Juvenile xanthogranuloma - Juvenilni Ksantogranulom
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Juvenile xanthogranuloma (JXG) je dokaj pogosto stanje in najpogostejša vrsta non-Langerhans cell histiocytic disorder pri otrocih. V približno 75% primerov se te lezije pokažejo v prvem letu življenja, več kot 15-20% bolnikov pa jih ima od rojstva. Čeprav je pri odraslih redek, se JXG običajno najpogosteje pojavi pri ljudeh v poznih dvajsetih do tridesetih letih in večina odraslih bolnikov ima samo eno lezijo. Klinično se kaže kot posamezne ali več rumeno-oranžno-rjavih čvrstih izboklin ali zatrdlin, predvsem na obrazu, vratu in zgornjem delu telesa. Oralne lezije so redke, vendar se lahko pojavijo kot rumena bula na straneh jezika ali drugje v ustih, kar lahko povzroči razjede in krvavitve. Kožne lezije običajno ne povzročajo simptomov in ponavadi izginejo same po nekaj letih. Čeprav redko, je prizadetost očesa najpogostejša težava zunaj kože, sledi pa ji prizadetost pljuč. Ocular JXG običajno prizadene le eno oko in se pojavi pri manj kot 0. 5 % bolnikov, čeprav ima približno 40 % tistih z očesnimi poškodbami ob diagnozi tudi več kožnih lezij.
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) so občasne, benigne bolezni, ki so del večje kategorije non-Langerhans cell histiocytoses. Običajno se pokažejo kot ena ali več rdečih ali rumenkastih zatrdlin, ki jih pogosto najdemo na glavi ali vratu. Večina JXG se razvije ob rojstvu ali v prvem letu življenja. Čeprav je nenavadno, včasih lahko prizadenejo področja zunaj kože, pri čemer je glede na obstoječo literaturo treba biti pozoren na prizadetost oči. Na splošno JXG na koži izginejo sami in običajno ne potrebujejo zdravljenja.
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.
Očesna lezija se pojavi pri do 10 % ljudi z JXG in lahko vpliva na njihov vid. Čeprav kožne lezije običajno spontano izginejo, se očesne lezije redko spontano izboljšajo in zahtevajo zdravljenje.