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Living with Odonto-onycho-dermal dysplasia

A rare genetic condition affecting teeth, nails, skin, and sweating, with missing teeth and cone-shaped front teeth.

Understanding Odonto-onycho-dermal dysplasia

Odonto-onycho-dermal dysplasia is a rare genetic ectodermal dysplasia that affects the teeth, nails, and skin.

Many people have missing baby and adult teeth, with cone-shaped incisors and canines. Nails are poorly formed, and skin on the palms and soles is thick and dry. Hair may be sparse, and sweating may be too high or too low. GARD lists childhood as the usual onset.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • Missing adult teeth
  • Cone-shaped front teeth
  • Dry skin and thick skin on the palms and soles
  • Poorly formed or ridged nails
  • Sparse scalp hair and eyebrows
  • Too little or too much sweating

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists, gum, teeth and mouth specialists and skin, hair, and nail specialists.

  3. Consider genetic testing

    Changes in WNT10A are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

  4. Connect with expert centers

    National Foundation for Ectodermal Dysplasias may keep lists of experts for this condition.

Care & treatment

Care team
Genetic specialists (geneticists); Gum, teeth and mouth specialists (odontologists); Skin, hair, and nail specialists (dermatologists)
Expert care
Expert lists: National Foundation for Ectodermal Dysplasias
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with National Foundation for Ectodermal Dysplasias and other patient organizations
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Which dental treatments suit missing or cone-shaped teeth?
How do I manage sweating differences, such as in hot weather?
Would WNT10A genetic testing help my relatives?

Quick facts

Symptoms usually startChild
InheritanceAutosomal Recessive
GenesWNT10A

Changes in the WNT10A gene cause this condition. It is inherited in an autosomal recessive way, meaning a child inherits a changed copy of the gene from each parent.

GeneticSkin

Also known as: oodd

Patient organizations

Source: NIH GARD · Odonto-onycho-dermal dysplasiaGARD ID 4054 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Odonto-onycho-dermal dysplasia.

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