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KMID : 0363220190570060328
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Korean Journal of Dermatology
2019 Volume.57 No. 6 p.328 ~ p.331
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Bilateral Segmental Neurofibromatosis with Lentiginosis Showing a Checkerboard Pattern
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Park Seh-Hyun
Lee Si-Hyung
Kim Soo-Chan
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Abstract
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Segmental neurofibromatosis, a subtype of neurofibromatosis type 1, is characterized by neurofibromas and/or cafeau- lait spots limited to an area or segment of the body. Checkerboard pattern is a rare type of cutaneous mosaic manifestation, characterized by squares or broad ribbons of affected skin with sharp demarcation at the midline.
Herein, we report the case of a patient with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern. Our patient had multiple hyperpigmented macules on her entire body in a checkerboard pattern since birth. Several cafe-au-lait patches were observed on the left buttock and right axilla. A neurofibroma was incidentally found beneath the cafe-au-lait patch by histological examination, which showed ill-defined spindle cells with elongated nuclei at the deep dermis that stained positive for S-100. Based on the clinical presentation and histopathologic results, the patient was diagnosed with bilateral segmental neurofibromatosis with lentiginosis showing a checkerboard pattern.
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KEYWORD
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Body patterning, Lentiginosis, Neurofibromatosis
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