Juvenile xanthogranuloma - 소아황색육아종
References
Juvenile xanthogranuloma (JXG) 은 상당히 흔한 질환이며 어린이에게 가장 흔한 유형의 non-Langerhans cell histiocytic disorder 입니다. 약 75%의 사례에서 이러한 병변은 생후 1년 이내에 나타나며, 15-20% 이상의 환자는 출생 시부터 병변을 가지고 있습니다. 성인에서는 드물지만 JXG는 일반적으로 20대 후반에서 30대 사이에 가장 자주 발생하며 대부분의 성인 환자에게는 병변이 하나만 있습니다. 임상적으로는 주로 얼굴, 목, 상체에 단일 또는 여러 개의 노란색-주황색-갈색의 단단한 돌기 또는 덩어리로 나타납니다. 구강 병변은 흔하지 않지만, 혀 옆이나 입 안의 다른 곳에 노란색 덩어리로 나타날 수 있으며, 이로 인해 궤양과 출혈이 발생할 수 있습니다. 피부 병변은 일반적으로 증상을 일으키지 않으며 몇 년에 걸쳐 저절로 사라지는 경향이 있습니다. 드물기는 하지만 안구 침범은 피부 이외의 가장 흔한 문제이고 폐 침범이 그 뒤를 따릅니다. Ocular JXG 은 일반적으로 한쪽 눈에만 영향을 미치며 환자의 0. 5 % 미만에서 발생하지만 안구 침범이 있는 환자의 약 40%는 진단 시 여러 피부 병변을 나타냅니다.
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) 은 non-Langerhans cell histiocytoses 의 더 큰 범주에 속하는 흔하지 않은 양성 질병입니다. 일반적으로 하나 이상의 빨간색 또는 노란색 덩어리로 나타나며 종종 머리나 목에서 발견됩니다. 대부분의 JXG는 출생 시 또는 생후 1년 이내에 발달합니다. 드문 일이지만 때로는 피부 너머의 영역에도 영향을 미칠 수 있으며, 기존 문헌에 따르면 눈의 침범을 주의 깊게 관찰해야 합니다. 일반적으로 피부의 JXG는 저절로 사라지며 일반적으로 치료가 필요하지 않습니다.
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.
안구 병변은 JXG 환자의 최대 10%에서 나타나며 시력에 영향을 줄 수 있습니다. 피부병변은 대개 저절로 사라지지만, 안구병변은 저절로 호전되는 경우가 거의 없어 치료가 필요합니다.