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xanthogranuloma

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https://www.readbyqxmd.com/read/28626159/limbal-xanthogranuloma-in-a-dog
#1
Juri Ota-Kuroki, Keiichi Kuroki
A 10-year-old female spayed American Pitt Bull Terrier was presented with a slow growing mass on the temporal limbus area of the right eye. Canine nodular granulomatous episclerokeratitis was suspected, and the affected eye was treated with 1% prednisolone acetate ophthalmic suspension and 0.03% Tacrolimus ophthalmic solution. As the lesion did not respond to the medical treatments and continued to grow, the mass was excised by lamellar sclerokeratectomy. Microscopically, the mass was composed of granulomatous inflammation with intrahistiocytic lipids and lakes of acicular cholesterol clefts, histopathology findings consistent with xanthogranuloma...
June 15, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28583250/oral-juvenile-xanthogranuloma-report-of-two-cases
#2
Mônica Simões Israel, Roman Carlos, Fábio Ramôa Pires
Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually occurs in the skin of children. Extracutaneous involvement is rare, and few cases affecting the oral cavity have been reported. The purpose of the present study was to report two cases of oral juvenile xanthogranuloma affecting the lower lip of a 14-year-old girl and the soft palate of a second 14-month-old girl, both present as yellowish papules. The lesions were surgically excised, and histological and immunohistochemical analysis showed a proliferation of non-Langerhans cells histiocytes and foamy cells, fulfilling the morphologic features of juvenile xanthogranuloma...
May 15, 2017: Pediatric Dentistry
https://www.readbyqxmd.com/read/28567509/-clinical-and-histological-spectrum-of-palisaded-granulomatous-dermatitides-granuloma-annulare-necrobiosis-lipoidica-rheumatoid-nodules-and-necrobiotic-xanthogranuloma
#3
REVIEW
B Weidenthaler-Barth
The palisading granulomatous dermatitides comprise a group of different skin diseases with similar histomorphologic forms of granuloma in the skin. Histopathologically characteristic are areas in the reticular dermis and subcutaneous fat with degenerated bundles of collagen surrounded by histiocytes and multinucleate giant cells aligned in a palisade (necrobiotic granuloma). Within the center of palisaded granulomas, mucin or fibrin can be found. The skin diseases presenting histologically with palisading granuloma are granuloma annulare and necrobiosis lipoidica...
May 31, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28551613/erdheim-chester-disease-comprehensive-review-of-molecular-profiling-and-therapeutic-advances
#4
REVIEW
Faysal Haroun, Kristen Millado, Imad Tabbara
The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E)...
June 2017: Anticancer Research
https://www.readbyqxmd.com/read/28522113/neonatal-glaucoma-associated-with-juvenile-xanthogranuloma-case-report
#5
A Ramos Suárez, I Ayet Roger, A Serra Castanera
CASE REPORT: The case concerns a 22 day-old male child with juvenile xanthogranuloma (JXG), which manifested as a unilateral glaucoma and with fibrinous haemorrhagic exudate in the anterior chamber affecting the angle of the right eye. Despite a high level of suspicion, the definitive diagnosis was not possible until the infant reached the age of 10 months, after the appearance of the skin lesions typical of this condition and histopathological study of them. CONCLUSION: JXG is a rare disease, characterised by yellowish skin lesions on the trunk, neck, or head...
May 15, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28512266/activating-mapk1-erk2-mutation-in-an-aggressive-case-of-disseminated-juvenile-xanthogranuloma
#6
Rikhia Chakraborty, Oliver A Hampton, Harshal Abhyankar, Daniel J Zinn, Amanda Grimes, Brooks Skull, Olive Eckstein, Nadia Mahmood, David A Wheeler, Dolores Lopez-Terrada, Tricia L Peters, John M. Hicks, Tarek Elghetany, Robert Krance, Poulikos I Poulikakos, Miriam Merad, Kenneth L McClain, Carl E Allen, Donald W. Parsons
Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that is usually benign and self-limiting. We present a case of atypical, aggressive JXG harboring a novel mitogen-activated protein kinase (MAPK) pathway mutation in the MAPK1 gene, which encodes mitogen-activated protein kinase 1 or extracellular signal-regulated 2 (ERK2). Our analysis revealed that the mutation results in constitutive ERK activation that is resistant to BRAF or MEK inhibitors but susceptible to an ERK inhibitor. These data highlight the importance of identifying specific MAPK pathway alterations as part of the diagnostic workup for patients with histiocytic disorders rather than initiating empiric treatment with MEK inhibitors...
April 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28504206/braf-v600e-mutation-in-pediatric-intracranial-and-cranial-juvenile-xanthogranuloma
#7
Piti Techavichit, Darintr Sosothikul, Thiamjit Chaichana, Chinachote Teerapakpinyo, Paul Scott Thorner, Shanop Shuangshoti
Juvenile xanthogranuloma (JXG) is a cutaneous form of non-Langerhans cell histiocytosis (LCH), primarily affecting children. The lesion is presumed to originate from either macrophages or dermal dendritic cells. JXG can rarely present as an isolated intracranial lesion and, in contrast to the dismal outcome of patients with systemic disease, cranial JXG has been shown to carry a more favorable prognosis. Here, we report for the first time 3 pediatric cases of JXG with a BRAF V600E mutation, 2 with intracranial lesions and one with cranial lesions...
May 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28500693/the-clinical-spectrum-of-xanthomatous-lesions-of-the-eyelids
#8
REVIEW
Can Baykal, Algun Polat Ekinci, Kurtulus D Yazganoglu, Nesimi Buyukbabani
Yellowish papules, nodules, or plaques, namely "xanthomatous" lesions, may be seen on the eyelids in the course of various disorders. The prototype is "xanthelasma palpebrarum" (XP) that is localized only to the eyelids and may be associated with hyperlipidemia. On the other hand, different types of normolipemic disorders may also cause xanthomatous eyelid lesions. Among these, Langerhans cell histiocytosis, diffuse normolipemic xanthoma, and non-Langerhans cell histiocytoses (papular xanthoma, juvenile xanthogranuloma, xanthoma disseminatum, adult-onset xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, and reticulohistiocytosis) can be listed...
May 13, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28403515/an-aggressive-multifocal-primary-cns-histiocytosis-with-ptpn11-shp2-mutation
#9
Qi Zhang, Areej Shibani, Bekim Sadikovic, Christopher J Howlett, Lee-Cyn Ang
Primary histiocytic tumors of the CNS are rare. The current WHO classification (2016) included 5 entities: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and histiocytic sarcoma (HS) (1). The diagnosis usually is made based on the tumor differentiation as to the counterpart in normal histiocyte development. This article is protected by copyright. All rights reserved.
April 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28398921/hemosiderotic-juvenile-xanthogranuloma
#10
Angel Fernandez-Flores, Ina Nicklaus, Fiona Browne, Isabel Colmenero
Juvenile xanthogranuloma is a non-Langerhans cell lesion mostly limited to the skin but occasionally presenting in extracutaneous locations or associated with systemic conditions. Lesions need to be distinguished mainly from dermatofibroma, xanthoma, Langerhans cell histiocytosis, or reticulohistiocytoma. Herein, we present a hemosiderotic variant of juvenile xanthogranuloma in a 12-year-old girl, which we have not found described in literature. The lesion presented at the back of the scalp as a slowly growing yellowish polypoid lesion showing occasional bleeding...
March 16, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28394239/management-of-uveitis-and-scleritis-in-necrobiotic-xanthogranuloma
#11
John A Gonzales, Anna Haemel, Andrew J Gross, Nisha R Acharya
Necrobiotic xanthogranuloma (NXG) is a chronic, progressive non-Langerhans histiocytic granulomatous disease. While case reports describing periorbital involvement of NXG are frequent, only a few case reports describing ocular involvement, such as scleritis and uveitis, exist. Herein, we present a case presenting initially as bilateral anterior and posterior scleritis, as well as a chronic bilateral granulomatous panuveitis, and discuss the immunosuppressant options that should be considered for this disease with protean manifestations...
May 2017: Journal of Ocular Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28372783/ultrasound-appearance-of-juvenile-xanthogranuloma
#12
C Martínez-Morán, B Echeverría-García, J C Tardío, J Borbujo
No abstract text is available yet for this article.
March 31, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28329596/coalescing-nodules-on-the-trunk-of-an-infant-juvenile-xanthogranuloma-en-plaque
#13
Jennifer T Hau, Kathy Langevin
Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytosis, which commonly manifests as a solitary papule or nodule in infants and young children. However, other less common clinical presentations have been reported including clustered, agminated or plaque-like. It is important to recognize these variants, as diagnosis is often clinical. We highlight a case of juvenile xanthogranuloma presenting as nodules on the trunk of an infant that gradually coalesced to form a larger plaque.
October 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28318197/-generalized-eruptive-histiocytosis-juvenile-xanthogranuloma-clinical-spectrum-in-a-pediatric-patient
#14
Ruth Alperovich, Pedro T Grassino, Raúl Asial, Luis Pasteris, María Del Carmen Boente
Both, generalized eruptive histiocytosis and juvenile xanthogranuloma are dendritic histiocytic disorders (also known as non-Langerhans cells histiocytosis) that share clinicopathological and immunohistiochemical characteristics. We present a 3-year-old female patient with skin lesions that were clinically compatible with generalized eruptive histiocytosis, confirmed by histopathological and immunohistochemical studies. During her development the disorder compromised the central nervous system, and surgical intervention of one symptomatic lesion was needed...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28318056/cutaneous-and-systemic-findings-in-mosaic-neurofibromatosis-type-1
#15
Igor Vázquez-Osorio, Anna Duat-Rodríguez, Francisco Javier García-Martínez, Antonio Torrelo, Lucero Noguera-Morel, Angela Hernández-Martín
BACKGROUND/OBJECTIVES: Mosaic neurofibromatosis type 1 (MNF1) is a variant of neurofibromatosis type 1 (NF1) in which clinical manifestations are limited to one or several body segments. The objective was to characterize the cutaneous features and associated systemic findings in a cohort of children with MNF1. METHODS: We performed a retrospective study of 40 children diagnosed with MNF1 at the Department of Dermatology, Hospital Infantil Niño Jesús, Madrid, Spain, from January 1, 1986, to October 31, 2015...
March 20, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28300446/a-rare-case-of-solitary-giant-congenital-juvenile-xanthogranuloma
#16
Catherine Vignault, Émilie Bourgeault, Éric Gagné, Janie Bujold
Juvenile xanthogranuloma (JXG) is the most frequent form of non-Langerhans cell histiocytosis. We present a case of giant congenital JXG in a 7-week-old boy, who had a firm and incompressible lesion, measuring 3 × 4 cm in diameter, on his right flank. The clinical appearance of the lesion and the ultrasound results suggested a vascular tumor, such as a hemangioma. Histology confirmed a JXG, although there was an absence of Touton cells, which are usually pathognomonic of JXG. In light of these findings, it would be important to include JXG in the differential diagnosis of congenital tumours, particularly vascular lesions...
February 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28297144/a-case-with-juvenile-xanthogranuloma-and-progressive-nodular-histiocytosis-overlap
#17
Lucia Guidolin, Lucero Noguera-Morel, Angela Hernández-Martín, Héctor Fernández-Llaca, José Luis Rodríguez-Peralto, Antonio Torrelo
A patient with overlap between juvenile xanthogranuloma and progressive nodular histiocytosis is presented. He had progressive, superficial, small, yellow-reddish papules and large dermal nodules, consistent with progressive nodular histiocytosis. Histopathology showed distinctive features of juvenile xanthogranuloma. Our patient reinforces the view that progressive nodular histiocytosis is not a distinct clinical entity, but an evolutionary form of juvenile xanthogranuloma.
March 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28295661/non-cirrhotic-portal-hypertension-in-necrobiotic-xanthogranuloma-associated-with-monoclonal-gammopathy
#18
LETTER
M Vignon, L Placais, M Malphettes, J D Bouaziz, B Asli, P Bedossa, J Rivet, R Szalat, A M Zagdanski, M Rybojad, J P Fermand, M Baron, P E Rautou, B Arnulf
No abstract text is available yet for this article.
March 10, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28286725/remarkable-diagnostic-magnetic-resonance-imaging-findings-in-sellar-xanthogranuloma-report-of-three-first-cases-in-latin-america
#19
Mario Tapia Céspedes, Jaime Pinto Vargas, Fernando Andrade Yañez, Loreto Spencer León, Pablo Álvarez Arancibia, Thomas Schmidt Putz
The sellar xanthogranuloma is a rare lesion of the sellar-parasellar region difficult to differentiate from other tumors such as craniopharyngiomas or Rathke's cleft cyst in the preoperative evaluation. As they are recently recognized as a separate entity and the few number of reports in the literature, its etiology is unknown and its impact remains uncertain. This article will describe the first three cases reported in Latin America, identified in one of them an imaging feature that may be helpful to elucidate an imaging growth pattern...
January 2017: Journal of Neurological Surgery Reports
https://www.readbyqxmd.com/read/28262109/adult-xanthogranulomatous-disease-of-the-orbit-clinical-presentations-evaluation-and-management
#20
J M Ortiz Salvador, D Subiabre Ferrer, A Pérez Ferriols
Adult xanthogranulomatous disease of the orbit refers to a heterogeneous group of clinical syndromes with differing degrees of systemic involvement and distinct prognoses. The different syndromes all present clinically with progressively enlarging, yellowish lesions of the orbit. Histologically, the lesions are characterized by an inflammatory infiltrate of foam cells and Touton-type multinucleated giant cells. The xanthomatized histiocytes are CD68(+), S100(-), and CD1a(-). There are 4 clinical forms of xanthogranulomatous disease of the orbit: adult xanthogranulomatous disease of the orbit, adult onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, and Erdheim-Chester disease...
June 2017: Actas Dermo-sifiliográficas
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