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Living with Naegeli-Franceschetti-Jadassohn syndrome

A rare skin condition with a net-like pattern of dark skin, thick skin on the palms and soles, and no fingerprints.

Understanding Naegeli-Franceschetti-Jadassohn syndrome

Naegeli-Franceschetti-Jadassohn syndrome (NFJS/DPR) is a rare type of ectodermal dysplasia that affects the skin, nails, teeth, and sweat glands.

Ectodermal dysplasias affect the skin, hair, nails, teeth, and sweat glands. A common sign is a net-like pattern of dark brown or gray skin on the neck, chest, and belly that appears in infancy or early childhood. It may fade with age. The palms and soles often become thick and hard. Most people lack fingerprints.

Signs & symptoms

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

  • Net-like dark skin patterns
  • Thick, hard skin on palms and soles
  • Brittle or poorly formed nails
  • No fingerprints (adermatoglyphia)
  • Reduced sweating (hypohidrosis)
  • Tooth differences

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 and skin, hair, and nail specialists.

  3. Consider genetic testing

    Changes in KRT14 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); 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
Should we have genetic testing for the KRT14 gene?
How can I stay cool if I sweat less?
How can I care for the skin on my hands and feet?

Quick facts

Symptoms usually startNewborn and as an Infant
InheritanceAutosomal Dominant
GenesKRT14

GARD lists changes in the KRT14 gene. The condition is autosomal dominant, which means one changed copy of the gene can be enough to cause it.

GeneticSkin

Also known as: franceschetti-jadassohn syndrome; naegeli syndrome; naegeli's syndrome; nfj syndrome; nfjs

Patient organizations

Source: NIH GARD · Naegeli-Franceschetti-Jadassohn syndromeGARD ID 3912 · 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 Naegeli-Franceschetti-Jadassohn syndrome.

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