Medical Center,Kyoto Prefectural University of Medicine, Molecular Gastro enterology and Hepatology, Kyoto Prefectural University of Medicine, Department of Surgery, North Medical Center,Kyoto Prefectural University of Medicine, 症例は80代、男性。市民検診での胃透視で前庭部に異常所見を指摘され、精査加療目的に当院受診となった。上部消化管内視鏡検査では胃幽門部に30mm大の3型腫瘍を認めた。生検結果はadenocarcinoma tub2-porであった。胸腹部造影CT検査では所属リンパ節腫大・遠隔転移は認めず、術前診断:cT2N0M0 cStage Iとして手術加療を行った。最終病理診断はadenocarcinoma(por1>tub2>tub1)pT1b(SM2 0.7mm)Ly0V1aN0であり、腫瘍直下の筋層内に2mmの壊死を伴う肉芽腫を認めた。肉芽腫内には好酸球浸潤を認め、遺伝子解析の結果Anisakis simplexが検出された。その後術後経過良好で、現在再発なく、当院外科外来で経過観察されている。(著者抄録)

The study aimed to identify, enlist, and analyze cases of unisystem LCH in the maxillofacial pediatric population to understand the clinical presentation and encourage the consideration of this rare disease in the differential diagnosis. Langerhans cell histiocytosis (LCH) is an aggressive benign condition affecting mainly the pediatric population. It can be easily masked as periodontal disease in the maxillofacial region. Early diagnosis and a systemic evaluation are of utmost importance.We are presenting a complete review of literature in the pediatric population according to PRISMA guidelines for clinicopathologic, histopathological, immunohistochemistry, and treatment for unisystem LCH. The risk of bias assessment across studies was done using a Case series appraisal checklist by Guo et al. 53 RESULTS: Forty-nine articles (152 cases) were selected which met our inclusion and exclusion criteria to be included in our review. Most of the patients fall in 6-12 years of age with the involvement of the mandibular body region in 40.79% cases. This disease mainly presents as erythematous gingiva, pain, swelling, and mobile teeth. Management can range from minimal intervention to chemotherapy and surgery.Although this is a rare condition, it should be considered especially in the pediatric population with periodontitis type lesions and floating teeth and comprehensive management should be followed. Early diagnosis of the disease is very important.

We report the case of an otherwise healthy 17-year-old female who presented for surgical removal of an enlarging “atypical cyst” on her scalp. During subtotal excision, only friable serosanguinous translucent ribbons of tissue were found. A histopathologic diagnosis of Langerhans cell histiocytosis (LCH) was rendered and imaging studies revealed extradural invasion of the tumor. Within weeks, the patient also developed progressive lymphadenopathy with grossly elevated levels of Epstein-Barr virus viral capsid antigen antibody levels. This report aims to highlight a unique presentation of LCH with discussion of workup, management, and avoidance of potential surgical complications.

BACKGROUND Hyaluronic acid filler reactions have been increasingly observed in recent years. Our study investigates whether the increased number of filler reactions observed since 2014 is associated with a specific histopathologic inflammatory pattern and type of filler. METHODS The institution's dermatopathology electronic database was retrospectively searched for histopathologic reactions to hyaluronic acid from January 2014 to December 2019. The age, sex, type of filler, procedure, location, and histopathologic patterns were recorded. RESULTS From 2014 to 2019, there were 15 cases of granulomatous reactions to hyaluronic acid filler. In 10 of these cases, there was a characteristic inflammatory pattern characterized by tightly cuffed palisades of histiocytes with varying numbers of eosinophils. Of the 11 cases in which the type of filler was known, all used Vycross technology, a novel manufacturing process in the production of hyaluronic acid filler. CONCLUSION A characteristic histopathologic pattern of discrete foci of tightly cuffed palisaded granulomas with eosinophils is associated with fillers manufactured using Vycross technology.

Descriptive, retrospective. Scientific level of evidence IV. The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine. A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2 years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed. Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5 years and 2 months (6 months to 13 years and 3 months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3 years and 5 months (range 2–12 years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment. Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.

Background: Langerhans cell histiocytosis (LCH) is a rare nonmalignant disease characterized by a clonal proliferation of mononuclear cells called Langerhans histiocytes and infiltrates surrounding tissues, mostly self-limiting and usually occurring in the first two decades of life. Vertebral involvement is rare, mostly seen in the thoracic region, and involves the anterior elements of the corpus. In the literature, several treatment options and surgical approaches have been reported concerning the treatment of this disease and surgery. Case Presentation: We report an 18-month-old male with thoracic LCH who underwent surgery due to progressive neurological deficit. Gross total removal of the tumor with one level corpectomy in this patient was achieved via a posterolateral approach with postoperative functional improvement. The surgical cavity was supported by corpectomy cage and unilateral screw-rod fixation system at the same stage. Conclusion: Gross total tumor removal, corpectomy, and 360° stabilization via posterolateral approach at a single stage are safe, effective, and definite neurosurgical methods in terms of providing neurological recovery, long-term tumor-free survival, and spinal stability.

La histiocitosis de células de Langerhans (HCL) es un tipo de neoplasia hematológica de origen mieloide, que puede afectar a diferentes órganos o tejidos, con gran variabilidad en la presentación clínica y comportamiento biológico, por lo que puede simular diferentes enfermedades. Se recomienda realizar diversas pruebas clínicas, analíticas y de imagen, para determinar la extensión de la afectación, que puede ser única o multisistémica, y la presencia o no de disfunción en órganos de riesgo como sistema hematopoyético, hígado y bazo. El diagnóstico se debe confirmar mediante biopsia y estudio histológico. Los estudios moleculares han permitido identificar mutaciones en la vía MAPK, lo que han ampliado las opciones terapéuticas. El diagnóstico es complejo y existe controversia en el manejo de ciertos casos. Las recomendaciones terapéuticas dependen de la localización de las lesiones y de la extensión de la afectación. Los estudios colaborativos internacionales han demostrado la efectividad de terapias prolongadas combinadas como vinblastina y prednisona en formas graves o multisistémicas y destaca el papel beneficioso de fármacos antiinflamatorios como indometacina y de otras combinaciones de citostáticos. La HCL representa un buen ejemplo de la importancia de la medicina de precisión y del beneficio de la identificación de dianas moleculares, comunes a diferentes neoplasias, para desarrollar nuevas terapias dirigidas. Los inhibidores de la vía MAPK representan una alternativa terapéutica en casos refractarios y en las formas neurodegenerativas de la HCL. Los estudios moleculares pueden contribuir en el pronóstico, el tratamiento y el seguimiento, especialmente en las formas graves, Langerhans cell histiocytosis (LCH) is a type of myeloid neoplasia that can affect different organs or tissues and exhibits substantial variability in its clinical presentation and biological behaviour, so it may mimic different diseases. Performance of different clinical assessments and laboratory and imaging tests is recommended to determine the extent of involvement, which may be of a single location or multisystemic, and the presence or absence of dysfunction in risk organs, such as the haematopoietic system, liver and spleen. The diagnosis must be confirmed by histological examination of a biopsy sample. Molecular tests have identified mutations in the mitogen-activated protein kinase (MAPK) pathway, which has expanded treatment options. The diagnosis is complex and there is controversy regarding the management of certain cases. Treatment recommendations depend on the location of the lesions and the extent of involvement. International collaborative studies have demonstrated the effectiveness of prolonged combination therapies such as vinblastine and prednisone in severe or multisystemic forms, and anti-inflammatory drugs such as indomethacin and other cytostatic combinations have proven beneficial. LCH is a good example of the importance of precision medicine and the benefit of identifying molecular targets, common to different neoplasms, to develop new therapies. MAPK pathway inhibitors offer an alternative treatment option in refractory cases and neurodegenerative forms of LCH. Molecular testing can contribute to the prognosis, treatment and follow-up of LCH, especially in severe forms of disease, Este trabajo ha sido financiado parcialmente por los fondos del Proyecto de Investigación sobre Histiocitosis de la Fundación Vasca de Innovación e Investigación Sanitaria BIOEF (BIO16/ER/020/BC) y de la Asociación Española contra la Histiocitosis-ACHE (BC/A/15/012, BIOEF11/017, BIOEF09/047)

INTRODUCTION Heart involvement in vasculitis is rare, but potentially severe. The ascertainment of cardiac disease in vasculitis is complex and requires an integrated multidisciplinary approach involving the Rheumatologist, Radiologist, Cardiologist, and Heart surgeon. AREAS COVERED the authors searched PubMed using the keywords 'heart'[Mesh] and vasculitis"[Mesh]. EXPERT OPINION Virtually any vasculitis can affect the heart, but cardiac involvement is more common in some vasculitides such as Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Immunosuppressive treatment and when indicated surgery can improve the prognosis.

BACKGROUND This study aimed to evaluate the long-term clinical and radiologic follow-up results of eosinophilic granulomas (EGs) of the axial and appendicular skeleton managed with biopsy alone. METHODS Fifty-five patients with unifocal osseous EGs of the axial and appendicular skeleton were followed after biopsy. Patients were divided into 2 groups on the basis of localization of the lesions. In group 1, there were 32 (58.2%) children with extremity long bone involvement: femur, tibia, humerus, ulna, and radius. Group 2 included 23 (41.8%) patients with lesions located in other appendicular and axial skeleton bones: pelvis, scapula, clavicle, lumbar, and thoracic vertebrae. After confirming the diagnosis by a closed biopsy, no further surgical intervention was performed. Clinical recovery included regression of the localized symptoms, mainly pain resolution. Functional improvement was assessed by Musculoskeletal Tumor Society (MSTS) scoring. Radiologic healing was defined as ossification of the entire lesion with cortical thickening in long and flat bones, and restoration of vertebral body height in the spine. Complications, including local recurrence, were determined. RESULTS The patients comprised 28 boys and 27 girls with an average age of 9.2 years (range, 3 to 16 y). The average follow-up was 76 months (range, 28 to 132 mo). The median time from biopsy to clinical recovery was 17 days [95% confidence interval (CI), 13.3-20.6] and 36 days (95% Cl, 32.8-39.1) in group 1 and group 2, respectively. MSTS scores increased progressively till the end of 12 months in both groups. The median time from biopsy to radiologic healing was 16 months (95% CI, 11.5-20.4) and 42 months (95% Cl, 39.3-44.6) in group 1 and group 2, respectively. Both clinical recovery (P=0.021) and radiologic healing (P=0.009) were significantly faster in group 1 compared with group 2. No major complication was seen after biopsy. All lesions regressed without a local recurrence. CONCLUSIONS Unifocal osseous EGs have spontaneous healing potential and confirming the diagnosis by biopsy is enough to obtain good clinical and radiologic results without any additional surgical intervention. TYPE OF STUDY This was a therapeutic study. LEVEL OF EVIDENCE Level IV.

Eosinophilic granuloma (EG) is a rare histiocytic disorder resulting from clonal proliferation of Langerhans cells. Eosinophilic granuloma, the most benign of the three entities of Langerhans cell histiocytosis, may be multiple or solitary. This lesion can affect any bones but commonly involves the mandible when the jaws are affected. It is difficult to make a correct diagnosis on it without proof of a pathological diagnosis, which correlates with the diverse clinical and radiographic presentations of EG in the jaws. This report describes a case of unifocal EG of the mandible occurring in a 6-year-old boy whose initial presentation led to confusion of any solitary bony lesion. A final diagnosis of EG was established after histopathological examination of the biopsy specimen.

Eosinophilic Granuloma represents one of a triad, of lesions encompassing a disease under the generic name Histiocytosis X or Langerhans Cell Histiocytosis. It is a benign inflammatory reaction to an unknown etiological agent and corresponds with bony lesions. The most commonly involved bones are the skull, ribs and the femur. The cranium, maxilla and mandible can also be affected.

We performed gross and histological examinations of the livers of sika deer (Cervus nippon yesoensis) in Hokkaido, Japan. Out of 1,381 deer slaughtered for venison production, thickening and dilation of the large intrahepatic bile ducts and Fasciola flukes in the duct lumens were detected in 621 deer (45.0%). Furthermore, 107 non-bile lesions (75 intrahepatic and 32 capsular lesions) were detected during gross examinations. Histologically, the bile duct lesions included chronic proliferative cholangitis, papillary hyperplasia, goblet cell and pyloric gland metaplasia, and periductal fibrosis. Many of the intrahepatic non-bile duct lesions (53/75, 71%) were considered to be Fasciola fluke migration-associated lesions, including two lesion types: necrosis, hemorrhage, and eosinophilic granuloma formation (29 lesions), and lymphoid tissue formation (24 lesions). Lymphoid tissue formation was considered to result from the persistent immune responses against dead Fasciola flukes. An epidermoid liver cyst was found incidentally, which has not been reported in the veterinary literature. In summary, this study demonstrated the predominance of fascioliasis-associated lesions in sika deer livers. The gross and histological lesions caused by Fasciola flukes in sika deer were similar to fascioliasis in other animals. Moreover, we described lymphoid tissue formation as a fascioliasis-associated lesion for the first time. The fact that bile duct lesions (45.0%) had a markedly higher prevalence than fascioliasis-associated parenchymal lesions (53/1,381, 3.8%) indicated that sika deer are a permissive host for fascioliasis. Our results provide information that will aid pathological examinations of sika deer.

Langerhans’ cell histiocytosis (LCH) is a rare disease of the reticuloendothelial system in which there are abnormal proliferation and accumulation of histiocytes, abnormal cells deriving from bone marrow that can migrate from the skin to the lymph nodes. Langerhans’ cell histiocytosis has three variants: unifocal (eosinophilic granuloma), multifocal unisystem (Hand–Schuller–Christian triad), and multifocal multisystem (Letterer–Siwe disease). We present a case of oral lesions associated with LCH in a young male aged 15 years. The history, radiological appearance, histopathology, and treatment options of the patient are discussed. How to cite this article: Soangra R, Kapoor A, Meena D, et al. Langerhans’ Cell Histiocytosis Diagnosed through Periodontal Lesion in a 15-year-old Child: A Case Report. Int J Clin Pediatr Dent 2020;13(S-1):S115–S118.

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease that is characterized by the clonal proliferation of bone marrow-derived Langerhans cells. It usually occurs in children at an incidence of 2.6–5.4/10,00,000 children/year. The disease occurs with a slight male predilection. The peak age ranges from 1 to 4 years. The common oral findings include the gingival enlargement, oral ulcers, and loosening of teeth. The variable radiographic appearance mimicking odontogenic cyst, osteomyelitis, or a malignancy poses a diagnostic challenge. This rare case report emphasizes on the differential diagnosis of LCH.

ObjectiveTo develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.ResultsThe development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×109/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3–ANCA positivity (−3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (−1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%).ConclusionThe 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.

Eosinophilic granuloma (EG) is the unifocal osseous form of Langerhans cell histiocytosis (LCH), which usually affects the skull and long bones. Although it most commonly affects the pediatric age group, it can rarely be seen in adults. Skeletal involvement is common in adult patients, but isolated rib involvement is extremely rare. Differential diagnosis includes other osteolytic lesions such as Ewing’s sarcoma, tuberculosis, multiple myeloma, lymphoma, and primary bone malignancy. The diagnosis must be confirmed histopathologically. In addition to pathological Langerhans cells, inflammatory cells such as lymphocytes, eosinophils, and macrophages are observed in microscopy. Immunohistochemically, CD1a, S-100, and Langerin positivity are observed in biopsy and/or surgical excision material. Treatment options may vary depending on the localization and extent of the disease. In unifocal EG, close observation of the patient may be preferred, as well as surgical excision, radiotherapy, and intra-lesional steroid administration. The prognosis in patients with a single bone lesion is quite good compared to other groups. In this case report, we present a metachronous EG of rib developed in two different ribs by an interval of seven years, which were both surgically treated. In this mild variant of LCH, surgical resection with clean margins has a favorable outcome without the need for additional adjuvant therapy. Metachronous tumors may develop in isolated unifocal bone EGs, and long-term follow-up is mandatory.

Background: Anisakiasis-related gastric eosinophilic granuloma is rare. Case Report: Herein, we report a patient with anisakiasis-related gastric eosinophilic granuloma who was treated with laparoscopic and endoscopic cooperative surgery (LECS). A 59-year-old woman was presented to our hospital for further examination of a gastric lesion that was initially diagnosed by a local medical doctor. Esophagogastroduodenoscopy showed a submucosal tumor-like lesion in the lower body of the stomach. Endoscopic ultrasonography showed a heterogeneous hypoechoic submucosal mass lesion in the submucosal layer measuring 10 mm, without evidence of deep involvement. Under a clinical diagnosis of gastrointestinal stromal tumor, the patient underwent LECS. Gross appearance of the resected specimen revealed a 1.5×1.0 cm submucosal tumor-like lesion. Microscopic examination revealed necrosed insects consistent with the characteristics of gastric anisakiasis, around which prominent eosinophilic infiltration and granulomas were observed. This prompted a diagnosis of gastric eosinophilic granuloma related to anisakiasis. Conclusion: To the best of our knowledge, this is the second case of gastric eosinophilic granuloma related to anisakiasis resected by LECS in the English medical literature. LECS might be a useful procedure for minimally invasive therapeutic diagnosis.

Background Non-traumatic acquired spinal deformities represent a separate entity within the axial pathologies in children. Their causes are variable (neurological, muscular, infectious, especially Pott's disease, inflammatory or tumoral). The etiological diagnosis can be clinically oriented by pain, medical history, especially rheumatic. The contribution of the CT scan is crucial, as it localizes the lesion, specifies its nature, but above all it helps the histological diagnosis by guided biopsies. Our case illustrates the difficulties in diagnosing the aetiology of spinal acquired deformity in a girl with a multi-evocative context. Observation A 14 years-old girl complains for a few months of a dorso-lumbar spinal pain and a vicious attitude. Her medical history includes femoral thrombophlebitis and psoriasis. She had no trauma or fever. Clinically, the general state is preserved, with no local inflammatory signs, a kyphotic attitude with an analgesic lumbar support was noted. The rest of the locomotor exam was normal. CT scan shows cancellous body geodes with a double mirror. Discussion Acquired spinal deformities are always secondary to general disease. The fever may point to bacterial infections, especially tuberculosis (Pott's disease), but also non-infectious causes such as a rheumatic disease. For our patient, psoriasis argues for the latter possibility. However, the rarity of isolated disco-vertebral involvement and the severity of the lytic lesions are against this diagnosis. The histopathological exam showed an eosinophilic granuloma, confirming the histiocytosis X diagnosis. The cortico-response strengthen the diagnosis. The course was marked by the bone remodeling with the posture correction. Conclusion A clinical, radiological, and histological analysis allow the etiological orientation in front of acquired spinal deformity.

We herein report a case of coagulation necrosis with granulation and eosinophilic infiltration of the liver. A 37-year-old woman was diagnosed with a new mass lesion in the liver 1 month after breast cancer surgery and admitted for a further examination. Because the tumor occurred immediately after surgery, it was considered essential to determine whether or not it was a metastatic liver tumor from breast cancer. A percutaneous liver tumor biopsy revealed eosinophilic granuloma of the liver, which is considered to have a high possibility of visceral larva migrans with suspected gnathostomiasis infection. A detailed medical history and histological diagnosis are important for making a differential diagnosis.

A retrospective study was performed on skin biopsy specimens from horses and cats having eosinophilic granulomas with ‘collagen degeneration’, eosinophilic skin diseases without ‘collagen degeneration’ and pyogranulomas without ‘collagen degeneration’. Neither the appearance of collagen fibres nor the dominant polymorphonuclear cells, as seen in H & E-stained sections, were predictive of Masson’s trichrome findings. Hence, a Masson’s trichrome staining abnormality of collagen fibres was no more likely to be present in lesions with ‘collagen degeneration’ than in those lesions without ‘collagen degeneration’. In addition, evaluation of trichrome-stained specimens suggested that there is no collagen degeneration in equine and feline eosinophilic granulomas and that the areas previously referred to as collagen degeneration are more appropriately called flame figures.

To investigate prevalence of anti-Pentraxin 3 (PTX3) antibodies in sera of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients.Anti-PTX3 and PTX3 levels were analysed by enzyme-linked immunosorbent assays in sera from unselected patients with AAV and compared with patients with systemic lupus erythematosus (SLE, n = 130), other connective tissue diseases (CTDs, n = 97) and matched healthy controls (n = 97). Optical density (OD) cut-off for positive anti-PTX3 antibodies was determined by ROC curve analysis and set as 0.234. Indirect immunofluorescence (IIF) on fixed human granulocytes was used to analyze the fluorescence pattern of anti-PTX3 antibodies. Liquid-phase inhibition tests were conducted to assess potential interferences.We included 101 AAV patients (females 58%, median age 60[51-69] years) affected either with granulomatosis with polyangiitis (GPA, n = 51), microscopic polyangiitis (MPA, n = 12) or eosinophilic granulomatosis with polyangiitis (EGPA, n = 38). Anti-PTX3 antibodies were detected in 29.7% AAV patients, being significantly higher than in healthy controls (p 0.001) and CTDs (p = 0.030) but lower than in SLE (p = 0.004). Anti-PTX3 antibody prevalence was 44.7% in EGPA, 25% in MPA and 19% in GPA (p = 0.034). Among ANCA negative patients, 35.7% displayed positive anti-PTX3 antibodies. Anti-PTX3 were associated with a lower prevalence of systemic (p = 0.002), ear-nose-throat (p = 0.006) and renal manifestations (p = 0.016). Anti-PTX3 antibodies were characterized by a specific IIF pattern on fixed granulocytes. PTX3 serum levels resulted lower in AAV than healthy controls (p 0.001). PTX3 inhibited anti-PTX3 binding in a dose-dependent manner.Anti-PTX3 autoantibodies appear a promising novel biomarker of AAV, especially EGPA.

Eosinophilic granuloma is a localized, non-invasive form of Langerhans cell histiocytosis. It usually develops in the long bones and is more frequent in children under the age of 10 years. It is very rare in adults. We present the case of a young woman admitted to hospital for persistent refractory left parietal headache, later revealed to be caused by an eosinophilic granuloma. LEARNING POINTS: Eosinophilic granuloma is a localized benign form of Langerhans cell histiocytosis. It is essential to exclude the presence of bone lesions in other locations and/or systemic involvement. The prognosis is good, with surgical resection, when necessary, being a curative intervention, although sometimes (as in this case) a conservative approach is sufficient.

— Mast cells were found in the surface epidermis and outer root sheath epithelium in 37 of 338 cats (11.6 per cent) with non-neoplastic skin disorders. Epidermal mast cells were most commonly found in the eosinophilic plaque and the eosinophilic granuloma. Thevast majority of dermatoses wherein epidermal mast cells were found were characterised by: (1) a presumed immune-mediated pathogenesis, and (2) tissue eosinophilias. Resume— Des mastocytes ont ete observes dans l'epiderme superficiel ainsi que dans l'epithelium folliculaire chez 37 chats parmi 338 (11,6%) atteints de dermatoses non neoplasiques. Les mastocytes epidermiques ont ete le plus souvent trouves dans la plaque eosinophilique et le granulome eosinophilique. La grande majorite des dermatoses presentant des mastocytes epidermiques etait caracterisee par: (1) la presomption d'une pathogenie immunodependante et (2) une eosinophilie tissulaire. Zusammenfassung— Mastzellen konnten in der oberflachlichen Epidermis und in den Epithelien der auseren Wurzelscheide bei 37 von 338 untersuchten Katzen (11.6%) mit nicht-neoplastischen Plaques oder eosinophilem Granulom gefunden werden. Die uberwiegende Zahl der Dermatosen, bei denen epidermale Mastzellen auftraten, war durch die beiden Kriterien 1) vermutlich immunvermittelte Pathogenese und 2) Gewebs-Eosionophilie charakterisiert. Resumen En el presente trabajo se describe la presencia de mastocitos en la epidermis superficial y en la vaina folicular externa de 37 gatos de 338 examinados (11,6%) ninguno de los gatos examinados padecia enfermedades neoplasicas. Los mastocitos epidermicos fueron mas frecuentes en animates que padecian placas eosinofilicas y granulomas eosinofilicos. La gran mayoria de las dermatosis en las cuales se detectan mastocitos epidermicos cuales se se caracterizan por: 1. Una posible patogenia inmunomediada y 2. eosinofilia tisular.

Introduction and importance Langerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people per year. LCH invades the reticuloendothelial system and causes the proliferation of Langerhans cells and mature eosinophils. LCH involving the temporoparietal bone has rarely been reported in the literature. Presentation of case A ten-year-old boy presented to the Neurosurgical outpatient clinic with a swelling on the right temporoparietal region following a fall from his bicycle. Local examination revealed a single, 3 × 3 cm, non-tender, cystic, immobile swelling in the right temporoparietal region. On evaluation for recent head trauma, an incidental finding of eosinophilic granuloma was discovered on a CT scan. The FNAC was suggestive of a histiocytic lesion pertaining to a diagnosis of LCH. The patient underwent wide excision of the mass and cranioplasty. A one-month follow-up CT scan of the head had no evidence of residual or recurrent disease. Discussion Eosinophilic granuloma is one of the three variants of LCH and has a relatively better prognosis. Clinical diagnosis can be challenging and mandates tissue sampling for histopathological examination. Treatment modalities including surgery, radiotherapy, chemotherapy, and steroid injection are used alone, or in combination, depending on the extent and severity of the disease. Conclusion Examining a swelling in the temporoparietal region with no other characteristic symptoms could be a case of LCH. The timely diagnosis and surgical excision with other adjuvant treatment options of this rare pediatric disease would help in a better outcome., Highlights • Langerhans cell histiocytosis can present as a mass in the temporal or parietal region with no symptoms otherwise. • The etiology of Langerhans cell histiocytosis is still unknown and diagnosis can be challenging. • The prognosis depends greatly on early identification and prompt surgical treatment.

Background Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation and infiltration of histiocytic cells. Methods This review focused on the main aspects associated with LCH. Results LCH can involve single or multiple organs and systems, with bone and skin being the most commonly affected sites. Regarding skeletal manifestations, the jawbones are involved in about 20%-30% of all cases. Such lesions may present as unilocular or multilocular images mainly affecting the posterior mandible. Oral soft tissue lesions may also occur, with the gingiva and hard palate being the most frequently affected sites. Conclusion The diagnosis and management of LCH are challenging, requiring a multidisciplinary approach, with dentists playing a central role since oral manifestations can be the first sign of the condition.

Intestinal anisakiasis is not only a rare but also a difficult to diagnose parasitic disease. The symptoms are not specific and are often severe and abrupt; therefore, patients are sometimes diagnosed as having surgical abdomen. The clinical imaging findings are remarkable, including ascites, enteritis, ileus, eosinophilic granuloma and sometimes perforation. We experienced a case of intestinal anisakiasis diagnosed on the basis of the Anisakis-specific immunoglobulin A level from paired sera and treated successfully with conservative therapy, although ileum perforation was complicated by a 50-mm abscess. Even the large abscess could be treated without drainage in thiscase.

Background Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is a rare self-limiting condition of the oral mucosa. The lesion manifests as an isolated ulcer that can be either asymptomatic or associated with mild to severe pain, and in most cases, it affects the tongue. TUGSE lesions may mimic malignancy such as squamous cell carcinoma, CD30 positive lymphoproliferative disorder, or infectious diseases such as primary syphilis, tuberculosis, or Epstein-Barr virus mucocutaneous ulcer. Histologically dominating cells are lymphocytes, histiocytes, and eosinophils. Case presentation We describe a TUGSE case of a patient with a solitary ulcer on the lower left retromolar buccal plane. Upon presentation, the patient reported a swelling on the buccal mucosa of the left lower jaw since 1 year with rapid growth over the last days and mild pain while chewing. The diameter of the intraoral lesion on the lower left retromolar buccal plane was approximately 4 × 3 cm; the lesion presented as indurated base with a central superficial ulceration of 2 × 1 cm, indicative for a malignant process. Histologically, the ulceration showed an expanding, infiltrative, and vaguely granulomatous morphology, involving the superficial mucosa and the fatty tissue, and extended between the deep striated muscle fibers. The lesion was rich in lymphocytes, histiocytes, and eosionophils intermingled with activated T-blasts without phenotypic abnormalities. TUGSE was then diagnosed based on the phenotype (especially the lacking expression of CD30, the retained T-cell phenotype, and the absence of Epstein-Barr virus), the clinical presentation, and the morphology. Twenty-six months after diagnosis, no recurrence of the ulceration was seen. Conclusions As TUGSE may mimic malignancy or infectious diseases, biopsy is mandatory and should be combined with thorough clinical examination. A screening for infectious diseases (mainly syphilis, Epstein-Barr virus, and HIV infections) must be performed routinely. In most cases, the lesions resolve spontaneously, obviating the need of further actions other than clinical follow-up. The pathogenesis of TUGSE lesions is still under debate, although local traumatic events and a locotypic immune response have been suggested to be major contributing factors.

Background Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. Methods We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis. Result After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved. Conclusion Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment., Highlights • Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a rare disorder resulting from an abnormal clonal proliferation of macrophages. • Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses. • In diagnosing LCH a thorough physical examination, Laboratory studies, and radiological studies are mandatory. • Many different treatments for multisystem LCH, from minimal therapy to intensive chemotherapy, have been tried. • Vinblastine and etoposide, have been identified as effective single agents. Both have activity against diverse disorders of the monocyte-macrophage system and combination chemotherapy including them had promising results in multisystem LCH.

Langerhans cell histiocytosis (LCH) is characterised by tissue destruction caused by the abnormal proliferation of pathogenic dendritic cells. We report a rare case of multi-system LCH with local invasion of the orbital apex.A 56-year-old woman suffered from a decrease of visual acuity in the left eye caused by central scotoma and the limitation of eye movement in all directions. Magnetic resonance imaging revealed an enhanced lesion in the left orbital apex, suggesting optic nerve compression. She had been diagnosed with eosinophilic granuloma 24 years previously. Two weeks after the current presentation, we admitted the patient for optic canal and orbital apex decompression and subtotal tumour resection. Histopathological analysis confirmed the diagnosis of LCH. Post-surgical treatment with low-dose cytarabine was initiated for the residual tumour. However, it was ceased because of myelosuppression-induced pyelonephritis. After surgery, the central scotoma disappeared on day 5 and eye movement palsy resolved by 6 months. After the cessation of cytarabine, she has received low-dose steroid therapy for 2 years with no recurrence. Early surgical intervention with low-dose steroid therapy can lead to recovery of visual acuity and resolve eye movement palsy in patients with lesions of the orbital apex caused by multi-system LCH.

Author(s): Johnson, Lynelle R; Johnson, Eric G; Hulsebosch, Sean E; Dear, Jonathan D; Vernau, William | Abstract: BackgroundEosinophilic lung disease is a poorly understood inflammatory airway disease that results in substantial morbidity.ObjectiveTo describe clinical findings in dogs with eosinophilic lung disease defined on the basis of radiographic, bronchoscopic, and bronchoalveolar lavage fluid (BAL) analysis. Categories included eosinophilic bronchitis (EB), eosinophilic granuloma (EG), and eosinophilic bronchopneumopathy (EBP).AnimalsSeventy-five client owned dogs.MethodsMedical records were retrospectively reviewed for dogs with idiopathic BAL fluid eosinophilia. Information abstracted included duration and nature of clinical signs, bronchoscopic findings, and laboratory data. Thoracic radiographs were evaluated for the pattern of infiltrate, bronchiectasis, and lymphadenomegaly.ResultsThoracic radiographs were normal or demonstrated a bronchial pattern in 31 dogs assigned a diagnosis of EB. Nine dogs had intraluminal mass lesions and were bronchoscopically diagnosed with EG. The remaining 35 dogs were categorized as having EBP based on radiographic changes, yellow green mucus in the airways, mucosal changes, and airway collapse. Age and duration of cough did not differ among groups. Dogs with EB were less likely to have bronchiectasis or peripheral eosinophilia, had lower total nucleated cell count in BAL fluid, and lower percentage of eosinophils in BAL fluid compared to dogs in the other 2 groups. In contrast to previous reports, prolonged survival (g55 months) was documented in dogs with EG.Conclusions and clinical importanceDogs with eosinophilic lung disease can be categorized based on imaging, bronchoscopic and BAL fluid cytologic findings. Further studies are needed to establish response to treatment in these groups.