CLINICAL FINDINGS OF BASAL CELL NEVUS SYNDROME.

Abstract

Background Basal cell nevus syndrome or Gorlin syndrome is a rare genetic autosomal dominant disorder associated with different neoplasms that may be aggressive with further aging so that earlier diagnosis of this syndrome is crucialAim of the study The aim of this study is to present different clinical pictures and diagnostic means to facilitate identification of patients affected by basal cell nevus bifid rib syndrome Subject and methods Seven patients presented with basal cell nevus syndrome were investigated through reporting medical and dental history, clinical and radiological examinations, histopathological investigations , routine laboratory investigations and referral to different specialties as dermatology , neurosurgery and orthopedics .Results All the patients presented with odontogenic keratocysts , bifid ribs, skin nevi, and skeletal disorders .. with three patients having family history affected by the same findings .Conclusionbasal cell nevus bifid rib syndrome has different clinical presentations and many specialists of different specialties as maxillofacial surgery, neurosurgery , dermatology and others must be aware of them ,also affected patients need frequent, follow up to avoid any complications associated with this syndrome ..