Biomaterial Database

[Focal dermal hypoplasia (Goltz's syndrome). Review of the literature and clinical contribution]. 1989

E Bucci, and M D Mignogna, and L Lo Muzio, and P Bucci

A clinical case of focal dermal hypoplasia or Goltz syndrome is described with details of the odontostomatological features both generally encountered and observed in the present case. The main aetiopathogenic hypotheses are also discussed and a dominant X-linked transmission is postulated.

UI	MeSH Term	Description	Entries
D009057	Stomatognathic Diseases	General or unspecified diseases of the stomatognathic system, comprising the mouth, teeth, jaws, and pharynx.	Dental Diseases,Mouth and Tooth Diseases,Dental Disease,Disease, Dental,Disease, Stomatognathic,Diseases, Dental,Diseases, Stomatognathic,Stomatognathic Disease
D010641	Phenotype	The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.	Phenotypes
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D004476	Ectodermal Dysplasia	A group of hereditary disorders involving tissues and structures derived from the embryonic ectoderm. They are characterized by the presence of abnormalities at birth and involvement of both the epidermis and skin appendages. They are generally nonprogressive and diffuse. Various forms exist, including anhidrotic and hidrotic dysplasias, FOCAL DERMAL HYPOPLASIA, and aplasia cutis congenita.	Anhidrotic Ectodermal Dysplasia,Aplasia Cutis Congenita,Clouston's Syndrome,Ectodermal Defect, Congenital,Hidrotic Ectodermal Dysplasia,Anhydrotic Ectodermal Dysplasia,Aplasia Cutis Congenita, Nonsyndromic,Autosomal Dominant Hidrotic Ectodermal Dysplasia,Clouston Hidrotic Ectodermal Dysplasia,Clouston Syndrome,Clouston's Hidrotic Ectodermal Dysplasia,Congenital Ectodermal Defect,Defect, Congenital Ectodermal,Ectodermal Dysplasia 2, Hidrotic,Ectodermal Dysplasia Anhidrotic,Ectodermal Dysplasia, Anhydrotic,Ectodermal Dysplasia, Hidrotic,Ectodermal Dysplasia, Hidrotic, Autosomal Dominant,Hidrotic Ectodermal Dysplasia, Autosomal Dominant,Hydrotic Ectodermal Dysplasia,Anhidrotic Ectodermal Dysplasias,Anhidrotic, Ectodermal Dysplasia,Anhidrotics, Ectodermal Dysplasia,Anhydrotic Ectodermal Dysplasias,Cloustons Syndrome,Congenital Ectodermal Defects,Defects, Congenital Ectodermal,Dysplasia Anhidrotic, Ectodermal,Dysplasia Anhidrotics, Ectodermal,Dysplasia, Anhidrotic Ectodermal,Dysplasia, Anhydrotic Ectodermal,Dysplasia, Ectodermal,Dysplasia, Hidrotic Ectodermal,Dysplasia, Hydrotic Ectodermal,Dysplasias, Anhidrotic Ectodermal,Dysplasias, Anhydrotic Ectodermal,Dysplasias, Ectodermal,Dysplasias, Hidrotic Ectodermal,Dysplasias, Hydrotic Ectodermal,Ectodermal Defects, Congenital,Ectodermal Dysplasia, Anhidrotic,Ectodermal Dysplasia, Hydrotic,Ectodermal Dysplasias,Ectodermal Dysplasias, Anhidrotic,Ectodermal Dysplasias, Anhydrotic,Ectodermal Dysplasias, Hydrotic,Hidrotic Ectodermal Dysplasias,Hydrotic Ectodermal Dysplasias,Syndrome, Clouston,Syndrome, Clouston's
D005260	Female		Females
D005489	Focal Dermal Hypoplasia	A genetic skin disease characterized by hypoplasia of the dermis, herniations of fat, and hand anomalies. It is found exclusively in females and transmitted as an X-linked dominant trait.	Dermal Hypoplasia, Focal,Goltz Gorlin Syndrome,Goltz Syndrome,Goltz's Syndrome,Goltz-Gorlin Syndrome,Dermal Hypoplasias, Focal,Focal Dermal Hypoplasias,Goltzs Syndrome,Gorlin Syndrome, Goltz,Hypoplasia, Focal Dermal,Hypoplasias, Focal Dermal,Syndrome, Goltz,Syndrome, Goltz Gorlin,Syndrome, Goltz's,Syndrome, Goltz-Gorlin
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man