3,271 results match your criteria Eosinophilic Granuloma Histiocytosis X


Multisystem Langerhans cell histiocytosis: Literature review and case report.

Radiol Case Rep 2022 May 2;17(5):1407-1412. Epub 2022 Mar 2.

Department of Radiology, Pham Ngoc Thach University of Medicine, Ho Chi Minh, Vietnam.

Langerhans cell histiocytosis (LCH) refers to a group of diseases of unknown etiology, typically discovered in childhood, characterized by the accumulation of Langerhans cells (white blood cells with large cell nuclei that may contain cytoplasmic histiocytosis X bodies) involving one or more organ systems, including bones, lungs, pituitary gland, skin, lymph nodes, and liver. This disease is also known as histiocytosis X or eosinophilic granuloma. Pulmonary LCH is common (identified in 40% of LCH patients) and may be isolated to the lung or involve other organs. Read More

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2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.

Ann Rheum Dis 2022 03 2;81(3):309-314. Epub 2022 Feb 2.

University of Pennsylvania, Philadelphia, Pennsylvania, USA

Objective: To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Patients 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. Read More

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Metachronous Eosinophilic Granuloma of Rib in an Adult Patient.

Cureus 2021 Dec 24;13(12):e20670. Epub 2021 Dec 24.

Thoracic Surgery, Gazi University School of Medicine, Ankara, TUR.

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. Read More

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December 2021

Unique Recognizable Histopathologic Variant of Palisaded Neutrophilic and Granulomatous Dermatitis that Is Associated With SRSF2-Mutated Chronic Myelomonocytic Leukemia: Case Report and Review of the Literature.

Am J Dermatopathol 2022 Mar;44(3):e33-e36

Department of Dermatology, Henry Ford Health System, Detroit, MI; and.

Abstract: 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. Read More

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First technical report of a pediatric case with thoracic Langerhans cell histiocytosis: Gross total tumor removal, corpectomy, and 360° stabilization via posterolateral approach at a single stage.

J Craniovertebr Junction Spine 2021 Jul-Sep;12(3):236-239. Epub 2021 Sep 8.

Department of Neurosurgery, Ankara University School of Medicine, Ibn-i Sina Hospital, Ankara, Turkey.

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. Read More

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September 2021

Ophthalmic histiocytic lesions: a baseline demographic and clinicopathological study of 28 cases from two eye centers.

Int Ophthalmol 2022 Apr 2;42(4):1221-1232. Epub 2021 Nov 2.

Pathology and Laboratory Medicine Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.

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. Read More

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Eosinophilic Granuloma of the Liver Mimicking Metastatic Liver Tumor.

Intern Med 2022 May 19;61(10):1511-1517. Epub 2021 Oct 19.

Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan.

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. Read More

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IgG anti-Pentraxin 3 antibodies are a novel biomarker of ANCA-associated vasculitis and better identify patients with eosinophilic granulomatosis with polyangiitis.

J Autoimmun 2021 11 14;124:102725. Epub 2021 Sep 14.

Division of Rheumatology, Department of Medicine DIMED, University of Padova, Italy. Electronic address:

Objective: To investigate prevalence of anti-Pentraxin 3 (PTX3) antibodies in sera of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients.

Methods: 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. Read More

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November 2021

Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE): a rare self-healing oral mucosal lesion.

BMJ Case Rep 2021 Aug 13;14(8). Epub 2021 Aug 13.

Oral & Maxillofacial Pathology & Microbiology, Institute of Dental Sciences, Siksha O Anusandhan Deemed to be University, Bhubaneswar, Odisha, India

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Eosinophilic Granuloma: A Rare and Often Benign Condition Presenting as a Lump on the Head, which was Easily Treated.

Eur J Case Rep Intern Med 2021 20;8(7):002727. Epub 2021 Jul 20.

Department of Internal Medicine, Centro Hospitalar de Leiria, Leiria, Portugal.

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. Read More

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Incidental finding of Langerhans cell histiocytosis of temporoparietal bone - A case report.

Int J Surg Case Rep 2021 Aug 7;85:106179. Epub 2021 Jul 7.

Department of Neurosurgery, Maharajgunj Medical Campus, Institute of Medicine, Kathmandu, Nepal.

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. Read More

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Eosinophilic granulomatosis with polyangiitis present and future.

Rheumatology (Oxford) 2021 06;60(Suppl 3):iii24-iii27

Department of Medicine, University of Cambridge, Cambridge Biomedical Campus, Cambridge, UK.

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Langerhans cell histiocytosis in the oral and maxillofacial region: An update.

J Oral Pathol Med 2021 Jul 20;50(6):565-571. Epub 2021 Jun 20.

Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.

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. Read More

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Recurrent eosinophilic granuloma involving maxilla and mandible in an adult male: an unusual case report.

Authors:
X Xie J Wang Y Ding

Aust Dent J 2021 03 11;66 Suppl 1:S88-S92. Epub 2021 Jul 11.

State Key Laboratory of Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, China.

Langerhans' cell histiocytosis (LCH) is a disease spectrum characterized by the intense infiltration/accumulation of Langerhans' cells in the affected tissues. Eosinophilic granuloma is the mildest form of LCH which mainly affects the skull, mandible, ribs and femur in children and young adults but rarely occurs in the maxilla. Here, we describe an unusual case of eosinophilic granuloma affecting the tissues adjacent to tooth #25 and tooth #46 (FDI numbering system) in a 35-year-old male. Read More

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Multifocal eosinophilic granuloma of the jaws with long-term follow-up: a case report.

Hua Xi Kou Qiang Yi Xue Za Zhi 2021 Jun;39(3):355-361

State Key Laboratory of Oral Diseases & National Clinical Research Center for Oral Diseases & Dept. of Periodontics, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.

Eosinophilic granuloma, a rare disease, has various clinical manifestations and no specific X-rays features and is thus easily misdiagnosed. This paper reports a case of multifocal eosinophilic granuloma of jaw with long-term follow-up. The patient initially presented with periodontal tissue destruction. Read More

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Multisystem Langerhans Cell Histiocytosis in an infant.

Radiol Case Rep 2021 Jul 6;16(7):1798-1805. Epub 2021 May 6.

Department of Radiology, Madigan Army Medical Center, 9040A Jackson Ave, Joint Base Lewis-McChord, WA 98431, USA.

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. Read More

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Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review.

Otolaryngol Head Neck Surg 2022 01 4;166(1):48-59. Epub 2021 May 4.

Hackensack University Medical Center, Hackensack, New Jersey, USA.

Objective: To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management.

Data Sources: PubMed/MEDLINE, Embase, and Cochrane Library.

Review Methods: A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. Read More

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January 2022

Eosinophilic granuloma of cranial location: report of a case and bibliographic review

Rev Fac Cien Med Univ Nac Cordoba 2021 03 12;78(1):48-51. Epub 2021 Mar 12.

Hospital Clinico Universitario Lozano Blesa. Zaragoza. España.

Introduction: Eosinophilic granuloma is an unusual benign disease that usually affects the pediatric population and young adults. It is the most benign of the diseases traditionally known as histiocytosis X that are now called Langerhans cell histiocytosis.

Clinical Case: Pediatric patient with a painful lump in the temporal region. Read More

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Role of Stereotactic Radiosurgery in Intracranial Histiocytosis: a Systematic Review of Literature of an Emerging Modality for Localized Disease.

World Neurosurg 2021 06 17;150:64-70. Epub 2021 Mar 17.

Department of Radiation Therapy, Postgraduate Institute of Medical Education and Research, Chandigarh. Electronic address:

Background: A histiocytosis is a group of immunoproliferative disorders of clonal cells. The management protocols are still evolving, with chemotherapy as the mainstay of treatment.

Objective: This study aims to evaluate the feasibility, safety, efficacy, and complication profile of stereotactic radiosurgery for intracranial histiocytosis. Read More

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Multifocal Langerhans Cell Histiocytosis: A Unique Presentation of Eosinophilic Granuloma with Intracranial Extension, Massive Lymphadenopathy, and Epstein-Barr Viral Infection in an Adolescent Female.

Case Rep Dermatol 2021 Jan-Apr;13(1):28-35. Epub 2021 Jan 25.

Division of Dermatology, Loyola University Chicago Stritch School of Medicine, Maywood, Illinois, USA.

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. Read More

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January 2021

Unisystem Langerhans cell histiocytosis in maxillofacial region in pediatrics: comprehensive and systematic review.

Oral Maxillofac Surg 2021 Dec 16;25(4):429-444. Epub 2021 Feb 16.

Department of Dentistry, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

Background: 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. Read More

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December 2021

Solitary tibial lesion as the initial presentation of Langerhans cell histiocytosis: report of two cases and literature review.

J Int Med Res 2021 Jan;49(1):300060520982826

Department of Orthopaedic Surgery, National Taiwan University Hospital, Taipei, Taiwan.

The various presentations of osseous Langerhans cell histiocytosis (LCH) make it difficult to distinguish from other bone diseases. In addition, there is no universally accepted protocol for managing osseous LCH for single non-central nervous system-risk lesions. Here, the rare cases of two paediatric patients, aged 1 and 2 years, who presented with a solitary tibial lesion at time of LCH diagnosis, are reported. Read More

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January 2021

Histiocytosis in the pediatric spine: a clinical and radiographic analysis of 50 patients.

Spine Deform 2021 05 5;9(3):823-831. Epub 2021 Jan 5.

Servicio de Patología Espinal, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Combate de los Pozos 1881, C1245AAM, Buenos Aires, CABA, Argentina.

Study Design: Descriptive, retrospective. Scientific level of evidence IV.

Objectives: The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Read More

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Histopathologic reaction patterns to differentially cross-linked hyaluronic acid fillers: A retrospective case series.

J Cutan Pathol 2021 Jun 10;48(6):758-762. Epub 2021 Jan 10.

The Ronald O. Perelman Department of Dermatology, New York School of Medicine, New York, New York, USA.

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. Read More

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Eosinophilic Granuloma of the Skull Presenting as Non-Traumatic Extradural Haematoma in Children.

Int J Gen Med 2020 24;13:1229-1234. Epub 2020 Nov 24.

Neurosciences Research Centre, Molecular and Clinical Sciences Research Institute, St George's University of London, London, UK.

Cranial extradural haematoma (EDH) is a neurosurgical emergency that can be caused by traumatic or non-traumatic causes with the former being more prevalent. Non-traumatic causes are variable and can include infection, vascular malformation and haematological disorders. This paper will address an extremely rare non-traumatic cause of EDH. Read More

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November 2020

Cardiac involvement in the adult primary vasculitides.

Expert Rev Clin Immunol 2020 10 24;16(10):985-991. Epub 2020 Dec 24.

Rheumatology Unit, Department of Internal Medicine, Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia , Reggio Emilia, Italy.

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]. Read More

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October 2020

Eosinophilic Granuloma of the Mandible.

Ann Maxillofac Surg 2020 Jan-Jun;10(1):254-257. Epub 2020 Jun 8.

Department of Oral and Maxillofacial Surgery, Pushpagiri College of Dental Sciences, Thiruvalla, Kerala, India.

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. Read More

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Nasal polyps in eosinophilic granulomatosis with polyangiitis: Structured histopathology and CD105 expression.

Am J Otolaryngol 2020 Nov - Dec;41(6):102661. Epub 2020 Aug 10.

Department of Neuroscience - DNS, Otolaryngology Section, Padova University, Via Giustiniani 2, 35128 Padova, Italy. Electronic address:

Purpose: Distinguishing the prodromal nasal polyposis of eosinophilic granulomatosis with polyangiitis (EGPA) from chronic rhinosinusitis with nasal polyps (CRSwNP) is a challenge for rhinologists and rheumatologists. It has recently been reported that angiogenesis and CD105 expressed on vascular endothelial cells could have a role in the pathogenesis and development of nasal polyps. This exploratory study examined the structured histopathology of nasal polyps in patients with EGPA and CRSwNP, comparing CD105 expression in their nasal tissue with that of a control group with no chronic sinonasal inflammation. Read More

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December 2020

Vertebra Plana in Children May Result from Etiologies Other Than Eosinophilic Granuloma.

Clin Orthop Relat Res 2020 10;478(10):2367-2374

F. Baky, T. A. Milbrandt, C. Arndt, M. T. Houdek, A. N. Larson, Department of Orthopedic Surgery, Mayo Clinic, Rochester MN, USA.

Background: Vertebra plana in children is a diagnostic dilemma for orthopaedic surgeons. This radiographic finding sometimes has been said to be pathognomonic for eosinophilic granuloma (Langerhans cell histiocytosis); however, vertebra plana may also be caused by a range of other conditions. We sought to determine whether vertebra plana can be associated with malignancies other than eosinophilic granuloma. Read More

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October 2020