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A Case of Hemifacial Hypertrophy due to Plexiform Neurofibroma with the Cafe-au-Lait Macules

Other Title
밀크커피색 반점이 동반된 얼기형 신경섬유종으로 인한 안면 편측비대 1예
Authors
도, 정은; 강, 희영; 이, 은소; 김, 유찬
Citation
Korean journal of dermatology, 47(1):89-94, 2009
Journal Title
Korean journal of dermatology; 대한피부과학회지
ISSN
0494-4739
Abstract
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.
Keywords
Cafe-au-Lait maculeHemifacial hypertrophyPlexiform neurofibroma
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Dermatology
AJOU Authors
강, 희영이, 은소김, 유찬
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