Juvenile xanthogranuloma associated with neurofibromatosis 1: 14 patients without evidence of hematologic malignancies.
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.
Cambiaghi
Stefano
S
Istituto di Scienze Dermatologiche, IRCCS, Ospedale Maggiore e Università di Milano, Via Pace 9, 20122 Milan, Italy. stefanodoc@tiscalinet.it
Restano
Lucia
L
Caputo
Ruggero
R
D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007938:Leukemia / Q000175:diagnosis / Q000209:etiology; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis*; D010859:Pigmentation Disorders / Q000209:etiology; D012075:Remission, Spontaneous; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis* / Q000209:etiology*