Juvenile xanthogranuloma with multiple lesions in central nervous system: A rare case report

Juvenile xanthogranuloma (JXG) preferentially occurs in childhood and is usually benign and limited to the skin. The systemic form is rare and may be associated with severe morbidity and mortality. We describe a three and a half year old boy with disseminated papular skin lesions and neurological si...

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Bibliographic Details
Published in:Indian journal of paediatric dermatology 2016-01, Vol.17 (1), p.71-74
Main Authors: Jain, Priyanka, Chandravathi, P, Siddaiahgari, Sirisha, Tourani, Vijaya
Format: Article
Language:English
Subjects:
Care and treatment
Case studies
Central nervous system
Chemotherapy
Diagnosis
Health aspects
juvenile xanthogranuloma
Nervous system cancer
papular
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Description
Summary:Juvenile xanthogranuloma (JXG) preferentially occurs in childhood and is usually benign and limited to the skin. The systemic form is rare and may be associated with severe morbidity and mortality. We describe a three and a half year old boy with disseminated papular skin lesions and neurological signs and symptoms. Diagnostic workup revealed multiple brain lesions. Skin and brain biopsy was suggestive of systemic JXG. Treatment with prednisolone, vinblastine, and methotrexate caused regression of skin and central nervous system (CNS) lesions. However, 6 months after completion of chemotherapy, cutaneous but not CNS lesions relapsed. Few case reports have been published in the past, particularly with multiple lesions in CNS, which as in our case, is an extremely rare finding.
ISSN:2319-7250
2319-7269
DOI:10.4103/2319-7250.172466