Juvenile xanthogranuloma - 若年性黄色肉芽腫症
References
Juvenile xanthogranuloma (JXG) はかなり一般的な症状であり、小児で最も頻繁に見られるタイプの non-Langerhans cell histiocytic disorder です。症例の約 75% で、これらの病変は生後 1 年以内に現れ、患者の 15 ～ 20% 以上は出生時から病変を持っています。成人ではまれですが、JXG は通常 20 代後半から 30 代の人に最も頻繁に発生し、ほとんどの成人患者には病変が 1 つだけあります。臨床的には、主に顔、首、上半身に、単一または複数の黄、オレンジ、茶色の硬い隆起またはしこりとして現れます。口腔病変はまれですが、舌の側面または口内の他の場所に黄色のしこりとして現れることがあり、潰瘍や出血を引き起こす可能性があります。皮膚病変は通常、症状を引き起こさず、数年かけて自然に消える傾向があります。まれではありますが、皮膚以外で最も一般的な問題は眼の病変であり、次に肺の病変が続きます。 Ocular JXG は通常、片方の目にのみ影響し、患者の 0. 5 % 未満で発生しますが、眼病変のある患者の約 40% には診断時に複数の皮膚病変も見られます。
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) はまれな良性疾患であり、 non-Langerhans cell histiocytoses という大きなカテゴリの一部です。通常、1 つ以上の赤または黄色がかったしこりとして現れ、頭や首によく見られます。ほとんどの JXG は、出生時または生後 1 年以内に発症します。珍しいことではありますが、皮膚を超えた領域に影響を与える場合があり、既存の文献によれば、眼への影響には注意が必要です。一般に、皮膚の JXG は自然に消失するため、通常は治療の必要はありません。
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.
眼病変は JXG 患者の最大 10% に現れ、視力に影響を与える可能性があります。皮膚病変は通常自然に消失しますが、眼病変が自然に改善することはほとんどなく、治療が必要です。