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1 OMIM reference -
1 associated gene
23 signs/symptoms
COMMON GENES: 1
COMMON SIGNS: 6
1 OMIM reference -
1 associated gene
11 signs/symptoms
Odonto-onycho-dermal dysplasia
Schöpf-Schulz-Passarge syndrome

WNT10A WNT10A


COMMON
GENES
WNT10A



Citations in the biomedical literature:


Odonto-onycho-dermal dysplasia
WNT10A
Schöpf-Schulz-Passarge syndrome



Odonto-onycho-dermal dysplasia
Schöpf-Schulz-Passarge syndrome

Synonym(s):
- OODD

Synonym(s):
- Eccrine tumors-ectodermal dysplasia
- Keratosis palmoplantaris - cystic eyelids - hypodontia - hypotrichosis
- Palmoplantar hyperkeratosis - cystic eyelids - hypodontia - hypotrichosis
- Palmoplantar keratoderma - cystic eyelids - hypodontia - hypotrichosis
- SSPS

Classification (Orphanet):
- Rare developmental defect during embryogenesis
- Rare genetic disease
- Rare odontologic disease
- Rare skin disease
Classification (Orphanet):
- Rare developmental defect during embryogenesis
- Rare genetic disease
- Rare odontologic disease
- Rare oncologic disease
- Rare skin disease

Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -
Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -

Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: childhood
Average age of death: normal
Type of inheritance: autosomal recessive
Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: childhood
Average age of death: normal
Type of inheritance: autosomal recessive

External references:
1 OMIM reference -
1 MeSH reference: C537742
External references:
1 OMIM reference -
No MeSH references


COMMON
SIGNS
- Absent / decreased / thin eyebrows
- Anodontia / oligodontia / hypodontia
- Autosomal recessive inheritance
- Hypotrichosis / atrichia / atrichiasis / scalp hairlessness
- Palmoplantar hyperkeratosis / keratoderma
- Skin / cutaneous neoplasm / tumor / carcinoma / cancer (excluding melanoma)


Odonto-onycho-dermal dysplasia
Schöpf-Schulz-Passarge syndrome

Very frequent
- Abnormal hair texture / hair dysplasia
- Cutaneous rash
- Decreased body hair / axillar / pubic hairlessness
- Dental malocclusion
- Dysplastic / thick / grooved fingernails
- Dysplastic / thick / grooved toenails
- Erythema / erythematous lesions / erythroderma / polymorphous erythema
- Hyperhidrosis / increased sweating
- Tooth shape anomaly
- Urticaria

Frequent
- Acanthosis nigricans
- Anomalies of tongue, gingiva and oral mucosa
- Blepharitis / eyelid inflammation
- Chronic uveitis / blepharitis / episcleritis / scleritis / conjonctivitis / keratitis
- Photophobia
- Skin photosensitivity
- Telangiectasiae of the skin



Very frequent
- Nails anomalies

Frequent
- Alopecia
- Anomalies of eyelids, eyelashes and lacrimal system
- Premature lost of decidious teeth

Occasional
- Ovary / Fallopian tube neoplasm / tumor / carcinoma / cancer (excl. teratoma / germinoma)