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Juvenile Xanthogranuloma Associated with Neurofibromatosis 1: 14 Patients without Evidence of Hematologic Malignancies
Author(s) -
Cambiaghi Stefano,
Restano Lucia,
Caputo Ruggero
Publication year - 2004
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.0736-8046.2004.21201.x
Subject(s) - medicine , juvenile xanthogranuloma , neurofibromatosis , dermatology , natural history , pediatrics , juvenile , pathology , histiocyte , biology , genetics
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.