A case of basal cell nevus syndrome: multiple jaw cysts associated with skin lesions and skeletal abnormalities

Basal cell nevus syndrome presenting as a tender mass of the jaw: A case report

[A case of basal cell nevus syndrome].

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...

Basal cell nevus syndrome. A case report.

An 11-year-old boy with multiple dentigerous cysts in the maxilla and mandible is described. Other findings seen in the face, plantar skin, skeletal system and oral cavity indicated the lesions to be due to the basal cell nevus syndrome. This was further confirmed by the presence of similar abnormalities in his father and brother.

Report of a case of Klippel-Trenaunay syndrome associated with bilateral nevus of Ota

Port-wine stain is a vascular malformation characterized pathologically by ectasia of superficial dermal capillaris and clinically by persistent macular erythema. The association of a port-wine stain on a limb with soft tissue swelling, with or without bony overgrowth, is termed klippel-Trenaunay syndrome. Phakomatosis pigmento-vascularis is a combination of port-wine stain and cutaneous ...

Resolution of odontogenic keratocysts of the jaw in basal cell nevus syndrome with GDC-0449.

BACKGROUND Odontogenic keratocysts of the jaw are a central feature of basal cell nevus syndrome (BCNS) and arise from the basal cell layer of the surface epithelium. Although they are benign, they tend to be aggressive, with local invasion of bony structures, extensive growth, and potential for substantial disfigurement and speech dysfunction. Complete surgical resection is the current standar...