表现为角化棘皮瘤和皮脂腺腺瘤的Muir-Torre综合征
A case of Muir-Torre syndrome presenting as keratoacanthoma and sebaceous adenoma
摘要患者男,44岁.右侧鼻唇沟外侧赘生物2个月余.皮肤科检查:右侧鼻唇沟外侧1.5 cm×1.5 cm半球状隆起,中央呈火山口状.皮损上方2.0 cm×1.5 cm半月形隆起.组织病理:半球状隆起皮损示表皮不规则向下增生,皮损中央呈充满嗜酸性角质栓的火山口样改变,周边两侧上皮向上隆起成领圈状.上皮细胞团块内中性粒细胞小脓肿,增生上皮周围大量淋巴细胞浸润,浸润的病变下界基本清楚.半月形隆起皮损真皮中部较多形态、大小不一小叶状结构,周围结缔组织增生,小叶内见不同成熟阶段的皮脂腺结构.免疫组化:角化棘皮瘤病变中CK,CK5,CK14,CK17,CEA,EMA在其浸润的鳞状细胞团块呈阳性表达;皮脂腺腺瘤中CK,CK5,CK14,EMA在皮脂腺细胞呈阳性表达.病理诊断:角化棘皮瘤合并皮脂腺瘤.患者2008年确诊为直肠中低分化腺癌.结合患者皮肤表现及肠道肿瘤病史,最终诊断:表现为角化棘皮瘤和皮脂腺腺瘤的Muir-Torre综合征.
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abstractsA 44-year-old male presented with a neoplasm on the buccal side of the right nasolabial fold for more than two months.Dermatological examination showed a hemispherical bulge sized 1.5 cm × 1.5 cm with central crater-like ulceration on the buccal side of the right nasolabial fold,as well as a crescent-shaped elevation measuring 1.5 cm × 2.5 cm above the hemispherical lesion.Histopathology of the hemispherical lesion revealed irregularly downward proliferation of epidermis,crater-like holes filled with eosinophilic keratinous plug in the center which were surrounded by collar-shaped epithelial cell projections.Small neutrophil abscesses were found in the clumps of epithelial cells,and massive lymphocyte infiltration with a clear bottom boundary was observed around the proliferating epithelial cells.Histopathologic examination of the crescent lesion showed multiple irregularly-shaped lobular-like structures of various sizes with sebaceous glands at different degrees of maturity in the mid dermis,which were surrounded by proliferating connective tissue.Immunohistochemical studies showed that the squamous cells stained positive for cytokeratin (CK),CK5,CK14,CK17,carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA) in the keratoacanthoma,and the sebaceous cells for CK,CK5,CK14 and EMA in the sebaceous adenoma.The pathological diagnosis was keratoacanthoma and sebaceous adenoma.The patient was diagnosed with moderately and poorly differentiated rectal adenocarcinoma in 2008.A diagnosis of Muir-Torre syndrome presenting as keratoacanthoma and sebaceous adenoma was finally made.
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