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

• Ryu DJ
• Kwon YS
• Roh MR
• Lee MG

• KMID: 2156399
• DOI: http://doi.org/10.5021/ad.2008.20.4.221

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

Figure

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  Fig. 1 Radiograph taken for the patient showing multiple cystic lesions in the lower maxilla.

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  Fig. 2 Pigmented macules were identified on the palmar skin surface of the right hand.

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  Fig. 3 Histological section of pigmented macules on the palmar surface shows a well-circumscribed neoplasm comprised of basaloid neoplastic cells arranged in an anastomosing fashion (H&E stain, ×100).

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  Fig. 4 Bilateral deformities of the 3rd, 4th and 5th ribs.

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