Ann Dermatol.  2008 Dec;20(4):221-225. 10.5021/ad.2008.20.4.221.

Two Cases of Nevoid Basal Cell CarcinomaSyndrome in One Family

Affiliations
  • 1Department of Dermatology and Cutaneous Biology Research Institute,Yonsei University College of Medicine, Seoul, Korea. mglee@yuhs.ac

Abstract

The nevoid basal cell carcinoma syndrome, or Gorlin-Goltz syndrome, is an autosomal dominant multiple system disorder with high penetrance and variable expressions, although it can also arise spontaneously. The diagnostic criteria for nevoid basal cell carcinoma syndrome include multiple basal cell carcinomas, palmoplantar pits, multiple odontogenic keratocysts, skeletal anomalies, positive family history, ectopic calcification and neurological anomalies. We report a brother and sister who were both diagnosed with nevoid basal cell carcinoma syndrome.

Keyword

Basal cell carcinoma; Nevoid basal cell carcinoma syndrome; Odontogenic keratocyst

MeSH Terms

Basal Cell Nevus Syndrome
Carcinoma, Basal Cell
Humans
Odontogenic Cysts
Penetrance
Siblings
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