Systemic juvenile xanthogranuloma with multiple central nervous system lesions |
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Authors: | Ali Meshkini Sohrab Shahzadi Alireza Zali Aram Tajeddini Javad Mirzayan Amir Hamdi |
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Affiliation: | Neurosciences Research Center, Tabriz University of Medical Science, Tabriz, Iran. |
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Abstract: | Juvenile xanthogranulomatosis (JXG) is an uncommon histiocytic disorder that is usually benign and limited to the skin. The systemic form of JXG is rare and may be associated with severe morbidity and mortality especially in central nervous system (CNS) involvement. Here, we describe a six-year-old boy with disseminated skin lesions and neurological signs and symptoms. Diagnostic work up revealed multiple brain lesions. A skin biopsy and a stereotactic brain biopsy considered suggestive of systemic JXG. Treatment with prednisolone, vinblastine and methotrexate was successful with regression of skin and CNS lesions. The patient has been in remission for almost three years. |
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