Juvenile xanthogranuloma - 幼年型黄色肉芽肿
References
Juvenile xanthogranuloma (JXG) 是一种相当常见的情况，也是儿童中最常见的 non-Langerhans cell histiocytic disorder 类型。大约 75% 的病例中，这些病变会在出生后一年内出现，超过 15-20% 的患者从出生起就有这些病变。虽然 JXG 在成人中很少见，但通常最常发生在二十岁到三十岁的人群中，并且大多数成年患者只有一个病变。临床上表现为单个或多个黄橙棕色坚硬肿块或肿块，好发于面部、颈部和上半身。口腔病变并不常见，但可能在舌头两侧或口腔其他部位出现黄色肿块，可能导致溃疡和出血。皮肤损伤通常不会引起症状，并且往往会在几年内自行消失。尽管罕见，但眼部受累是皮肤以外最常见的问题，其次是肺部受累。 Ocular JXG 通常只影响一只眼睛，发生率不到 0. 5 % 的患者，但约 40% 的眼部受累患者在诊断时也有多个皮肤病变。
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) 是不常见的良性疾病，属于更大类别的 non-Langerhans cell histiocytoses 的一部分。它们通常表现为一个或多个红色或淡黄色肿块，通常出现在头部或颈部。大多数 JXG 在出生时或出生后第一年内发育。虽然这很不寻常，但有时它们会影响皮肤以外的区域，根据现有文献，需要注意眼睛受累。一般来说，皮肤上的 JXG 会自行消失，通常不需要治疗。
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.
多达 10% 的 JXG 患者会出现眼部病变，并可能影响他们的视力。虽然皮肤病变通常会自行消失，但眼部病变很少会自行改善并需要治疗。