Juvenile xanthogranuloma
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References
Juvenile xanthogranuloma (JXG) ass eng zimlech heefeg Konditioun an déi heefegst Aart vu non-Langerhans cell histiocytic disorder bei Kanner. An ongeféier 75% vun de Fäll weisen dës Läsionen am éischte Joer vum Liewen op, an iwwer 15-20% vun de Patienten hunn se vun der Gebuert. Wärend seelen bei Erwuessener, geschitt JXG typesch am meeschte bei Leit an de spéiden zwanzeger bis drëssegjärege, an déi meescht erwuesse Patienten hunn nëmmen eng Läsion. Klinesch erschéngt et als eenzel oder multiple giel-orange-brong fest Knollen oder Klumpen, haaptsächlech am Gesiicht, Hals an Uewerkierper. Oral Läsionen sinn ongewéinlech awer kënnen als giel Klump op de Säiten vun der Zong oder soss anzwousch am Mond optrieden, méiglecherweis zu Geschwüren a Blutungen. Hautläsiounen verursaachen normalerweis keng Symptomer an tendéieren eleng iwwer e puer Joer ze goen. Obwuel seelen ass, ass d'Okulär Bedeelegung dat heefegst Thema iwwer d'Haut, gefollegt vun der Lungebedeelegung. Ocular JXG beaflosst typesch nëmmen een Aen a geschitt a manner wéi 0. 5 % vun de Patienten, obwuel ongeféier 40% vun deenen mat Okulären Engagement och méi Hautläsiounen hunn wann se diagnostizéiert ginn.
Juvenile xanthogranuloma (JXG) is a relatively common entity and is the most common form of non-Langerhans cell histiocytic disorder of childhood., It is estimated that in 75% of cases, lesions appear during the first year of life, with >15-20% of patients having lesions at birth. JXG is rare in adults, with a peak incidence in the late twenties to thirties. The majority of adult patients have solitary lesions. Typically, the clinical presentation consists of solitary or multiple yellow-orange-brown firm papules or nodules. The most common locations are the face, neck, and upper torso. Oral lesions are rare and often occur as a yellow nodule on the lateral aspects of the tongue. Oral lesions can also arise on the gingival, buccal mucosa, and midline hard palate and may ulcerate and bleed. Cutaneous lesions are usually asymptomatic, and most lesions spontaneously involute over the course of several years. Although occurring rarely, ocular involvement is the most common extracutaneous site involved, followed by the lungs. Ocular JXG is nearly always unilateral and develops in less than 0.5% of patients. Approximately 40% of patients with ocular JXG, however, have multiple cutaneous lesions at the time of diagnosis.
Juvenile xanthogranuloma (JXG) is a relatively common entity and is the most common form of non-Langerhans cell histiocytic disorder of childhood., It is estimated that in 75% of cases, lesions appear during the first year of life, with >15-20% of patients having lesions at birth. JXG is rare in adults, with a peak incidence in the late twenties to thirties. The majority of adult patients have solitary lesions. Typically, the clinical presentation consists of solitary or multiple yellow-orange-brown firm papules or nodules. The most common locations are the face, neck, and upper torso. Oral lesions are rare and often occur as a yellow nodule on the lateral aspects of the tongue. Oral lesions can also arise on the gingival, buccal mucosa, and midline hard palate and may ulcerate and bleed. Cutaneous lesions are usually asymptomatic, and most lesions spontaneously involute over the course of several years. Although occurring rarely, ocular involvement is the most common extracutaneous site involved, followed by the lungs. Ocular JXG is nearly always unilateral and develops in less than 0.5% of patients. Approximately 40% of patients with ocular JXG, however, have multiple cutaneous lesions at the time of diagnosis.
Juvenile xanthogranulomas (JXGs) sinn ongewéinlech, benigne Krankheeten déi Deel vun enger méi grousser Kategorie vun non-Langerhans cell histiocytoses sinn. Si weisen normalerweis als een oder méi rout oder gielzeg Klumpen op, dacks um Kapp oder Hals fonnt. Déi meescht JXGs entwéckelen entweder bei der Gebuert oder am éischte Joer vum Liewen. Och wann et ongewéinlech ass, kënnen se heiansdo Gebidder iwwer d'Haut beaflossen, mat Aenbedeelegung ass eppes fir no der existéierender Literatur ze kucken. Allgemeng ginn JXGs op der Haut eleng ewech a brauche normalerweis keng Behandlung.
Juvenile xanthogranulomas (JXGs) are uncommon, benign diseases that are part of a larger category of non-Langerhans cell histiocytoses. They typically show up as one or more red or yellowish lumps, often found on the head or neck. Most JXGs develop either at birth or within the first year of life. While it's unusual, sometimes they can affect areas beyond the skin, with eye involvement being something to watch for according to existing literature. Generally, JXGs on the skin go away on their own and typically don't need treatment.
Juvenile xanthogranulomas (JXGs) are uncommon, benign diseases that are part of a larger category of non-Langerhans cell histiocytoses. They typically show up as one or more red or yellowish lumps, often found on the head or neck. Most JXGs develop either at birth or within the first year of life. While it's unusual, sometimes they can affect areas beyond the skin, with eye involvement being something to watch for according to existing literature. Generally, JXGs on the skin go away on their own and typically don't need treatment.
Okulär Läsion manifestéiert a bis zu 10% vu Leit mat JXG a kann hir Visioun beaflossen. Och wann Hautläsiounen normalerweis spontan verschwannen, verbesseren d'Okulär Läsionen selten spontan a brauche Behandlung.