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Letterer-Siwe disease presenting with gastrointestinal and cutaneous manifestations.
Dermatol Online J
December 2023
Federal State Autonomous Educational Institution of Higher Education "Peoples' Friendship University of Russia" of the Ministry of Education and Science of the Russian Federation, Children's City Clinical Hospital named after PER, Bashlyaeva, Moscow Department of Health, Moscow, Russia.
Histiocytosis is a set of distinct proliferative illnesses defined by the proliferation and infiltration of varied numbers of dendritic cells, macrophages, and monocytes in the afflicted tissues. The skin and other organs may be impacted by the inflammatory infiltration. It can occur at any age.
View Article and Find Full Text PDFOral mucosal lesions with transient self-healing of Langerhans cell histiocytosis: a case report.
Hua Xi Kou Qiang Yi Xue Za Zhi
October 2023
State Key Laboratory of Oral Diseases & National Center for Stomatology & National Clinical Research Center for Oral Diseases & Dept. of Oral Medicine, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.
Article Synopsis
- - Langerhans cell histiocytosis (LCH) is a condition where there is an abnormal increase of Langerhans cells, leading to various health issues, particularly in children.
- - LCH has four types: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, and congenital self-healing LCH, with symptoms depending on the organs involved.
- - The study discusses a unique case of LCH that temporarily healed on its own, which could help improve understanding and treatment of this disease.
Multifocal, Multisystem Presentation of Adult-Onset Langerhans Cell Histiocytosis on 18F-Fluorodeoxyglucose Positron-Emission Tomography-Computed Tomography: A Rare Case Report.
Indian J Nucl Med
March 2022
Department of Pathology, Max Super Speciality Hospital, Shalimarbagh, New Delhi, India.
Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a rare systemic disorder arising from clonal proliferation of immature CD207-positive (langerin) myeloid dendritic cells (histiocytes) in the skin and visceral organs with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. The incidence of LCH is very less in adult and occurs almost exclusively in children. Genital, perianal, and lung lesions are considered to be rare manifestations of adult LCH.
View Article and Find Full Text PDFUnique Case of Congenital Langerhans Cell Histiocytosis Presenting as Intrauterine Fetal Demise.
Pediatr Dev Pathol
July 2022
Department of Pathology, 54473McGill University, Montreal, QC, Canada.
Congenital Langerhans cell histiocytosis (LCH) (formerly called Letterer-Siwe disease) is characterized by a clonal proliferation of Langerhans cells occurring in children at birth and manifests typically with multifocal cutaneous lesions, hepatosplenomegaly, lymphadenopathy, pulmonary lesions, and destructive osteolytic bone lesions. We present a case of LCH involving multiple systems high-risk organs (LCH MS-RO+), in a 32-week stillborn from a 20-year-old G2A1. The fetus was mildly hydropic and pale.
View Article and Find Full Text PDFRecurrent pulmonary infections as the first presentation of Letterer Siwe disease.
Oxf Med Case Reports
July 2021
Department of Dermatology, Faculty of Medicine, Tishreen University Hospital, Lattakia, Syria.
Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology that affects several organs. The fatal type of Langerhans cell histiocytosis is called Letterer Siwe disease (LSD) which is multisystem with a poor prognosis. Herein, we report a 20-month-old male who was admitted for recurrent pulmonary infections at the age of 10 months.
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