±¸°Á¡¸·ÀÇ Pachyonychia Congenita
Pachyonychia congenita of the oral mucosa
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½É¿µÁÖ ( Shim Young-Joo ) - ¿ø±¤´ëÇб³ Ä¡°ú´ëÇÐ ±¸°³»°úÇб³½Ç
À±Á¤ÈÆ ( Yoon Jung-Hoon ) - ¿ø±¤´ëÇб³ Ä¡°ú´ëÇÐ ´ëÀüÄ¡°úº´¿ø ±¸°º´¸®°ú
°Áø±Ô ( Kang Jin-Kyu ) - ¿ø±¤´ëÇб³ Ä¡°ú´ëÇÐ ±¸°³»°úÇб³½Ç
KMID : 0355220130380020103
Abstract
Pachyonychia congenita´Â Äɶóƾ À¯ÀüÀÚÀÇ µ¹¿¬º¯ÀÌ·Î ¹ß»ýÇϸç, »ó¿°»öü ¿ì¼ºÀ¸·Î À¯ÀüµÇ´Â ¸Å¿ì µå¹® À¯Àü¼º ÇǺÎÁúȯÀÌ´Ù. ÁÖ·Î ¼ÕÅé°ú ¹ßÅé, ¹ß¹Ù´Ú, ±¸°Á¡¸· µî¿¡ ÀÌȯµÇ¸ç, ÁÖµÈ Áõ»óÀ¸·Î´Â ¼Õ¹ßÅ鱸¸¸Áõ, ¼Õ°ú ¹ßÀÇ °ú°¢ÈÁõ, ¼ÒÆ÷¼º°¢ÈÁõ, ±¸°Á¡¸·ÀÇ ¹é»ö°¢ÈÁõ µîÀÌ ÀÖ´Ù. ±¸°Á¡¸·ÀÇ Á¶Á÷ÇÐÀû ¼Ò°ßÀ¸·Î´Â °ú°¢ÈÁõ, ±Ø¼¼Æ÷Áõ, »óÇǼ¼Æ÷ÀÇ ÇÙ ÁÖÀ§ ¼¼Æ÷Áú³» °øÆ÷ Çü¼ºÀÌ ³ªÅ¸³´Ù. ÇǺκ´¼ÒÀÇ Ä¡·á¹ýÀ¸·Î´Â ¼ö¼úÀû ¿ä¹ý, ±â°èÀûÀÎ ¹æ¹ýÀÇ Ä¡·á, ÈÇÐÀû ¿ä¹ý, ¾à¹°¿ä¹ý µîÀÌ ÀÌ¿ëµÇ°í ÀÖÀ¸¸ç, ±¸°º´¼Ò´Â ´ë°³ Ä¡·á¸¦ ÇÊ¿ä·Î ÇÏÁö´Â ¾ÊÁö¸¸ ȯÀÚÀÇ ºÒÆíÇÔÀÌ ¹ß»ýÇÏ¸é ´ëÁõ¿ä¹ýÀ» ½ÃÇàÇÑ´Ù. ÇǺκ´¼Ò¿Í ±¸°º´¼Ò°¡ µ¿¹ÝµÇ´Â ȯÀÚÀÇ °æ¿ì ¸é¹ÐÇÑ °üÂûÀ» ÅëÇØ ¼ÕÅé°ú ¹ßÅéÀÇ ÀÌ¿µ¾çÁõ, ¹ß¹Ù´ÚÀÇ °ú°¢È, ±¸°Á¡¸·ÀÇ ¹é»ö°¢ÈÁõ µîÀÇ Æ¯Â¡ÀûÀÎ º´¼Ò°¡ ³ªÅ¸³ª¸é Á¶Á÷ »ý°ËÀ» ½ÃÇàÇÏ¿© À̸¦ È®ÁøÇÒ ¼ö ÀÖ¾î¾ß Çϸç, ȯÀÚ°¡ È£¼ÒÇÏ´Â ºÒÆí°¨¿¡ µû¸¥ ÀûÀýÇÑ Ä¡·á¸¦ ½ÃÇàÇÏ¿©¾ß ÇÑ´Ù.
Pachyonychia congenita is a very rare group of an autosomal dominant genodermatosis caused by heterozygous mutations in the keratin genes. The clinical findings affect nail and toenails, soles, and oral mucosa, etc. The main symptoms include nail and toenail dystrophy, hyperkeratosis of hands and feet, follicular hyperkeratosis, oral leukokeratosis. Many therapeutic modalities have been used to treat skin lesion, including surgical and mechanical procedures, chemical agents, medications. Oral lesions but not usually require treatment, if the patient"s discomfort occurs, symptomatic therapy is performed. In the patients accompanied by oral and skin lesions, clinician have to observe specific manifestations with dystrophy of the fingernails and toenails, plantar hyperkeratosis, oral leukokeratosis and tissue biopsy is required for diagnosis confirmed.
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Pachyonychia congenita; Oral mucosa; Leukokeratosis
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