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ANALYTICAL REPORT
Year : 2012  |  Volume : 8  |  Issue : 2  |  Page : 311-313

Systemic juvenile xanthogranuloma with multiple central nervous system lesions


1 Neurosciences Research Center, Tabriz University of Medical Science, Tabriz; Department of Neurosurgery, Shohada Hospital, Shahid Beheshti Medical University, Tehran, Iran
2 Department of Neurosurgery, Shohada Hospital, Shahid Beheshti Medical University, Tehran, Iran
3 Department of Pathology, Shohada Hospital, Shahid Beheshti Medical University, Tehran, Iran
4 Department of Neurosurgery, Medical School, Hannover, Germany
5 Neurosciences Research Center, Tabriz University of Medical Science, Tabriz, Iran

Correspondence Address:
Amir Hamdi
Neurosciences Research Center, Tabriz University of Medical Sciences, Imam Reza Medical Center, Golgasht Street, Tabriz, Eastern Azerbaijan 5166614756
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.99001

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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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