Syn .: Gorlin syndrome, Gorlin-Goltz syndrome.
Def .: Autosomal dominant syndrome with the presence of a large number of basal cell epitheliomas from early childhood along with other developmental defects, especially of the skeletal system and the CNS.
Clinical: Numerous basal cell carcinomas, which in the early period resemble melanocytic nevi or skin fibromas, and later – typical epitheliomas. In addition, there are small depressions, lipomas and epidermal cysts on the skin of the hands and soles. In the skeletal system – a large skull, hypertelorism, odontogenic cysts in the mandible, less often in the maxilla. In the CNS – calcification of the dura mater, mental retardation. The risk of developing systemic tumors.
Heal: Observation, removal of skin cancers (radiotherapy contraindicated).
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House