Ectodermal Dysplasia (definition)

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Ectodermal Dysplasia

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Definition: 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.

Examples Ectodermal Dysplasia 1, Anhidrotic; Ectodermal Dysplasia 3, Anhidrotic; Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive; Ellis-Van Creveld Syndrome; Focal Dermal Hypoplasia; Neurocutaneous Syndromes

Other names Anhidrotic Ectodermal Dysplasias; Hydrotic Ectodermal Dysplasia; Ectodermal Dysplasia Anhidrotic; Defect, Congenital Ectodermal; Congenital Ectodermal Defect; Anhydrotic Ectodermal Dysplasia; Hidrotic Ectodermal Dysplasia; Ectodermal Defect, Congenital; Clouston's Syndrome; Aplasia Cutis Congenita; Anhidrotic Ectodermal Dysplasia; Syndrome, Clouston's; Hydrotic Ectodermal Dysplasias; Hidrotic Ectodermal Dysplasias; Ectodermal Dysplasias, Hydrotic; Ectodermal Dysplasias, Hidrotic; Ectodermal Dysplasias, Anhydrotic; Ectodermal Dysplasias, Anhidrotic; Ectodermal Dysplasias; Ectodermal Dysplasia, Hydrotic

Substance	Categories	Source
NEMO mutation with immunodeficiency	Ectodermal Dysplasia Immunologic Deficiency Syndromes *Genetic Diseases, X-Linked.
Naegeli syndrome	Ectodermal Dysplasia Hypohidrosis *Keratoderma, Palmoplantar.
Anal sphincter dysplasia	Constipation Ectodermal Dysplasia Encopresis Fecal Incontinence Anal Canal/abnormalities.
Adams Oliver syndrome	Ectodermal Dysplasia Scalp Dermatoses/congenital *Limb Deformities, Congenital.
Ladda Zonana Ramer syndrome	Arthrogryposis Cleft Palate Ectodermal Dysplasia Growth Disorders.
Propping Zerres syndrome	Anodontia Ectodermal Dysplasia Lacrimal Duct Obstruction Nails, Malformed Pigmentation Disorders Limb Deformities, Congenital Breast/abnormalities.
Ectodermal dysplasia mental retardation syndactyly	Ectodermal Dysplasia Intellectual Disability *Syndactyly.
Ectodermal dysplasia alopecia preaxial polydactyly	Alopecia Ectodermal Dysplasia Polydactyly Facies.
Ectodermal dysplasia adrenal cyst	Adrenal Gland Diseases Cysts *Ectodermal Dysplasia.
Ectodermal dysplasia 2, hidrotic	*Ectodermal Dysplasia.
Madokoro Ohdo Sonoda syndrome	Ectodermal Dysplasia Ectromelia Nasolacrimal Duct/abnormalities.
Aplasia cutis congenita intestinal lymphangiectasia	Ectodermal Dysplasia Lymphangiectasis, Intestinal.
Odontoonychodermal dysplasia	Ectodermal Dysplasia Odontodysplasia.
Odontomicronychial dysplasia	Ectodermal Dysplasia Nail Diseases *Tooth Abnormalities.
Basan syndrome	Ectodermal Dysplasia Nails, Malformed.
Seres-Santamaria Arimany Muniz syndrome	Cleft Palate Ectodermal Dysplasia Eyelids/abnormalities.
Sener syndrome	Cysts Brain Diseases Ectodermal Dysplasia Craniofacial Abnormalities.
Arthrogryposis and ectodermal dysplasia	Arthrogryposis Ectodermal Dysplasia.
AREDYLD Syndrome	Ectodermal Dysplasia Diabetes Mellitus, Lipoatrophic *Tooth Abnormalities.
Bustos Simosa Pinto Cisternas syndrome	*Ectodermal Dysplasia.
Brunoni syndrome	Radius Dwarfism Ectodermal Dysplasia *Facies Thumb/abnormalities.
Pinheiro Freire-Maia Miranda syndrome	Anodontia Ectodermal Dysplasia *Nails, Malformed.	Clin Genet. 1986;29(4):332-6
Focal facial dermal dysplasia	*Ectodermal Dysplasia.
Taurodontia absent teeth sparse hair	Anodontia Ectodermal Dysplasia.
Aplasia cutis congenita of limbs recessive	Choanal Atresia Ectodermal Dysplasia.
Zlotogora syndrome	Cleft Palate Ectodermal Dysplasia Intellectual Disability Syndactyly.
Yunis Varon syndrome	Cleidocranial Dysplasia Ectodermal Dysplasia Micrognathism Limb Deformities, Congenital.
Alves Castelo dos Santos syndrome	*Ectodermal Dysplasia.
Trueb Burg Bottani syndrome	Ectodermal Dysplasia Syndactyly *Monilethrix.
Trichoscyphodysplasia	Ectodermal Dysplasia Osteochondrodysplasias.
Trichodental syndrome	Ectodermal Dysplasia Intellectual Disability Microcephaly Odontodysplasia.
Ectodermal dysplasia, ectrodactyly, and macular dystrophy	Corneal Dystrophies, Hereditary Ectodermal Dysplasia *Hand Deformities, Congenital.
Ectrodactyly-cleft lip/palate syndrome	Cleft Lip Cleft Palate *Ectodermal Dysplasia.
Ectodermal dysplasia/ skin fragility syndrome	Ectodermal Dysplasia Skin Diseases.
Ectodermal dysplasia, sensorineural hearing loss, and distinctive facial features	Ectodermal Dysplasia Hearing Loss, Sensorineural *Facies.
Ectodermal dysplasia, hypohidrotic, with immune deficiency	Ectodermal Dysplasia Immunologic Deficiency Syndromes.
Ectodermal dysplasia, hidrotic, Christianson-Fourie type	*Ectodermal Dysplasia.
Jones Hersh Yusk syndrome	Ectodermal Dysplasia Craniofacial Abnormalities.
Johanson Blizzard syndrome	Deafness Ectodermal Dysplasia Hypothyroidism Pancreatic Diseases Nose/abnormalities.
Robinson Miller Bensimon syndrome	Ectodermal Dysplasia Hearing Loss, Sensorineural.
Hay-Wells syndrome	Cleft Lip Cleft Palate Ectodermal Dysplasia Eye Abnormalities Eyelids/abnormalities.
Hay Wells syndrome recessive type	Cleft Lip Cleft Palate *Ectodermal Dysplasia Eyelids/abnormalities.
Euhidrotic ectodermal dysplasia	Ectodermal Dysplasia Hyperopia *Tooth Abnormalities.
Congenital ectodermal dysplasia with hearing loss	Contracture Ectodermal Dysplasia *Hearing Loss, Sensorineural Fingers/abnormalities.
Freire-Maia odontotrichomelic syndrome	Ectodermal Dysplasia Limb Deformities, Congenital.
Halal Setton Wang syndrome	*Ectodermal Dysplasia.
Cardiofaciocutaneous syndrome	Ectodermal Dysplasia Failure to Thrive Heart Defects, Congenital Facies.
Contractures ectodermal dysplasia cleft lip palate	Arthrogryposis Cleft Palate Ectodermal Dysplasia Growth Disorders.
Epidermolysis bullosa with pyloric atresia	*Ectodermal Dysplasia.
Dermatoosteolysis Kirghizian type	Anodontia Ectodermal Dysplasia Keratitis Osteolysis *Skin Ulcer/congenital.
Cerebellar ataxia ectodermal dysplasia	Cerebellar Ataxia Ectodermal Dysplasia.
Rapp-Hodgkin syndrome	Cleft Lip Cleft Palate *Ectodermal Dysplasia.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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