Gorlin Goltz Syndrome, or Nevoid Basal Cell Carcinoma is an autosomal dominant genetic disease characterized especially by the manifestation of basal cell carcinomas in skin and keratocysts jaws. Additionally, patients may present with several other manifestations, such as bifid ribs, hypertelorism calcification falx cerebri. The diagnosis has physicians dentists protagonists investigation invo...

The Nevoid Basal-Cell Carcinoma Syndrome (NBCC), or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associate...

The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome-NBCCS) is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched) gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multi...

Essential generalized telangiectasia as the result of postcapillary venule dilatation, is characterized by the sudden development of generalized telangiectasias, sometimes involving the conjunctiva. A few pediatric cases have been reported. The main condition in the differential diagnosis includes syndromes with telangiectasia such as hereditary hemorrhagic telangiectasia of Rendu-Osler, unilat...

Sir, Linear and whorled nevoid hypermelanosis (LWNH) is characterized by hyperpigmented macules in a streaky configuration along the lines of Blaschko, without preceding inflammation or atrophy.[1-3] Lesions are distributed mainly on the trunk and extremities, sparing palms, soles, and mucosae. The usual age of the onset of hyperpigmentation occurs within the first few weeks of life, continues ...

The lesion traditionally known as odontogenic keratocyst has been renamed by WHO in 2005, as “keratocystic odontogenic tumor” as it is more appropriate and reflects its potential for local, destructive behavior. It is a benign intraosseous neoplasm of jaw, which is unusual due to its characteristic histopathological and clinical features, including potentially aggressive behavior, high recurren...

A 55-year-old Caucasian man presented with unilateral right facial spider nevi. Relevant medical history included a right-sided cerebrovascular accident (CVA) in 1997 and decompensated alcoholic liver disease, diagnosed in 2007. The literature describes approximately 100 cases of unilateral spider nevi associated with different aetiologies, grouped under 'unilateral nevoid telangiectasia syndro...

During a routine faculty prosection of the head and neck region of an adult female cadaver, a partial ossification of the falx cerebri was found. Ossification of other dural areas or regions of the body were not found. In addition, the brain and remaining organs appeared to be grossly normal. Reports of partial ossification of the falx cerebri are still rare and while certain pathologies such a...

Among all pathology fields, the analysis of melanocytic lesions has one of the highest rates of review for legal reasons, particularly regarding the distinction between nevus and melanoma (desmoplastic melanoma, nevoid melanoma, Spitz nevus vs. spitzoid melanoma, etc). Therefore, it follows that pathologists should pay special attention when dealing with such type of lesions. This lecture will ...

Tufted hair folliculitis is a rare folliculitis of the scalp that resolves with patches of scarring alopecia within multiple hair tufts emerging from dilated follicular orifices. Tufting of hair is caused by clustering of adjacent follicular units due to a fibrosing process and to retention of telogen hairs within a dilated follicular orifice. Various pathogenetic mechanisms have been proposed ...