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KMID : 0363219950330061179
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Korean Journal of Dermatology
1995 Volume.33 No. 6 p.1179 ~ p.1183
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A Case of Von Recklinghausen's Disease with Plexiform Neurofibroma, Giant Pigmentation, and Skeletal Abnormalities
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황상민
안성구/이범주/이원수/최응호
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Abstract
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Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual
nerve
facicles, without an increased number of nerve fibers. We experienced a case of Von Recklinghausen's disease in a 24-year-old male who had variable cutaneous, skeletal, and CNS lesions. He presented multiple neurofibromas, caf*-au-lait spots, and
axillary frecklis as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the
body of
the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation. (Kor J Dermatol 1995;33(6) : 1179~1183)
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