Gorlin–Goltz Syndrome
Pronunciation: GOR-lin GOLTS SIN-drohm
An autosomal dominant condition caused by PTCH1 gene mutation, characterised by multiple odontogenic keratocysts, skeletal anomalies, and predisposition to basal cell carcinomas.
Full Definition
Gorlin–Goltz syndrome (also called nevoid basal cell carcinoma syndrome) is a hereditary multisystem disorder with significant oral and maxillofacial manifestations, most notably the development of multiple odontogenic keratocysts in the jaws. Editors working on genetics, oral pathology, or dermatology reports must render the eponym with an en dash between the two surnames (Gorlin–Goltz), not a hyphen. The condition is referenced in both the OMIM database and WHO tumour classifications. Its mention in a pathology report obligates follow-up genetic counselling documentation, and editors should flag missing cross-references. Variant spellings such as 'Gorlin syndrome' alone are acceptable in some style guides but should be confirmed with the house style before use.
Usage
Usage note: Use en dash between eponyms (Gorlin–Goltz), not a hyphen. Confirm whether 'Gorlin syndrome' alone is acceptable in the relevant house style.
In Context
- "A diagnosis of Gorlin–Goltz syndrome was suspected given the patient's history of three odontogenic keratocysts before the age of 20." — Clinical case report
- "Editors must use an en dash in 'Gorlin–Goltz Syndrome,' not a hyphen, per AMA style for compound eponyms." — Journal copyediting guideline