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A Case of Diffuse Cutaneous Mastocytosis in a Newborn

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¹Ú¹Ì³ª(Park Mi-Na) - ÀÎÁ¦´ëÇб³ »ó°è¹éº´¿ø ¼Ò¾ÆÃ»¼Ò³â°ú
±è±Ù¾Æ(Kim Geun-A) - ÀÎÁ¦´ëÇб³ »ó°è¹éº´¿ø ¼Ò¾ÆÃ»¼Ò³â°ú
ÃÖ¸íÀç(Chey Myoung-Jae) - ÀÎÁ¦´ëÇб³ »ó°è¹éº´¿ø ¼Ò¾ÆÃ»¼Ò³â°ú
½É±ÔÈ«(Shim Gyu-Hong) - ÀÎÁ¦´ëÇб³ »ó°è¹éº´¿ø ¼Ò¾ÆÃ»¼Ò³â°ú

Abstract

¹Ì¸¸¼º ÇǺΠºñ¸¸ ¼¼Æ÷ÁõÀº ÀϹÝÀûÀ¸·Î Ãʱâ À¯¾Æ±â ´Ü°è¿¡¼­ ÀÓ»óÀûÀ¸·Î ¸í¹éÇÑ ±¤¹üÀ§ È«¹ÝÀ» °¡Áö´Â ºñ¸¸ ¼¼Æ÷ ÁúȯÀÇ Èñ±ÍÇÑ º¯Á¾ÀÌ´Ù. ±¤¹üÀ§ÇÑ È«Á¶¿Í °¡·Á¿ò ¹× ¹Ì¸¸¼º ¼öÆ÷¸¦ µ¿¹ÝÇÑ »ýÈÄ 1ÀÏ µÈ ½Å»ý¾Æ°¡ º»¿øÀ¸·Î Àü¿øµÇ¾ú´Ù. È®ÁøÀ» À§ÇÏ¿© ½ÃÇàµÈ ÇǺΠ»ý°Ë °á°ú¿¡¼­´Â CD117À» ÅëÇÑ ºñ¸¸ ¼¼Æ÷ÀÇ Á¶¹ÐÇÑ ÇǺΠħÅõ¼Ò°ßÀÌ °üÂûµÇ¾ú´Ù. ¹Ì¸¸¼º ÇǺΠºñ¸¸¼¼Æ÷ÁõÀº ´Ù¾çÇÑ ÁßÁõÀÇ ÇǺΠÁúȯÀ¸·Î ³ªÅ¸³ª¸ç, ÁßÀçÀÚ °ü·Ã Áõ»óÀ» º¸ÀÌ°í ¾Æ³ªÇʶô½Ã½ºÀÇ À§Ç輺µµ °¡Áö°í ÀÖ´Â ÁúȯÀÌ´Ù. ÀúÀÚµéÀº ½Å»ý¾Æ¿¡¼­ ¹ß»ýÇÑ ¹Ì¸¸¼º ÇǺΠºñ¸¸¼¼Æ÷Áõ 1·Ê¸¦ °æÇèÇÏ¿´±â¿¡ À̸¦ º¸°íÇÏ°í ¹®Çå°íÂûÀ» ÇϰíÀÚ ÇÏ´Â ¹ÙÀÌ´Ù.
Diffuse cutaneous mastocytosis (DCM) is a rare variant of mast cell disease with widespread erythema and is clinically apparent in early infancy. We report the case of a 1-day-old female neonate who presented with diffuse flush, pruritus, and extensive blistering. DCM was diagnosed by immunohistochemical staining with anti-CD117, which revealed mast cell infiltration. DCM is a severe and heterogeneous cutaneous disease, and is associated with mast cell mediator-related symptoms and risk of anaphylactic shock. We describe this case and provide the first literature review of neonatal onset DCM in Korea.

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ÇǺΠºñ¸¸¼¼Æ÷Áõ, ¹Ì¸¸¼º ÇǺΠºñ¸¸¼¼Æ÷Áõ, ½Å»ý¾Æ
Cutaneous mastocytosis, Diffuse cutaneous mastocytosis, Newborn
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ÇмúÁøÈïÀç´Ü(KCI) KoreaMed ´ëÇÑÀÇÇÐȸ ȸ¿ø 
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DOI
KCDÄÚµå
ICD 03
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