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Living with Odonto-tricho-ungual-digito-palmar syndrome

A rare genetic condition with teeth at birth, fragile hair and nails, and differences in the hands and feet.

Understanding Odonto-tricho-ungual-digito-palmar syndrome

Odonto-tricho-ungual-digito-palmar syndrome is a rare ectodermal dysplasia affecting the teeth, hair, nails, hands, and feet.

Babies have teeth at birth (neonatal teeth). Hair is straw-like, discolored, and fragile, and nails are poorly formed. The hands and feet have differences: deep palm creases, short fingers, and very short first hand and foot bones. GARD lists newborn and infant years as the usual onset.

Signs & symptoms

GARD doesn't list specific symptoms for this condition yet. Based on its summary, features may include:

  • Teeth present at birth (neonatal teeth)
  • Straw-like, discolored, fragile hair
  • Poorly formed nails
  • Short fingers (brachydactyly)
  • Deep palm creases
  • Very short first hand and foot bones

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. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

  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
What dental care is needed for teeth present at birth?
How should we care for fragile hair and nails?
What is the chance of passing this condition on?

Quick facts

Symptoms usually startNewborn and as an Infant
InheritanceAutosomal Dominant

GARD doesn't describe a cause for this condition yet. It is inherited in an autosomal dominant way, meaning one changed copy of the gene can be enough.

GeneticSkin

Also known as: odonto-tricho-ungual-digito-palmar syndrome mendoza-valiente type; odonto-tricho-ungual-digito-palmar syndrome, mendoza-valiente type; odontotrichoungual-digital-palmar syndrome; otudp syndrome

Patient organizations

Source: NIH GARD · Odonto-tricho-ungual-digito-palmar syndromeGARD ID 16679 · 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-tricho-ungual-digito-palmar syndrome.

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