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目的了解毛囊皮脂腺囊性错构瘤(FSCH)的临床特点。方法收集诊治的2例FSCH的临床资料并复习国内文献报道的资料完整的10例病例,分析疾病的临床表现、鉴别诊断与治疗方法。结果共12例,男9例,女3例,平均年龄(30.67±9.98)岁,平均病程(13.5±8.40)年。8/12(66.67%)的患者发生于青春期后,以头颈部为主9/12(75.00%)。临床表现皮损为单发或多发,肤色、淡红色的丘疹、斑块或结节,表面呈现脑回状或簇状,稍用力挤压时可见乳白色皮脂溢出。病理变化主要位于真皮层,可见较多扩大、畸形的毛囊皮脂腺囊性结构;皮脂腺小叶大量增生,呈放射状排列,与周围间质有明显裂隙;缺乏二级毛囊结构;治疗多采取手术切除。结论毛囊皮脂腺囊性错构瘤临床少见,主要在发生青春期后发病,皮疹多发,好发于头颈部,预后良好。
Objective To understand the clinical features of follicular sebaceous cystic hamartoma (FSCH). Methods The clinical data of 2 cases diagnosed and treated by FSCH were collected and 10 cases with complete data reported in domestic literature were reviewed. The clinical manifestations, differential diagnosis and treatment of the disease were analyzed. Results A total of 12 cases, 9 males and 3 females, the average age (30.67 ± 9.98) years, the average duration (13.5 ± 8.40) years. 8/12 (66.67%) patients occurred after puberty, mainly in the head and neck 9/12 (75.00%). Clinical manifestations of skin lesions as single or multiple, complexion, pink papules, plaques or nodules, the surface showed the brain back or cluster, a little squeeze squeeze visible milky seborrhea. Pathological changes are mainly located in the dermis, showing more enlarged, deformity follicular sebaceous cystic structure; sebaceous glands lobular hyperplasia, radially arranged, with the surrounding interstitial significant cleft; the lack of secondary hair follicle structure; treatment to take more resection. Conclusions Hair follicle sebaceous cystic hamartoma is rare in clinical practice. It mainly develops after onset of puberty. It often occurs in head and neck with a good prognosis.