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Concept information

Preferred term

Basal Cell Nevus Syndrome  

Type

  • Topical Descriptor

Entry terms

  • Fifth Phacomatoses
  • Fifth Phacomatosis
  • Gorlin Goltz Syndrome
  • Gorlin-Goltz Syndrome
  • Gorlin Syndrome
  • Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies
  • NBCCS
  • Nevoid Basal Cell Carcinoma Syndrome
  • Nevus Syndrome, Basal Cell
  • Syndrome, Gorlin
  • Syndrome, Gorlin-Goltz

Note

  • coordinate IM with precoordinated organ/neoplasm (IM or NIM) only if pertinent; do not confuse entry term GORLIN-GOLTZ SYNDROME with GOLTZ- GORLIN SYNDROME see FOCAL DERMAL HYPOPLASIA

Scope note

  • Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant.

History note

  • 91(80); was see under CARCINOMA, BASAL CELL 1980-90

In other languages

URI

http://www.yso.fi/onto/mesh/D001478

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