Juvenile Xanthogranuloma Associated with Neurofibromatosis 1: 14 Patients without Evidence of Hematologic Malignancies

Pediatric Dermatology - Tập 21 Số 2 - Trang 97-101 - 2004
Stefano Cambiaghi1, Lucia Restano1, R. Caputo1
1Istituto di Scienze Dermatologiche, IRCCS, Ospedale Maggiore e Università di Milano, Milan, Italy

Tóm tắt

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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