Juvenile xanthogranuloma - Juvenilni Ksantogranulom
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ReferencesJuvenile xanthogranuloma (JXG) je prilično česta bolest i najčešći oblik non‑Langerhans cell histiocytic disorder kod djece. U oko 75 % slučajeva lezija se pojavi unutar prve godine života, a kod više od 15‑20 % pacijenata prisutna je od rođenja. Iako je rijedak kod odraslih, JXG se najčešće javlja kod osoba u kasnim dvadesetim do tridesetim godinama, a većina odraslih pacijenata ima samo jednu leziju. Klinički se manifestira kao pojedinačne ili višestruke žuto‑narančasto‑smeđe čvrste kvržice, uglavnom na licu, vratu i gornjem dijelu tijela. Oralne lezije nisu uobičajene, ali se mogu pojaviti kao žuta kvržica na stranama jezika ili drugdje u usnoj šupljini, što može dovesti do čira i krvarenja. Kožne promjene obično ne uzrokuju simptome i najčešće nestaju same od sebe tijekom nekoliko godina. Iako rijetko, zahvaćenost oka je najčešći izvankožni problem, a slijedi zahvaćenost pluća. Ocular JXG obično zahvaća samo jedno oko i javlja se u manje od 0,5 % pacijenata, iako oko 40 % onih s oštećenjem oka također ima više kožnih lezija u trenutku dijagnoze.
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) su neuobičajene, benigne bolesti koje pripadaju širem spektru non‑Langerhans cell histiocytoses. Obično se pojavljuju kao jedna ili više crvenih ili žućkastih kvržica, najčešće na glavi ili vratu. Većina JXG‑a razvija se pri rođenju ili u prvoj godini života. Iako je to rijetko, ponekad mogu zahvatiti i druge organe izvan kože; prema dostupnoj literaturi treba posebno obratiti pažnju na moguće zahvaćenje očiju. Općenito, JXG‑i na koži spontano nestaju i obično ne zahtijevaju liječenje.
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.
Očna lezija pojavljuje se kod do 10 % osoba s JXG‑om i može utjecati na njihov vid. Iako kožne lezije obično nestaju spontano, očne lezije rijetko se spontano poboljšaju i zahtijevaju liječenje.