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KMID : 0363220090470010089
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Korean Journal of Dermatology
2009 Volume.47 No. 1 p.89 ~ p.94
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A Case of Hemifacial Hypertrophy due to Plexiform Neurofibroma with the Cafe-au-Lait Macules
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Do Jeong-Eun
Kang Hee-Young
Lee Eun-So
Kim You-Chan
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Abstract
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Plexiform neurofibromas are among the most pathognomonic features of neurofibromatosis type 1 (NF1). Although
characteristically benign, plexiform neurofibroma can cause pain, disfigurement and functional changes, and more
importantly, it may turn malignant. We present here the case of a 3-year-old boy with progressive hemifacial
hypertrophy that was colocalized with light brown-colored macules. The biopsy specimen showed that the bundles
of nerve fibers were arranged in a concentric manner with myxoid changes. On the evaluation of the pigmented
lesion, only the basal melanin pigments were increased in the epidermis without any melanin-laden pigmented cells
in the dermis. We have demonstrated that neurofbroma needs to be considered in the differential diagnosis of
hemifacial hypertrophy since this is likely to have implications for the further management of these patients. The
absence of other findings does not exclude NF1 as the underlying disease since many NF1 patients have not yet
developed cafe-au-lait macules or freckles in early childhood.
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KEYWORD
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Cafe-au-Lait macule, Hemifacial hypertrophy, Plexiform neurofibroma
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