Juvenile xanthogranuloma - Xanthogranuloma Tal-Minorenni
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ReferencesJuvenile xanthogranuloma (JXG) hija kundizzjoni pjuttost komuni u l-aktar tip frekwenti ta’ disordri istjokitiċi mhux Langerhans (non-Langerhans cell histiocytic disorder) fit-tfal. F’ madwar 75 % tal-każijiet, dawn il-lesjonijiet jidhru fl-ewwel sena tal-ħajja, u aktar minn 15‑20 % tal-pazjenti jkollhomhom mit-twelid. Filwaqt li rari fl‑adulti, JXG tipikament iseħħ l-aktar spiss f’ nies fl-aħħar ta’ għoxrin sa tletinijiet, u l-biċċa l-kbira tal-pazjenti adulti għandhom lesjoni waħda biss. Klinikament, jidher bħala ħotob sodi waħdieni jew multipli isfar‑oranġjo‑canella jew ċapep, prinċipalment fuq il-wiċċ, l-għonq u l-parti ta’ fuq tal-ġisem. Lesjonijiet orali mhumiex komuni iżda jistgħu jidhru bħala biċċa safra fuq il-ġnub tal-ilsien jew xi mkien ieħor fil-ħalq, li possibbilment twassal għal ulċeri u fsada. Il-lesjonijiet tal-ġilda normalment ma jikkawżawx sintomi u għandhom it‑tendenza li jmorru waħedhom fuq diversi snin. Għalkemm rari, l‑involviment okulari huwa l‑aktar kwistjoni komuni lil hinn mill‑ġilda, segwit minn involviment tal‑pulmuni. Ocular JXG tipikament taffettwa għajn waħda biss u jseħħ f’inqas minn 0.5 % tal-pazjenti, għalkemm madwar 40 % ta’ dawk b’involviment okulari għandhom ukoll lesjonijiet multipli fil-ġilda meta jiġu dijanjostikati.
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) huma mard mhux komuni, beninni li huwa parti minn kategorija akbar ta' non-Langerhans cell histiocytoses. Tipikament jidhru bħala ċapep aħmar jew safrani wieħed jew aktar, ħafna drabi jinstabu fuq ir-ras jew l-għonq. Ħafna mill-JXGs jiżviluppaw jew mat-twelid jew fl-ewwel sena tal-ħajja. Filwaqt li mhux tas-soltu, xi drabi jistgħu jaffettwaw żoni lil hinn mill-ġilda, bl-involviment tal-għajnejn ikun xi ħaġa li għandek toqgħod attent għaliha skont il-letteratura eżistenti. Ġeneralment, JXGs fuq il-ġilda jmorru waħedhom u tipikament m'għandhomx bżonn trattament.
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.
Il‑leżjonijiet okulari jinstabu f’10 % tan‑nies b’JXG u jistgħu jaffettwaw il‑viżjoni tagħhom. Għalkemm il‑leżjonijiet tal‑ġilda normalment jisparixxu spontanjament, il‑leżjonijiet okulari rari ma jisparixxux spontanjament u jeħtieġu trattament.