눈꺼풀에 발생한 림프종모양 구진증 1예
A Case of Lymphomatoid Papulosis of the Eyelid
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목적: 림프종모양 구진증은 피부에서 발생하는 원발성 CD양성림프구증식질환의 한 종류로서 눈꺼풀에 발생하는 경우는 매우 드물게보고되었다. 저자들은 안쪽눈구석에 발생한 전형적인 림프종모양 구진증 1예를 경험하여 문헌고찰과 함께 보고하고자 한다. 증례요약: 40세 여자환자가 두 달전부터 발생한 좌측 안쪽눈구석부위의 피부 종괴를 주소로 내원하였다. 발생초기 좌측 안쪽눈구석부위에 작은 피부병변이 발생하여 보존적 치료를 받았으나 호전되지 않고 피부부종이 점점 심해지면서 중심부에 궤양이 있는 종괴가형성되었으며, 증상발생 6주경부터 부종은 차차 호전되었다고 하였다. 눈검사에서 좌측 안쪽눈구석에 6 × 6 mm 크기의 반구형으로융기된 종괴가 관찰되었고, 종괴의 중심부는 원형의 분화구(crater)에 가피가 덮힌 모양이었다. 각질가시세포종 의심하에 절개생검 후종괴의 완전절제 및 전층피부이식술을 시행하였다. 조직검사상 진피층에 다수의 염증세포들의 침윤과 CD 30 양성을 보이는 비전형림프구가 관찰되어 림프종모양 구진증으로 진단하였다. 전신검사 결과 전신침범 소견은 발견되지 않았고, 병변 발생 후 1년이 지난현재까지 재발이나 다른 림프종의 병발 소견은 없는 상태이다.
Abstract
Purpose: Lymphomatoid papulosis (LyP) is one of the primary cutaneous CD30-positive lymphoproliferative disorders. LyP of the eyelid has rarely been reported. Herein, a case of typical LyP of the medial canthal area is reported. In addition,a literature review was performed. Case summary: A 40-year-old female presented with a skin mass in the medial canthal area of the left eye that developed 2 months earlier. Initially, a focal skin lesion developed, and even with conservative treatment at a local clinic, progressed to a mass lesion having a central ulceration and adjacent edema. After 6 weeks, the adjacent edema had gradually decreased. On ophthalmic examination, the left medial canthal lesion was a 6 x 6 mm sized elevated mass with a central crater covered by crust. The clinical impression was keratoacanthoma. The lesion was widely excised and reconstructed by a full-thickness skin graft after an incisional biopsy. Histopathologic findings showed dermal infiltration of various inflammatory cells with atypical lymphocytes showing positivity to the CD30 antigen, and LyP was diagnosed. Systemic evaluation showed no evidence of systemic lymphoma and the patient has remained free of recurrence or systemic disease after a 1-year follow-up.
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