Multiple skin cysts in nevoid basal cell carcinoma syndrome: a case report and review of the literature.
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.
Motegi
Sei-ichiro
S
Department of Dermatology, Gunma University Graduate School of Medicine, Gunma, Japan. smotegi@showa.gunma-u.ac.jp
Nagai
Yayoi
Y
Tamura
Atsushi
A
Ishikawa
Osamu
O
D000368:Aged; D001478:Basal Cell Nevus Syndrome / Q000150:complications* / Q000473:pathology; D003560:Cysts / Q000150:complications* / Q000473:pathology; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D012871:Skin Diseases / Q000150:complications / Q000175:diagnosis; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology