코우덴 증후군과 연관된 다발성 눈꺼풀 모종 1예
A Case of Multiple Eyelid Trichilemmoma Associated with Cowden Syndrome
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목적: 눈꺼풀과 얼굴에 발생한 다발성 모종(Trichilemmoma)이 Cowden 증후군과 연관되었던 1예를 보고하고자 한다. 증례요약: 27세 여자 환자가 위, 아래 눈꺼풀의 다발성 종양을 주소로 내원하였다. 종양은 백색의 좁쌀 크기로 양안 눈꺼풀과 눈꺼풀테,뺨 주위에서 여러 개가 관찰되었다. 환자는 과거 갑상선 양성 종양으로 갑상선 부분절제술을 시행 받았으며, 팔, 다리의 혈관종 제거수술을 시행 받은 병력이 있었다. 조직 검사를 시행한 결과 모종에 합당한 피부병리소견이 관찰되었다. 이에 다발성 모종과 연관된Cowden 증후군으로 진단하고, 추가적 종양 발견을 위해 유방 검사를 시행한 결과 종양이 발견되었으며 조직검사에서 관내 유두종과섬유선종이 발견되었다. 결론: 모종은 모낭에 생기는 양성 종양으로 눈꺼풀에 다발성으로 발생한 경우 Cowden 증후군의 가능성을 고려하여야 할 것이며, 타장기의 종양 동반 유무를 확인해야 한다.
Abstract
Purpose: To report a case of multiple eyelid trichilemmomas associated with Cowden syndrome. Case summary: A 27-year-old woman presented with multiple upper and lower eyelid skin masses that developed over several years. The masses were as large as whitish millet, and were around the upper and lower eyelid margin and the face. The patient had previously undergone subtotal thyroidectomy for a thyroid mass and a mass excision for extremity hemangioma. Excisional biopsy was performed for the diagnosis, and trichilemmoma was diagnosed based on histopathologic examination. Consequently, multiple trichilemmoma associated with Cowden syndrome was diagnosed, and breast evaluations for existence of further masses were recommended. On breast examinations, intraductal papilloma and fibroadenoma were detected. Conclusions: The trichilemmoma was a hair-follicle benign tumor that also appeared on the skin around the eyelid. If multiple trichilemmoma is diagnosed, an association with Cowden syndrome should be considered as well as presence of masses in other organs.
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