Juvenile xanthogranuloma - 幼年型黃色肉芽腫
References
Juvenile xanthogranuloma (JXG) 是一種相當常見的情況，也是兒童中最常見的 non-Langerhans cell histiocytic disorder 類型。大約 75% 的病例中，這些病變會在出生後一年內出現，超過 15-20% 的患者從出生起就有這些病變。雖然 JXG 在成年人中很少見，但通常最常發生在二十歲到三十歲的人群中，大多數成年患者只有一個病變。臨床上表現為單一或多個黃橙棕色堅硬腫塊或腫塊，好發於臉部、頸部和上半身。口腔病變並不常見，但可能在舌頭兩側或口腔其他部位出現黃色腫塊，可能導致潰瘍和出血。皮膚損傷通常不會引起症狀，並且往往在幾年內自行消失。儘管罕見，但眼部受累是皮膚以外最常見的問題，其次是肺部受累。 Ocular JXG 通常只影響一隻眼睛，發生率不到 0. 5 % 的患者，但約 40% 的眼部受累患者在診斷時也有多個皮膚病變。
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) 是不常見的良性疾病，屬於更大類別的 non-Langerhans cell histiocytoses 的一部分。它們通常表現為一個或多個紅色或淡黃色腫塊，通常出現在頭部或頸部。大多數 JXG 在出生時或出生後第一年內發育。雖然這很不尋常，但有時它們會影響皮膚以外的區域，根據現有文獻，需要注意眼睛受累。一般來說，皮膚上的 JXG 會自行消失，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.
多達 10% 的 JXG 患者會出現眼部病變，並可能影響他們的視力。雖然皮膚病變通常會自行消失，但眼部病變很少會自行改善並需要治療。