Neonatal Systemic Juvenile Xanthogranuloma with an Ominous Presentation and Successful Treatment
نویسندگان
چکیده
This case report originated from a case of neonatal multisystemic juvenile xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis. Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.
منابع مشابه
Juvenile Xanthogranuloma Presenting as Bilateral Non-Infiltrative Extraconal Superior Orbital Tumour in a 27 Year Old Nigerian Woman: Features, Management and Outcome
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Juvenile xanthogranuloma (JXG) is an uncommon histiocytic cutaneous lesion. It is a type of non-Langerhans cell histiocytosis(WHO Class IIb). The mean age of onset is 2 years of age. The adult form of JXG is relatively rare. The most common affectedarea is the face or the scalp and most of the lesions are less than 5 mm in diameter. This lesion tends to show a self-limited course over several m...
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