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Pachydermoperiostosis: extensive cutis verticis gyrata may be a significant diagnostic clue
( Jisang Yun ),( Kyung-nam Bae ),( Jin-hwa Son ),( Kihyuk Shin ),( Hyun-chang Ko ),( Byungsoo Kim ),( Moon-bum Kim ),( Hoon-soo Kim ) 대한피부과학회 2021 대한피부과학회 학술발표대회집 Vol.72 No.2
Pachydermoperiostosis (PDP) is a rare hereditary disease diagnosed by the presence of digital clubbing, periostosis, and pachydermia, including cutis verticis gyrata (CVG). PDP is categorized into three forms: the complete form involving all three major symptoms; the incomplete form, without pachydermia; and the forme fruste form, without periostosis. Herein, we present a case of the complete form of PDP with extensive CVG. A 22-year-old man presented to our clinic with the deepened forehead and scalp skin creases, which was extensive CVG for two years. He had folliculitis with seborrhea on the face. In addition, the clubbing of fingers and toes was presented along with the broadening of hands and feet. Since he had grown rapidly in adulthood, gigantism or acromegaly was initially suspected. However, laboratory results, including growth hormone and insulin-like growth factor 1, were within normal limits. Only testosterone level was slightly lower than the normal value. Through chromosome analysis, Klinefelter syndrome was also excluded. Radiologic examinations revealed periostosis on both hands. Based on pachydermia, digital clubbing, and periosteal reaction, the complete form of PDP was diagnosed. To our knowledge, this is a rare case of the complete form of PDP in Korean dermatologic literature. Through our case, dermatologists need to know that extensive CVG involving face could be considered as a significant diagnostic clue of PDP.