Korean J Dermatol.  1992 Dec;30(6):951-957.

Two cases of nevoid basal cell carcinoma syndrome

• Joh GY
• Kim KH
• Nam JT
• Kim YS
• Lee BK

Abstract

Nevoid basal cell carcinoma syndrome is inherited as an autcsonal dominant trait with a high degree of penetrane and variable expressivity. The mair features are basal cell carcinoma. jaw cysts, skeletal anornalies, ectopic calcifications and palmoplantar pits. We report varous clinical ma.nifestations in brother and sister, wnich are jaw cyst,, bifid rib, shortened fourth metacarpals, ectopie calcification, palrnoplanar pits and basal cell carcinoma, Microscopically, histologic patterns of basal cell cacinomas are superficial, nodulocystic, keratotic, pigmented, adenoid and follicullar nevoid. Lange numbers of basal cell carcinomas were electrodessicated and curetted under general,nesthesia.

Keyword

Nevoid basal cell car cinoma symdrome; Basal cell caroinoma; Palmoplantar pits; Follicular nevoid

MeSH Terms

Adenoids
Basal Cell Nevus Syndrome*
Carcinoma, Basal Cell
Humans
Jaw Cysts
Metacarpal Bones
Ribs
Siblings