Juvenile xanthogranuloma - 幼年型黄色肉芽肿
relevance score : -100.0%
ReferencesJuvenile 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% 的病例在出生后一年内出现，超过 15‑20% 的患者从出生起就有病变。虽然 JXG 在成人中很少见，但通常最常见于二十至三十岁的人群，并且大多数成年患者仅有单个病变。临床表现为单个或多个黄橙棕色坚硬的结节或肿块，常见于面部、颈部和上半身。口腔病变并不常见，但可在舌侧或口腔其他部位出现黄色结节，可能导致溃疡和出血。皮肤病变通常无症状，且往往在数年内自行消退。尽管罕见，眼部受累是皮肤外最常见的并发症，其次是肺部受累。Ocular JXG 通常仅累及单眼，发生率不足 0.5%，但约 40% 的眼部受累患者在诊断时同时存在多个皮肤病变。
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) 是一种不常见的良性疾病，属于更大类的 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的结节，常见于头部或颈部。大多数 JXG 在出生时或出生后第一年内出现。虽然较为少见，但有时它们会累及皮肤以外的部位，文献提示需关注眼部受累。总体而言，皮肤上的 JXG 多数会自行消退，通常无需治疗。
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.
约 10% 的 JXG 患者会出现眼部病变，可能影响视力。虽然皮肤病变大多自行消失，但眼部病变很少自行改善，往往需要治疗。