Stockfleth Eggert, Ulrich Claas, Hauschild Axel, Lischner Stephan, Meyer Thomas, Christophers Enno
Department of Dermatology of the Charité, Humboldt University, Schumannstra e 20/21, 10117 Berlin, Germany.
Eur J Dermatol. 2002 Nov-Dec;12(6):569-72.
Gorlin-Goltz syndrome, also referred to as naevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant skin disease with complete penetrance and inconstancy of the four major findings: multiple naevoid basal cell carcinomas (BCCs), pits on palms and soles, skeletal abnormalities (for example, jaw cysts), and ectopic calcification. The treatment of multiple BCCs is still a matter of debate. We report three cases of multiple BCCs in Gorlin-Goltz syndrome treated with topical 5% imiquimod cream, an immune response modifier. Patients were successfully cleared of BCCs after treatment for 6-8 weeks. Histologically no apparent signs of BCC-persistence could be detected and no recurrences were detected during the 12 month follow up period.
戈林-戈尔茨综合征,也称为痣样基底细胞癌综合征(NBCCS),是一种常染色体显性皮肤病,具有完全外显率,且四大主要表现具有变异性:多发性痣样基底细胞癌(BCC)、手掌和足底凹陷、骨骼异常(如颌骨囊肿)以及异位钙化。多发性基底细胞癌的治疗仍存在争议。我们报告了3例戈林-戈尔茨综合征患者的多发性基底细胞癌,采用免疫反应调节剂5%咪喹莫特乳膏局部治疗。患者在治疗6 - 8周后成功清除基底细胞癌。组织学检查未发现基底细胞癌持续存在的明显迹象,在12个月的随访期内未发现复发。