Juvenile xanthogranuloma - Juveniilne Ksantogranuloom
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References
Juvenile xanthogranuloma (JXG) on üsna levinud haigusseisund ja kõige sagedasem non-Langerhans cell histiocytic disorder tüüp lastel. Umbes 75% juhtudest ilmnevad need kahjustused esimesel eluaastal ja enam kui 15–20% patsientidest on need sünnist saati. Kuigi täiskasvanutel esineb JXG-d harva, esineb seda tavaliselt kahekümnendates kuni kolmekümnendates eluaastates inimestel ja enamikul täiskasvanud patsientidel on ainult üks kahjustus. Kliiniliselt ilmneb see üksikute või mitmete kollakasoranžikaspruunide kindlate punnidena või tükkidena, peamiselt näol, kaelal ja ülakehal. Suukahjustused on aeg-ajalt, kuid need võivad ilmneda kollase tükina keele külgedel või mujal suus, mis võib põhjustada haavandeid ja verejooksu. Nahakahjustused ei põhjusta tavaliselt sümptomeid ja kipuvad mitme aasta jooksul iseenesest kaduma. Kuigi see on haruldane, on silmade haaratus kõige levinum probleem väljaspool nahka, millele järgneb kopsukahjustus. Ocular JXG mõjutab tavaliselt ainult ühte silma ja esineb vähem kui 0. 5 % patsientidest, kuigi umbes 40% silmahaigusega patsientidest on diagnoosimisel ka mitu nahakahjustust.
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) on aeg-ajalt healoomulised haigused, mis kuuluvad suuremasse kategooriasse non-Langerhans cell histiocytoses. Tavaliselt ilmnevad need ühe või mitme punase või kollaka tükina, mida leidub sageli peas või kaelas. Enamik JXG-sid areneb kas sündides või esimesel eluaastal. Kuigi see on ebatavaline, võivad need mõnikord mõjutada nahast kaugemal asuvaid piirkondi, kusjuures olemasoleva kirjanduse kohaselt tuleb silmahaardega jälgida. Üldiselt kaovad nahal olevad JXG-d ise ega vaja tavaliselt ravi.
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.
Silmakahjustus ilmneb kuni 10% JXG-ga inimestel ja võib mõjutada nende nägemist. Kuigi nahakahjustused kaovad tavaliselt spontaanselt, paranevad silmakahjustused harva spontaanselt ja vajavad ravi.