Juvenile xanthogranuloma - 幼年型黃色肉芽腫
relevance score : -100.0%
ReferencesJuvenile xanthogranuloma (JXG) 是相當常見的疾病，也是兒童中最常見的 non‑Langerhans cell histiocytic disorder 類型。約 75% 病例在出生後一年內出現，另有 15‑20% 患者自出生即有病變。雖然 JXG 在成人中較少見，但最常見於 20 至 30 歲的人群，且大多數成年患者僅有單一病變。臨床上表現為單個或多個黃橙棕色、堅硬的結節或腫塊，常見於臉部、頸部及上半身。口腔病變較少見，可能出現在舌側或口腔其他部位，形成黃色結節，並可能引發潰瘍或出血。皮膚病變通常無症狀，且多在數年內自行消退。雖然罕見，眼部受累是皮膚以外最常見的併發症，其次為肺部受累。Ocular JXG 通常僅累及單眼，發生率不到 0.5% 的患者，但約 40% 的眼部受累者在診斷時同時有多發性皮膚病變。
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) 是罕見的良性疾病，屬於更大類別的 non‑Langerhans cell histiocytoses。它們通常表現為一個或多個紅色或淡黃色的腫塊，常見於頭部或頸部。大多數 JXG 在出生時或出生後的第一年內出現。雖然較少見，但有時會累及皮膚以外的部位，文獻指出需特別留意眼部受累。一般而言，皮膚上的 JXG 會自行消退，通常不需要治療。
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.
約有 10% 的 JXG 患者會出現眼部病變，可能影響視力。雖然皮膚病變大多會自行消失，眼部病變則較少自行改善，需接受治療。