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Alma V Huidobro-Chávez, Gianmarco D Vigo Pareja, Carlos Pachas-Peña, Karina Patiño-Calla
The Langerhans cell histiocytosis is a rare disease characterized by the clonal proliferation of CD1a + myeloid dendritic cells associated with a significant inflammatory component. The localized form of the disease is called eosinophilic granuloma. Bone involvement is common; in children, lytic lesions are most frequently found in the cranial dome being rare in the orbit. We present an 18-month-old infant who consulted due to periorbital edema and proptosis of the right eye, with two months of evolution. The computed tomography and the magnetic resonance imaging showed a maxillary sinus tumor mass of expansive growth and erosion of the roof of the orbit...
April 1, 2018: Archivos Argentinos de Pediatría
Shinsaku Imashuku, Miyako Kobayashi, Yoichi Nishii, Keisuke Nishimura
Diagnosis and treatment of Langerhans cell histiocytosis (LCH) in elderly patients are often difficult. We report here a 61-year-old female suffering from a refractory axillary ulcer for nearly a year, whose biopsy revealed LCH. It was also noted that the patient had other cutaneous papulovesicular eruptions of LCH as well as central diabetes insipidus. The patient was first successfully treated with multiagent chemotherapy (cytosine arabinoside/vinblastine/prednisolone). DDAVP also well controlled diabetes insipidus; however, the axillary ulcer and cutaneous LCH relapsed...
2018: Case Reports in Dermatological Medicine
José Manuel Fernández Armenteros, Nuria Arco Huguet, Veronica Sanmartin Novell, Felip Vilardell Villellas, Ana Velasco Sanchez, Elisa Martró Català, Rosa Maria Martí Laborda, Xavier Soria Gili
Langerhans cell histiocytosis (LCH) is a disorder characterized by accumulation of Langerhans-like cells in one or various organs. A correct staging work-up is essential since there are multiorgan presentations with a poor prognosis. We report three patients with LCH skin lesions mimicking molluscum contagiosum in association with both high and low risk organ involvement. This peculiar cutaneous presentation can be a clue for the diagnosis of LCH, a disease with potentially severe systemic involvement.
March 12, 2018: Pediatric Blood & Cancer
Vitor Gabriel Ribeiro Grossi, Karen Lee, Léa Maria Demarchi, Jussara Bianchi Castelli, Vera Demarchi Aiello
Mesothelial/monocytic incidental cardiac excrescences (MICE) are unusual findings during the histological analysis of material from the pericardium, mediastinum, or other tissues collected in open-heart surgery. Despite their somewhat worrisome histological appearance, they show a benign clinical course, and further treatment is virtually never necessary. Hence, the importance of recognizing the entity relays in its differential diagnosis, as an unaware medical pathologist may misinterpret it for a malignant neoplasm...
January 2018: Autopsy & Case Reports
Ifeyinwa E Obiorah, Alicia Henao Velasquez, Bhaskar Kallakury, Metin Özdemirli
BACKGROUND: Langerhans cell histiocytosis is characterized by abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population and presentation in adults remain a rare event. The presentation of langerhans cell histiocytosis is highly variable but skin, bone and lung involvement are very common. Langerhans cell histiocytosis presenting as a bile duct mass is rare and usually occurs as part of a multi-organ system disease. CASE REPORT: We present a case of langerhans cell histiocytosis confined to the extrahepatic bile duct in a 62-year-old female with sclerosing cholangitis...
March 8, 2018: Balkan Medical Journal
Gaurav Narula, Nirmalaya D Pradhan, Brijesh Arora, Sripad D Banavali
BACKGROUND: Involvement of risk-organs (RO+) in Langerhans cell histiocytosis (LCH) and inadequate early response identifies patients at high risk for relapse and mortality requiring intensive salvage therapy including stem cell transplant, adding cost and toxicity. To mitigate this, we used a standard induction, augmented with metronomic etoposide, and prolonged maintenance-similarly augmented for RO+, and retrospectively analyzed its impact. PROCEDURE: LCH patients from 2009 through 2014 were included...
March 7, 2018: Pediatric Blood & Cancer
Lauren A Dalvin, Diva R Salomão, Sanjay V Patel
AIM: To determine population-based incidence of conjunctival tumours in Olmsted County, Minnesota. METHODS: The Rochester Epidemiology Project medical record linkage system was used to identify patients with conjunctival tumours in Olmsted County (1 January 1980 to 31 December 2015). Records were reviewed for demographics, types of tumours, histopathology, treatment and clinical course. Incidence rate of all tumours was calculated per 1 000 000 person-years. Poisson regression analysis was used to assess changes in incidence over time...
March 6, 2018: British Journal of Ophthalmology
XiangYu Xu, SongBo Han, Liang Jiang, ShaoMin Yang, XiaoGuang Liu, HuiShu Yuan, Feng Wei, FengLiang Wu, Lei Dang, Hua Zhou, Hua Zhang, ZhongJun Liu
BACKGROUND CONTEXT: Langerhans cell histiocytosis (LCH) of the spine is a relatively rare condition with unknown etiology. The diagnosis and treatment protocols for spine LCH remain controversial. PURPOSE: In this study, we evaluated the efficacy and safety of our proposed diagnosis and treatment protocol introduced in 2009. STUDY DESIGN: This is a retrospective study. PATIENT SAMPLE: One hundred and ten patients with spine LCH who had been diagnosed and treated in our hospital from October 1997 to November 2015 were included in this study...
March 2, 2018: Spine Journal: Official Journal of the North American Spine Society
Omar Hariri, Seckin Ulualp, Ron B Mitchell
No abstract text is available yet for this article.
January 2018: Ear, Nose, & Throat Journal
Mouhamad Nasser, Julie Traclet, Vincent Cottin
Cladribine therapy may be beneficial in advanced forms of pulmonary Langerhans cell histiocytosis, even that with multiple cystic changes
January 2018: ERJ Open Research
Katherine M Stiff, Philip R Cohen
Scabies, a mite infestation caused by 'Sarcoptes scabiei', most commonly presents as pruritic linear burrows where the mite has invaded the skin. Scabies variant such as bullous, crusted, hidden, incognito, nodular and scalp-mimic the other conditions. In addition, atypical presentations of scabies can masquerade as dermatitis herpetiformis, ecchymosis, Langerhans cell histiocytosis, systemic lupus erythematosus, urticaria, and urticaria pigmentosa. A 59-year-old male presented with non-pruritic papulosquamous plaques on his chest, abdomen, and back resembling lesions of pityriasis rosea in morphology and distribution...
December 19, 2017: Curēus
Shweta Bansal, Arun Garg, Richa Khurana
Langerhans cell histiocytosis (LCH) is a group of rare disorders histologically characterized by the proliferation of LC, involving multiple organs and systems. Typically, there is bone involvement and, less frequently, lesions may be found in the lungs, liver, lymph nodes, skin, and mucosae. Oral soft tissue lesions without bone involvement are rare. Antigenic markers that react with CD1a glycoprotein, cytoplasmatic protein S100 detected by immunoperoxidase staining, and/or presence of Birbeck granules on electron microscopic examination are required for a definitive diagnosis of LCH...
October 2017: Journal of Cutaneous and Aesthetic Surgery
Esma A Akin, Murat Osman, Amy L Ellenbogen
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis with deposition of lipid-laden macrophages in numerous organs. A 74-year-old man with a history of coronary artery disease, hypertension, and hyperlipidemia presented with nonspecific symptoms including back pain, nausea, vomiting, vertigo, and left leg pain. A neutrophil-predominant elevated WBC count and a bone biopsy revealing histiocytic proliferation positive for CD68 and CD163 and negative for S100 was noted. FDG PET/CT, MRA, and CTA images were obtained...
February 27, 2018: Clinical Nuclear Medicine
Uwe Wollina, Jacqueline Schönlebe, Georgi Tchernev, Torello Lotti
Diffuse normolipemic plane xanthoma (DNPX) is an uncommon subtype of non-Langerhans histiocytosis. DNPX is characterised by xanthelasma palpebrarum, diffuse plane xanthoma of the head, neck, trunk, or extremities, and normal plasma lipid levels. The neck is the most common site. We report about a 62-year-old female Caucasian patient, who developed an asymptomatic fine wrinkling and loose skin on the neck and décolleté about three years ago. The skin colour became yellowish. Xanthelasma was absent. Histopathology of a skin biopsy confirmed the diagnosis of DNPX...
January 25, 2018: Open Access Macedonian Journal of Medical Sciences
Pooja R Shah, Michelle A Pitch, Glynis Scott, Mary Gail Mercurio
No abstract text is available yet for this article.
February 23, 2018: Journal of Lower Genital Tract Disease
Ankush Bhatia, Gary Ulaner, Raajit Rampal, David M Hyman, Omar Abdel-Wahab, Benjamin H Durham, Ahmet Dogan, Neval Ozkaya, Mario E Lacouture, Julio Hajdenberg, Chezi Ganzel, Eli L Diamond
No abstract text is available yet for this article.
February 22, 2018: Haematologica
Noah A Brown, Kojo S J Elenitoba-Johnson
PURPOSE OF REVIEW: Langerhans cell histiocytosis (LCH) is a neoplasm of dendritic cells with a wide clinical spectrum. Localized pulmonary LCH occurs in young adults with a history of smoking and can either resolve spontaneously or lead to progressive decline in pulmonary function. Young children can also present with localized disease - frequently bone or skin - or with multifocal or multisystem disease. Clinical outcomes in these patients also vary widely, ranging from spontaneous resolution to multiorgan failure and death...
February 20, 2018: Current Opinion in Pulmonary Medicine
Qiping Chen, Joyce Siong-See Lee, Weiting Michelle Liang, Mei Fung Chan, Ramón M Pujol, Requena Luis, Suat Hoon Tan
Indeterminate cell histiocytosis (ICH) is an extremely rare cutaneous neoplastic disorder. It has the immunophenotypic features of both Langerhans and non-Langerhans cell histiocytosis. We report here a case of a healthy young Chinese woman who presented with disfiguring thick, infiltrated cutaneous nodules on the face, trunk and extremities which appeared progressively over a period of 4 years. No systemic involvement has been detected so far. Results of a skin biopsy showed diffuse dermal infiltration of histiocytoid cells with indented nuclei and positive staining for S100 and CD1a and negativity for CD207 (langerin)...
February 22, 2018: Journal of Cutaneous Pathology
Maria Papadopoulou, Paraskevi Panagopoulou, Anastasia Papadopoulou, Emmanuel Hatzipantelis, Ioannis Efstratiou, Assimina Galli-Tsinopoulou, Efimia Papadopoulou-Alataki
Langerhans cell histiocytosis (LCH) is a rare hematologic disorder that results from the clonal multiplication and accumulation of immature dendritic Langerhans cells. Its reported incidence rate varies, but is considered to be 2.6-8.9 per million children who are <15 years of age each year. It may affect any system or organ. The present study reported 4 pediatric LCH cases in order to highlight the heterogeneity of the initial presentation, and the pitfalls that may mislead clinicians and delay diagnosis...
March 2018: Molecular and Clinical Oncology
Maria Francesca Gicchino, Mario Diplomatico, Carmela Granato, Daniela Capalbo, Pierluigi Marzuillo, Alma Nunzia Olivieri, Emanuele Miraglia Del Giudice
BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO), also known as chronic nonbacterial osteomyelitis, is a rare, noninfectious inflammatory disorder that causes multifocal bone lesions with swelling and pain. Lytic and sclerotic bone lesions could be found on X-ray. Short tau inversion recovery magnetic resonance imaging (STIR MRI) shows bone marrow oedema, bone expansion, lytic areas and periosteal reaction. CRMO is characterized by periodic exacerbations and remissions of unclear/unknown pathogenesis...
February 17, 2018: Italian Journal of Pediatrics
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