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Pediatr Dermatol. 2004 Mar-Apr;21(2):97-101.

Juvenile xanthogranuloma associated with neurofibromatosis 1: 14 patients without evidence of hematologic malignancies.

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1
Istituto di Scienze Dermatologiche, IRCCS, Ospedale Maggiore e UniversitĂ  di Milano, Via Pace 9, 20122 Milan, Italy. stefanodoc@tiscalinet.it

Abstract

The clinical features and natural history of juvenile xanthogranuloma (JXG) in 14 children affected by neurofibromatosis 1 (NF1) are reported. Mean follow-up in 11 of these patients was 4.3 years (range 1-10 years). None of the children developed hematologic malignancies during this period. The onset of JXG was in the first 2 years of life in 13 of the patients. In this series, the association between JXG and six or more café au lait spots more than 5 mm in diameter was a good marker for NF1 in the first few years of life. Overall the JXG in these patients did not show any features distinguishable from those of "classical" JXG.

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