Juvenile xanthogranuloma - Juvenilný Xantogranulóm
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Juvenile xanthogranuloma (JXG) je pomerne bežný stav a najčastejší typ non-Langerhans cell histiocytic disorder u detí. Asi v 75 % prípadov sa tieto lézie prejavia v priebehu prvého roku života a viac ako 15 – 20 % pacientov ich má od narodenia. Zatiaľ čo je u dospelých zriedkavý, JXG sa zvyčajne vyskytuje najčastejšie u ľudí vo veku od 20 do 30 rokov a väčšina dospelých pacientov má len jednu léziu. Klinicky sa prejavuje ako jednotlivé alebo viacnásobné žlto-oranžovo-hnedé pevné hrbolčeky alebo hrčky, hlavne na tvári, krku a hornej časti tela. Ústne lézie sú menej časté, ale môžu sa objaviť ako žltá hrčka po stranách jazyka alebo inde v ústach, čo môže viesť k vredom a krvácaniu. Kožné lézie zvyčajne nespôsobujú príznaky a majú tendenciu zmiznúť samy počas niekoľkých rokov. Hoci je to zriedkavé, očné postihnutie je najčastejším problémom mimo kože, po ktorom nasleduje postihnutie pľúc. Ocular JXG zvyčajne postihuje iba jedno oko a vyskytuje sa u menej ako 0. 5 % pacientov, hoci približne 40 % pacientov s očným postihnutím má pri diagnostikovaní aj viaceré kožné lézie.
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) sú menej časté, benígne ochorenia, ktoré sú súčasťou väčšej kategórie non-Langerhans cell histiocytoses. Zvyčajne sa prejavujú ako jedna alebo viac červených alebo žltkastých hrudiek, ktoré sa často nachádzajú na hlave alebo krku. Väčšina JXG sa vyvíja buď pri narodení alebo v prvom roku života. Aj keď je to nezvyčajné, niekedy môžu postihnúť oblasti za kožou, pričom podľa existujúcej literatúry je potrebné sledovať postihnutie očí. Vo všeobecnosti JXG na koži odchádzajú samé a zvyčajne nevyžadujú liečbu.
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.
Očná lézia sa prejavuje až u 10 % ľudí s JXG a môže ovplyvniť ich zrak. Hoci kožné lézie zvyčajne spontánne vymiznú, očné lézie sa spontánne zlepšia a vyžadujú si liečbu.