odontogenic keratocysts (okcs) are one of the most frequent features of nevoid basal cell carcinoma syndrome (nbs). it is linked with mutation in the ptch gene. partial expression of the gene may result in occurrence of only multiple recurring okc. our patient presented with nine cysts with multiple recurrences over a period of 11 years without any other manifestation of the syndrome.

gorlin-goltz syndrome (ggs) consists of ectodermal and mesodermal abnormalities. in this case report we will investigate lower extremity lesions of ggs. a 52-year-old man with ggs underwent skull and lower extremity computer tomography. radiographic findings included cervical spondylosis, transparent areas with slurred margins, and cerebral falx calcification. tibial and fibular specific cortic...

Mutations in PATCHED (PTC), the human homolog of the Drosophila patched gene, have been identified in most exons of the gene in patients with the basal cell nevus syndrome and in sporadic basal cell carcinomas. We have screened the 23 PTC exons for mutations using single strand conformation polymorphism analysis of DNA from 86 basal cell nevus syndrome probands, 26 sporadic basal cell carcinoma...

nevoid basal cell carcinoma syndrome (bcns) is an autosomal dominant inherited disorder. multiple organ systems may be affected in this syndrome including abnormalities of the skin, skeletal system, genitourinary system and central nevus system. in this report, we present a case of nevoid basal cell carcinoma syndrome in a 26-year-old male patient. the patient had multiple odontogenic keratocys...

Basal cell nevus syndrome is an autosomal dominant disorder characterized by multiple basal cell carcinomas, along with numerous other documented clinical features. Acrochordons (or skin tags) are common benign neoplasms that are appropriately left untreated in most patients. We describe two patients with known BCNS who were found to have multiple BCCs that clinically resembled acrochordons. Ou...

Mutations in the tumor suppressor gene PATCHED (PTC) are found in human patients with the basal cell nevus syndrome, a disease causing developmental defects and tumors, including basal cell carcinomas. Gene regulatory relationships defined in the fruit fly Drosophila suggest that overproduction of Sonic hedgehog (SHH), the ligand for PTC, will mimic loss of ptc function. It is shown here that t...

A case of basal cell nevus syndrome is reported. The patient, a 25-year-old man, had no family history of this condition. The patient showed multiple mandibular cysts, calcification of the falx cerebri, bridging of the sella turcica, ocular hypertelorimsm, a broad nasal root, and pitting of the palms. These findings correlate with reports in the literature and permitted the diagnosis of basal c...