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Odonto-Onycho-Dermal Dysplasia

OODD


Presentation

  • We report on three boys, two brothers and their maternal cousin, presenting with dry hair, pilar keratosis, severe hypodontia, smooth tongue, onychodysplasia, and keratoderma and hyperhidrosis of palms and soles.[ncbi.nlm.nih.gov]
  • A review of seventeen ectodermal dysplasias is presented for different diagnosis.[wwww.unboundmedicine.com]
  • Odonto-onycho-dermal dysplasia is a rare autosomal recessive syndrome in which the presenting phenotype is dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform and filiform papillae, onychodysplasia, keratoderma and hyperhidrosis[ncbi.nlm.nih.gov]
  • He presented the same clinical featuresas his cousins, but the whole presentation was more severe inhis case (Fig. 6) (Table I). Palmar creases and dermatoglyphicpatterns were unremarkable.[myslide.es]
  • At physical exam, she presented with sparse hair on the scalp and eyebrows, delayed dentition and pointed teeth, and onychodysplasia.[epostersonline.com]
Asymptomatic
  • The patient’s mother, also an obligate carrier of the mutation, was completely asymptomatic. It is not known whether the patient’s grandparents had any symptoms. The patient’s nephews also presented with tooth agenesis and thin hair.[bmcdermatol.biomedcentral.com]
  • This may be the initial or only sign of the condition. [43] Involvement of the dorsum of the tongue might cause an altered sense of taste (dysgeusia). [1] Papular This form is characterized by small white pinpoint papules that are asymptomatic.[en.wikipedia.org]
Malocclusion
  • OMIM reference - 1 MeSH reference: C537742 Very frequent - Abnormal hair texture / hair dysplasia - Anodontia / oligodontia / hypodontia - Autosomal recessive inheritance - Cutaneous rash - Decreased body hair / axillar / pubic hairlessness - Dental malocclusion[csbg.cnb.csic.es]
  • – Jaw joints, chewing muscles and bite abnormalities Bruxism Condylar resorption Mandibular dislocation Malocclusion Crossbite Open bite Overbite Overeruption Overjet Prognathia Retrognathia Scissor bite Maxillary hypoplasia Temporomandibular joint dysfunction[en.wikipedia.org]
Widely Spaced Teeth
  • The characteristicerythematous, reticulated, telangiectatic, atrophicmalar eruption, combined with the presence ofdystrophic nails, palmoplantar hyperkeratosis, con-ical widely spaced teeth with a lack of permanentteeth, and hyperhidrosis typify this[docslide.net]
Hyperhidrosis
  • A thorough clinical examination revealed hypotrichosis and hyperhidrosis of the soles and dental examination revealed agenesis of permanent teeth except the two maxillary central incisors.[ncbi.nlm.nih.gov]
  • We report on three boys, two brothers and their maternal cousin, presenting with dry hair, pilar keratosis, severe hypodontia, smooth tongue, onychodysplasia, and keratoderma and hyperhidrosis of palms and soles.[ncbi.nlm.nih.gov]
  • Odonto-onycho-dermal dysplasia is a rare autosomal recessive syndrome in which the presenting phenotype is dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform and filiform papillae, onychodysplasia, keratoderma and hyperhidrosis[ncbi.nlm.nih.gov]
  • […] autosomal recessive ectodermal dysplasia involving hair, teeth, nails, and skin, characterized by hypodontia (tooth agenesis), smooth tongue with marked reduction of filiform and fungiform papillae, nail dysplasia, dry skin, palmoplantar keratoderma, and hyperhidrosis[ncbi.nlm.nih.gov]
Dry Hair
  • We report on three boys, two brothers and their maternal cousin, presenting with dry hair, pilar keratosis, severe hypodontia, smooth tongue, onychodysplasia, and keratoderma and hyperhidrosis of palms and soles.[ncbi.nlm.nih.gov]
  • Odonto-onycho-dermal dysplasia is a rare autosomal recessive syndrome in which the presenting phenotype is dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform and filiform papillae, onychodysplasia, keratoderma and hyperhidrosis[ncbi.nlm.nih.gov]
  • A rare autosomal recessive ectodermal dysplasia (OMIM:257980) characterised by dry hair, severe hypodontia, smooth tongue with marked reduction of papillae, onychodysplasia, keratoderma and hyperhidrosis of palms and soles, and cutaneous hyperkeratosis[medical-dictionary.thefreedictionary.com]
  • Definition A rare autosomal recessive ectodermal dysplasia characterized by dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform and filiform papillae, onychodysplasia, keratoderma and hyperhidrosis of palms and soles, and hyperkeratosis[uniprot.org]
Dry Skin
  • OODD is a rare form of autosomal recessive ectodermal dysplasia involving hair, teeth, nails, and skin, characterized by hypodontia (tooth agenesis), smooth tongue with marked reduction of filiform and fungiform papillae, nail dysplasia, dry skin, palmoplantar[ncbi.nlm.nih.gov]
  • Odonto-onycho-dermal dysplasia (OODD) is a rare form of ectodermal dysplasia characterized by severe oligodontia, onychodysplasia, palmoplantar hyperkeratosis, dry skin, hypotrichosis, and hyperhidrosis of the palms and soles.[ncbi.nlm.nih.gov]
  • A nonsense mutation in WNT10A was recently identified in odonto-onycho-dermal dysplasia (OODD; MIM 257980), a rare syndrome characterised by severe hypodontia, nail dystrophy, smooth tongue, dry skin, keratoderma and hyperhydrosis of palms and soles.[ncbi.nlm.nih.gov]
  • The patient, the last son in an outbred sibship of four males, presents scalp hypotrichosis, aplasia cutis congenita of the scalp, dental abnormalities, onychodyplasia, dry skin with hypochromic and atrophic (poikiloderma-like) spots with vicarious (marginal[wwww.unboundmedicine.com]
  • skin, palmoplantar keratoderma, and hyperhidrosis of palms and soles, caused by a mutation in the WNT10A gene. * [17641] The statements on this Web site have not been evaluated by the Food and Drug Administration (FDA).[wellnessadvocate.com]
Sparse Eyebrows
  • Some patients may have dry, thin hair and sparse eyebrows, and may have a smooth tongue with missing taste buds.[sema4genomics.com]
  • eyebrows [ 16 ].[bmcdermatol.biomedcentral.com]
  • Some STHAG4 patients manifest mild features of ectodermal dysplasia, including sparse hair, sparse eyebrows, short eyelashes, abnormalities of the nails, sweating anomalies and dry skin.[genecards.org]
Palmar Keratosis
  • keratosis, dermatoglyphic alterations, syndactyly, clinodactyly, phalangeal aplasias and hypoplasias, right leukoma, abnormal EEG, and other findings.[wwww.unboundmedicine.com]
  • Fig. 4. a: Photograph of Patient 2.b: Online:Hands showing the fingernail dystrophy and the palmar keratosis.[myslide.es]
Malar Rash
  • We describe a boy who developed a blistering malar rash soon after birth. When examined at 11 months, and then at 27 months of age, he had persistent atrophic malar plaques that reddened with heat.[ncbi.nlm.nih.gov]

Treatment

  • The information presented is not intended to replace medical advice or treatment from your own doctor or healthcare provider.[wellnessadvocate.com]
  • It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice .[uniprot.org]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • […] side effects with indication on their severity and prevalence, 5) self-care for after the treatment.[bad.org.uk]

Prognosis

  • Relative worth of estrogen or progesterone receptor and pathologic characteris- tics of differentiation as indicators of prognosis of node negative breast cancer patients: findings from national surgical adjuvant breast and bowel protocol B-06.[docslide.net]
  • Prognosis - Odonto onycho dysplasia with alopecia Not supplied. Treatment - Odonto onycho dysplasia with alopecia Not supplied. Resources - Odonto onycho dysplasia with alopecia Not supplied.[checkorphan.org]
  • At present, the prognosis of three of the molars is very poor. Fig. 1 Upper panel : Hyperkeratosis of the palms with erythema, scaling, erosions and fissuring. Dystrophic finger nails with spooning.[bmcdermatol.biomedcentral.com]
  • "Cutaneous and mucosal lichen planus: a comprehensive review of clinical subtypes, risk factors, diagnosis, and prognosis" . TheScientificWorldJournal . 2014 : 1–22. doi : 10.1155/2014/742826 . PMC 3929580 .[en.wikipedia.org]
  • Prognosis Life expectancy is normal ; the main challenge is the symptomatic management of whichever ectodermal pathologies cause the patient the most concern.[atlasgeneticsoncology.org]

Etiology

  • Etiology Homozygous mutations have been described in the WNT10A gene in six patients born to three consanguineous families. Genetic counseling Transmission is autosomal recessive.[orpha.net]
  • The etiology of oral LP are unknown. It is not clear whether the mechanisms causing isolated oral LP are different from those causing oral LP with cutaneous LP.[en.wikipedia.org]

Epidemiology

  • ORPHA:2721 Synonym(s): OODD Prevalence: Inheritance: Autosomal recessive Age of onset: Childhood ICD-10: Q82.4 OMIM: 257980 UMLS: C0796093 C1275074 MeSH: C537742 GARD: 4054 MedDRA: - Summary Epidemiology Less than 15 cases have been described so far.[orpha.net]
  • - OODD Classification (Orphanet): - Rare developmental defect during embryogenesis - Rare genetic disease - Rare odontologic disease - Rare skin disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological[csbg.cnb.csic.es]
  • A Clinical, Genetic and Epidemiological Study . Baltimore : Johns Hopkins Press . Goodman , R.M. , Gorlin , R.J. 1970 . The Face in Genetic Disorders . Saint Louis : The C.V. Mosby Co. Gorlin , R.J. , Pindborg , J.J. 1964 .[cambridge.org]
  • Community Dentistry and Oral Epidemiology . 15 (1): 52–6. PMID 3467894 . Alabi GO, Akinsanya JB (June 1981). "Lichen planus in tropical Africa". Tropical and Geographical Medicine . 33 (2): 143–7. PMID 7281214 .[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • PMID 24672362 . a b "Lichen Planus: Practice Essentials, Background, Pathophysiology" . 2018-08-14. Gorouhi F, Davari P, Fazel N (2014-01-30).[en.wikipedia.org]

Prevention

  • And are not intended to diagnose, treat, cure or prevent disease. The information presented is not intended to replace medical advice or treatment from your own doctor or healthcare provider.[wellnessadvocate.com]
  • […] of Low Birth Weight Prevention of Neural Tube Defects Prevention of Prematurity Preventative Medicine Preventing Illness Prickly Heat Primary Fibromyalgia Syndrome Primary Nocturnal Enuresis Prostadynia Prostatalgia Prostate Enlargement Prostatitis Psoriasis[olathehealth.org]
  • Webbed toes * Toenail abnormalities * Small teeth Causes - Odonto onycho dysplasia with alopecia * Acrofacial dysostosis Preis type * Acropectoral syndrome * Acropectorovertebral dysplasia * Bardet-Biedl syndrome, type 1 * Bardet-Biedl syndrome, type 10 Prevention[checkorphan.org]
  • Physicians may recommend that impacted nasal secretions be carefully removed on a regular basis to help prevent or limit the severity of rhinitis.[rarediseases.org]
  • The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer.[gulfdoctor.net]

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