Juvenile xanthogranuloma - Xanthogranuloma Remaja
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ReferencesJuvenile xanthogranuloma (JXG) adalah kondisi yang relatif umum dan merupakan jenis non‑Langerhans cell histiocytic disorder yang paling sering terjadi pada anak‑anak. Pada sekitar 75 % kasus, lesi ini muncul pada tahun pertama kehidupan, dan lebih dari 15‑20 % pasien mengalaminya sejak lahir. Meskipun jarang pada orang dewasa, JXG paling sering muncul pada individu berusia akhir dua puluhan hingga tiga puluhan, dan kebanyakan pasien dewasa hanya memiliki satu lesi. Secara klinis, lesi muncul sebagai nodul atau papul keras berwarna kuning‑oranye‑coklat, tunggal atau ganda, terutama pada wajah, leher, dan bagian atas tubuh. Lesi pada mulut jarang, namun dapat muncul sebagai nodul kuning di sisi lidah atau area lain di mulut, yang dapat menyebabkan bisul dan perdarahan. Lesi kulit biasanya tidak menimbulkan gejala dan cenderung hilang dengan sendirinya dalam beberapa tahun. Meskipun jarang, keterlibatan mata merupakan komplikasi paling umum selain manifestasi kulit, diikuti oleh keterlibatan paru‑paru. Ocular JXG biasanya hanya menyerang satu mata dan terjadi pada kurang dari 0,5 % pasien, meskipun sekitar 40 % pasien dengan kelainan mata juga memiliki lesi kulit multipel pada saat 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 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) jarang terjadi, merupakan penyakit jinak yang termasuk dalam kategori non‑Langerhans cell histiocytoses yang lebih luas. Biasanya muncul sebagai satu atau lebih benjolan berwarna merah atau kekuningan, sering ditemukan di kepala atau leher. Sebagian besar JXG muncul sejak lahir atau pada tahun pertama kehidupan. Meskipun jarang, penyakit ini dapat melibatkan organ di luar kulit; menurut literatur, keterlibatan mata perlu diwaspadai. Pada umumnya, JXG pada kulit akan menghilang dengan sendirinya dan tidak memerlukan pengobatan.
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.
Lesi mata terjadi pada 10 % penderita JXG dan dapat memengaruhi penglihatan. Meskipun lesi kulit biasanya menghilang secara spontan, lesi pada mata jarang membaik dengan sendirinya dan memerlukan pengobatan.