多方向分化附属器肿瘤伴斑痣一例

Skin adnexal tumor with multipotential differentiation complicated by nevus spilus： a case report

患者女，41岁。患者于不到1岁时无明显诱因左侧头顶部出现淡黄色圆形斑片，随年龄增长逐渐增大呈斑块状。2年前局部出现乳头状损害，表面发红、潮湿。约同一时间左颞部出现黑色肿物，逐渐增大并中心破溃，均无自觉症状。患者约1岁时在左颌面、颈项、躯干、上肢淡褐色斑基础上出现黑褐色斑疹，渐增多、扩大，累及同侧手背，并出现丘疹。皮肤科检查：左侧头部皮肤淡黄色斑块基础上分别见约3 cm × 2 cm、2 cm × 1 cm新生物，色鲜红，表面如菜花状，境界清楚，触之较硬，表面糜烂，其间散在大小不等的黑色及淡红色丘疹。左侧颞部一约1.5 cm × 1.5 cm大环形黑色肿物，中心溃疡形成。左侧面颊、下颌、颈项、前胸、肩背、上肢、手背淡褐色斑片，边界清，其上可见黑褐色斑疹及丘疹，触之韧。取多处皮损同时行组织病理检查，头部菜花状皮损诊断为乳头状汗管囊腺瘤，黄色斑块状皮损诊断为乳头状汗管囊腺瘤合并皮脂腺瘤，颞部黑色增生性皮损诊断为毛母细胞瘤并基底细胞上皮瘤，下颌黑素丘疹样皮损及下颌褐色斑丘疹样皮损均诊断为斑痣。结合患者临床表现，诊断：多方向分化附属器肿瘤（乳头状汗管囊腺瘤、乳头状汗管囊腺瘤和皮脂腺瘤、毛母细胞瘤并基底细胞癌），斑痣。

A 41-year-old female patient developed round, bright yellow patches on the left calvarial region without obvious precipitating factors 40 years prior to the presentation, which gradually grew to form plaques with age. Two years prior to the presentation, nipple-like lesions appeared in the calvarial and temporal region with an erythematous and wet surface; concurrently, black masses developed in the left temporal region and gradually enlarged with central ulceration but no subjective symptoms. At about 1 year of age, pitchy macules developed on the light tan patches located on the left jaw, posterior and anterior neck, trunk and upper limbs, and gradually increased in quantity and size with the involvement of the homolateral dorsal hand and gradual appearance of papules. Skin examination revealed two well-marginated, indurated, bright red neoplasms sized 3 cm × 2 cm and 2 cm × 1 cm respectively, with erosive and cauliflower-like surface; black or pink papules were scattered between these neoplasms. There was a ring-shaped black mass sized 1.5 cm × 1.5 cm in the left temporal region with central ulceration. Pitchy tough macules and papules were observed on the light tan patches located in the left cheek, lower mandible, posterior and anterior neck, protothorax, shoulder and back, upper limbs and dorsal hand. Based on the histopathology of multiple lesions, the cauliflower-like lesions on the head were diagnosed as syringocystadenoma papilliferum, the yellow plaques as syringocystadenoma papilliferum complicated by sebaceous adenoma, the black proliferative lesions in the temporal region as trichoblastoma accompanied by basal cell epithelioma,the black papuloid lesions and brown maculopapuloid lesions on the lower mandible as nevus spilus. The patient was diagnosed with skin adnexal tumor with multipotential differentiation （syringocystadenoma papilliferum, sebaceous adenoma, trichoblastoma and basal cell epithelioma） accompanied by nevus spilus.