Pratt M D, Jackson R
J Am Acad Dermatol. 1987 May;16(5 Pt 1):964-70.
The nevoid basal cell carcinoma syndrome is an autosomal dominant condition that consists of multiple basal cell carcinomas of the skin, odontogenic keratocysts, pits of the hands and feet (especially the palms and soles), various ectopic calcifications, notably lamellar calcification of the falx cerebri and skeletal anomalies. As well as these major findings, there are many other associated abnormalities. This paper is a 15-year follow-up study on twelve cases from the Ottawa Valley with details on nine new cases discovered during that time. Sixteen were males; 5, females. The number of basal cell cancers requiring removal varied greatly from patient to patient. There was one patient with a horror lesion; none had metastatic disease.
痣样基底细胞癌综合征是一种常染色体显性遗传病，其特征包括多发性皮肤基底细胞癌、牙源性角化囊肿、手足凹陷（尤其是手掌和脚底）、各种异位钙化，特别是大脑镰层状钙化和骨骼异常。除了这些主要表现外，还有许多其他相关异常。本文是对渥太华谷12例患者进行的15年随访研究，并详细介绍了在此期间发现的9例新病例。其中16例为男性，5例为女性。需要切除的基底细胞癌数量在患者之间差异很大。有1例患者出现可怕的病变；均无转移性疾病。
