Case Report

Multiple Skin Cysts in Nevoid Basal Cell Carcinoma Syndrome: A Case Report and Review of the Literature
Sei-ichiro Motegi, Yayoi Nagai, Atsushi Tamura, Osamu Ishikawa

Department of Dermatology, Gunma University Graduate School of Medicine, Gunma, Japan

Address of Corresponding Author

Dermatology 2008;216:159-162 (DOI: 10.1159/000111514)

  Key Words

• Nevoid basal cell carcinoma syndrome
• Skin cyst
• Odontogenic keratocyst
• Cutaneous keratocyst
• Epidermal cyst

  Abstract

We report a 72-year-old man with nevoid basal cell carcinoma syndrome (NBCCS) who presented with 25 skin cysts on his trunk and extremities. Fourteen of 25 skin cysts (56%) were observed on his hands. Histological examination demonstrated that most of the excised cysts had an epithelial wall with typical epidermal keratinization. However, 4 skin cysts (4/16; 25%) showed a corrugated or festooned inner surface of wall without a granular cell layer, which resembled an odontogenic keratocyst, a characteristic feature of skin cysts in NBCCS. A review of the literature suggested that acral localization of skin cysts is a distinctive feature of NBCCS. In addition, the histological findings of skin cysts resembling an odontogenic keratocyst might be a hallmark of NBCCS.

Copyright © 2008 S. Karger AG, Basel

  Author Contacts

Dr. Sei-ichiro Motegi
Department of Dermatology, Gunma University Graduate School of Medicine
3-39-22 Showa-machi, Maebashi
Gunma 371-8511 (Japan)
Tel. +81 27 220 8284, Fax +81 27 220 8285, E-Mail smotegi@showa.gunma-u.ac.jp

  Article Information

Received: May 15, 2007
Accepted: June 20, 2007
Published online: January 23, 2008
Number of Print Pages : 4
Number of Figures : 3, Number of Tables : 1, Number of References : 22