Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1 |
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Authors: | Yuko Ehara Monji Koga Shinichi Imafuku Osamu Yamamoto Yuichi Yoshida |
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Affiliation: | 1. Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago, Japan;2. Department of Dermatology, Fukuoka University School of Medicine, Fukuoka, Japan |
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Abstract: | Neurofibromatosis 1 (NF1) is a genetic disease characterized by cutaneous, neurological and osseous abnormalities. Approximately 20% of patients develop plexiform neurofibroma (PN), resulting in impaired quality of life. To evaluate distribution of diffuse PN on the body surface, a retrospective study was conducted for 354 patients with NF1 from 2007 to 2018 in Japan. We investigated a total of 40 patients with clinically apparent superficial diffuse PN. In the cases evaluated, 57.4% of the diffuse PN were located on the trunk, 19.2% the head and neck, 12.8% the lower limbs and 10.6% the upper limbs. Remarkably, 75.0% of the diffuse PN were located on the dorsal side. The frequency was significantly higher on the trunk than on the head and neck (P = 0.026). Our findings provide useful information for giving attention to the high possibility of diffuse PN on the dorsal side before progression in childhood and for future treatment in NF1. |
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Keywords: | distribution neurofibromatosis 1 plexiform neurofibroma predilection body site selumetinib |
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