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Langerhans histiocytosis

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https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#1
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28087041/dermoscopy-of-langerhans-cell-histiocytosis
#2
Biswanath Behera, Munisamy Malathi, Nagendran Prabhakaran, Kamat Divya, Devinder Mohan Thappa, Bheemanathi Hanuman Srinivas
No abstract text is available yet for this article.
February 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28078666/pediatric-erdheim-chester-disease-with-aggressive-skin-manifestations
#3
H-H Su, W Wu, Y Guo, H-D Chen, S-J Shan
Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68+/CD1a- foamy histiocytes. It is extremely rare in children. The skin lesions of pediatric ECD was not systemically described before. We report a case of ECD in a 3.5 year old Chinese boy. The patient present with generalized skin and bone involvement for 3 years duration. Marked generalized annular maculopapular lesions with central atrophy which differed from previously reported adult xanthoma-like papules or periorbital xanthelasma-like lesions...
January 12, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28077484/-bizarre-rash-adult-onset-cutaneous-langerhans-cell-histiocytosis
#4
Andre Boo Shern Khoo, Jennifer Garioch
No abstract text is available yet for this article.
January 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28076684/radiographic-differentiation-of-advanced-fibrocystic-lung-diseases
#5
Masanori Akira
The concept of end-stage lung disease suggests a final common pathway for most diffuse parenchymal lung diseases. In accordance with this concept, end-stage disease is characterized radiographically and pathologically by the presence of extensive honeycombing. However, sequential computed tomographic (CT) scans obtained from patients with chronic diffuse lung disease evolve over time to show various advanced lung disease patterns other than honeycombing. Also, several radiographically distinct honeycomb patterns, including microcystic, macrocystic, mixed, and combined emphysema and honeycombing differentiate one advanced lung disease from another...
January 11, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28072848/physiologic-determinants-of-exercise-capacity-in-pulmonary-langerhans-cell-histiocytosis-a-multidimensional-analysis
#6
Camille Rolland-Debord, Stephanie Fry, Jonathan Giovannelli, Carole Langlois, Nicolas Bricout, Bernard Aguilaniu, Agnes Bellocq, Olivier Le Rouzic, Stephane Dominique, Alain Delobbe, Geraldine François, Abdellatif Tazi, Benoit Wallaert, Cecile Chenivesse
BACKGROUND: Reduced exercise capacity severely impacts quality of life in pulmonary Langerhans cell histiocytosis. Ascertaining mechanisms that impair exercise capacity is necessary to identify targets for symptomatic treatments. METHODS: Dyspnea, pulmonary function tests and cardiopulmonary exercise test were analysed in 62 study participants. Data were compared between subjects with impaired and normal aerobic capacity (V'O2 peak less than 84% versus 84% predicted or more)...
2017: PloS One
https://www.readbyqxmd.com/read/28061539/langerhans-cell-histiocytosis-of-skull-a-retrospective-study-of-18-cases
#7
Xiang-Heng Zhang, Ji Zhang, Zheng-He Chen, Ke Sai, Yin-Sheng Chen, Jian Wang, Chao Ke, Chen-Chen Guo, Zhong-Ping Chen, Yong-Gao Mou
BACKGROUND: The present study presents 18 cases of Chinese patients harboring a Langerhans cell histiocytosis (LCH) of the skull. METHODS: Eighteen consecutive patients were diagnosed as LCH of the skull and confirmed pathologically between March 2002 and February 2014. In the present study, the patients of LCH without skull involvement were excluded. According to disease extent at diagnosis, the 18 LCH patients with skull involvement were divided into three groups: (I) unifocal-monosystem group, including ten cases with solitary skull lesion; (II) multifocal-monosystem group, including two cases with multiple bone lesions and no extra-skeletal involvement; (III) multisystem group, including six cases with LCH lesions involving both skeletal and extra-skeletal system...
November 28, 2016: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/28058158/a-rare-cause-of-proptosis-in-childhood-langerhans-cell-histiocytosis
#8
Mustafa Vatansever, Esra Vatansever, Erdem Dinç, Ayça Sarı, Tuba Kara
A three-year-old male patient was admitted to the clinic with proptosis in his right eye. He had a history of fever with an unknown etiology. In examination, right proptosis was observed and an immobile mass was palpated at the lateral wall of the right orbita. Eye movement was unrestricted in all directions and anterior and posterior segment examination was normal in both eyes. On computed tomography, diffuse bone destruction and expansion was observed in the right orbital lateral wall and other cranial bones...
August 2016: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28045847/langerhans-cell-histiocytosis-presenting-as-chronic-otitis-externa
#9
William P McCullough, Avrum N Pollock
No abstract text is available yet for this article.
January 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28000234/eruptive-xanthogranuloma-in-a-healthy-adult-male
#10
Ashley Ederle, Kevin H Kim, Jerad M Gardner
Xanthogranuloma is a benign, non-Langerhans cell histiocytosis primarily diagnosed in infants and children, although a subset occurs in adults. Multifocal eruptive presentation of xanthogranuloma is very rare with only four previous cases reported in the literature to our knowledge. We describe a case of eruptive xanthogranuloma in a 49-year-old man who presented with sudden onset of numerous asymptomatic, red-yellow to orange papules on the face, scalp, axilla, flank, and scrotum. Histologic features were consistent with xanthogranuloma with diffuse mixed infiltrate of foamy histiocytes, Touton giant cells, and lymphocytes...
December 21, 2016: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/27990389/laryngeal-involvement-with-fatal-outcome-in-progressive-nodular-histiocytosis-a-rare-case-report
#11
Aarti Salunke, Vasudha Belgaumkar, Ravindranath Chavan, Rinkesh Dobariya
Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive noduloulcerative lesions, ichthyotic patches, and laryngeal involvement culminating in fatal outcome prior to therapeutic intervention. Although the presenting features were baffling, histopathology and immunohistochemistry clinched the diagnosis of PNH...
November 2016: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/27981225/langerhans-cell-histiocytosis-presenting-as-fingernail-changes
#12
Ignasi Figueras-Nart, Asunción Vicente, Júlia Sánchez-Schmidt, Cristina Jou-Muñoz, Xavier Bordas-Orpinell, Veronica Paola Celis-Passini, Ofelia Cruz-Martínez, Maria Antonia González-Ensenyat
No abstract text is available yet for this article.
November 2016: JAAD Case Reports
https://www.readbyqxmd.com/read/27951529/predictor-variables-of-developing-anterior-pituitary-deficiencies-in-a-group-of-paediatric-patients-with-central-diabetes-insipidus-and-langerhans-cell-histiocytosis
#13
Elisa Vaiani, Carmen Malossetti, Lina Margarita Vega, Pedro Zubizarreta, Jorge Braier, Alicia Belgorosky
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Central diabetes insipidus (CDI) is the most frequent endocrine manifestation and is a known risk factor for the development of further anterior pituitary hormone deficiencies (APD). However, not all CDI patients develop APD, as observed during prolonged periods of follow-up. AIM: To find predictors of developing APD in LCH children with CDI followed in our institution...
December 13, 2016: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/27932189/recurrence-pattern-of-pulmonary-langerhans-cell-histiocytosis-after-lung-transplantation-a-case-report
#14
A Yazicioglu, S Turkkan, F Demirag, M Subasi, E Yekeler
Pulmonary Langerhans cell histiocytosis is an uncommon indication for lung transplantation. The recurrence of the primary pathology after transplantation is rare. Extra-pulmonary involvement of Langerhans cell histiocytosis before transplantation and resuming of smoking after transplantation are risk factors for recurrence. Herein, we present a 48-year-old woman with a recurrence pattern 24 months after bilateral sequential lung transplantation. On thoracic computed tomography, progressive parenchyma infiltrates were present without clinical symptoms...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27920713/late-onset-langerhans-cell-histiocytosis-with-cerebellar-ataxia-as-an-initial-symptom
#15
Jung-Min Pyun, Hyeyoung Park, Kyung Chul Moon, Beomseok Jeon
Late-onset progressive cerebellar ataxia is a diagnostic challenge because of a poor correlation between genotype and phenotype, and a broad range of secondary causes that extend beyond the neurological field. We report the case of a 45-year-old woman admitted after 2 years of slowly progressing cerebellar ataxia, dysarthria, and emotional instability. Notably, she was diagnosed with diabetes insipidus at the age of 35. As 'idiopathic cerebellar ataxia' was suspected, diagnostic tests, including genetic testing as well as serum and cerebrospinal fluid analyses, and brain magnetic resonance imaging (MRI) were performed...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27908938/a-rare-case-of-extensive-cranial-langerhans-cell-histiocytosis-synchronously-presenting-as-otitis-externa-and-giant-cell-arteritis
#16
Adnan Darr, Zahir Mughal, Thomas Martin
Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unpredictable clinical course and varied modes of presentation. The spectrum of presentation is wide, ranging from isolated eosinophilic granulomas to multiple lesions and diffuse systemic involvement. We present the case of a 52-year-old man, who presented with an 8-week history of worsening otalgia and superficial temporal tenderness attributed to otitis externa within the community and subsequently giant cell arteritis. Computed tomography and magnetic resonance imaging were undertaken due to atypical features, which demonstrated bony destruction within the right greater wing of the sphenoid, squamous part of temporal and mastoid bone, with middle cranial fossa communication...
December 1, 2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27898473/histopathological-and-clinical-findings-in-cutaneous-manifestation-of-erdheim-chester-disease-and-langerhans-cell-histiocytosis-overlap-syndrome-associated-with-the-brafv600e-mutation
#17
Julia Liersch, J Andrew Carlson, Jörg Schaller
The overlap of Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) is more common than it was generally accepted. Both diseases seem to be linked by a mutation in oncogenic BRAFV600E, probably an early event which occurs in bone marrow progenitor cells. In this article are described the clinical and histological findings in 2 cases of ECD-LCH overlap syndrome bearing the BRAFV600E mutation in both ECD and LCH lesions in bone and skin. In one case, lesions of ECD and LCH were situated directly site-to-site in the same bone section leading to the assumption of a common myeloid precursor cell for these diseases...
November 23, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27894453/usual-and-unusual-manifestations-of-familial-hemophagocytic-lymphohistiocytosis-and-langerhans-cell-histiocytosis
#18
REVIEW
Craig Erker, Paul Harker-Murray, Julie-An Talano
Familial hemophagocytic lymphohistiocytosis (FHL) and Langerhans cell histiocytosis (LCH) are histiocytic diseases that occur most commonly in young children. Improvements in recognition and treatment have been substantial for both diseases in the past decade, although early and late morbidity continue to be major concerns. These two diagnoses behave differently, although the clinical spectra for both diseases are diverse and can lead to confusion and delays in diagnosis and treatment. This article focuses on the clinical and genetic spectrum of FHL as well as the clinical and treatment variations of LCH...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27889325/-langerhans-cell-histiocytosis-and-erdheim-chester-disease-a-continuity
#19
S Parreau, J Haroche, I Pommepuy, J F Emile, J C Bourras, F Archambeaud
INTRODUCTION: Erdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described. OBSERVATION: A 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Bone biopsy showed langerhans cell histiocytosis CD1a positive with the presence of BRAF V600E mutation...
November 23, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27886798/multicentric-reticulohistiocytosis-rheumatology-perspective
#20
REVIEW
Bahtiyar Toz, Nesimi Büyükbabani, Murat İnanç
Multicentric reticulohistiocytosis (MRH) is a rare, multisystemic non-Langerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. There are no specific laboratory findings for MRH. Diagnosis is based on clinical findings and skin or synovial biopsy results. There is currently no consensus for the treatment of MRH. Here, we review the differential diagnosis and treatment options of MRH from the rheumatologist's perspective. We also report an index case of MRH associated with Sjögren's syndrome and pulmonary embolism...
April 2016: Best Practice & Research. Clinical Rheumatology
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