Completed

Research Registry for Inherited Disorders of Keratinization

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What is being collected

Data Collection

Collected from past medical records and data - Retrospective
DNA Samples
Who is being recruted

Congenital Abnormalities+16

+ Ichthyosis

+ Infant, Newborn, Diseases

See all eligibility criteria
How is the trial designed

Cohort

Tracking disease incidence in order to identify risk factors and understand disease progression over time.
Observational
Study Start: September 1994
See protocol details

Summary

Principal SponsorNational Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Last updated: January 18, 2026
Sourced from a government-validated database.Claim as a partner

Study start date: September 1, 1994

Actual date on which the first participant was enrolled.

The purpose of this registry is to support studies aimed at determining the cause of the ichthyoses and treating them more effectively. The Registry offers blinded information about well-characterized groups of individuals with specific diagnoses for study by skin biologists, pharmacologists, and others. The Registry also provides information about research projects to those that have enrolled in the Registry and expressed an interest in participating in studies. Participants enrolled in the Registry by contacting the registry officials. All participants participated in a phone interview with the study research nurse. Participants were asked about diagnostic testing, treatments, birth history, medical history, degree and type of involvement, current physical condition, and other family members with skin disorders. A quality of life index was embedded in the interview. Participants also indicated whether they would like to be contacted about participating in clinical research. This information was complemented by an enrollment form from the enrollee's caregiver. Diagnosis was confirmed by specific criteria based on clinical involvement, review of histology, and where appropriate, serum cholesterol sulfate determination or DNA analysis.

Official TitleResearch Registry for Inherited Disorders of Keratinization 
NCT00074685
Principal SponsorNational Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Last updated: January 18, 2026
Sourced from a government-validated database.Claim as a partner

Protocol

This section provides details of the study plan, including how the study is designed and what the study is measuring.
Design Details

610 patients to be enrolled

Total number of participants that the clinical trial aims to recruit.

Cohort

These studies follow a group of individuals with common characteristics (such as a condition or birth year) over a specific period to study health outcomes or exposures.


Eligibility

Researchers look for people who fit a certain description, called eligibility criteria: person's general health condition or prior treatments.
Conditions
Criteria

Any sex

Biological sex of participants that are eligible to enroll.

Healthy volunteers not allowed

If individuals who are healthy and do not have the condition being studied can participate.

Conditions

Pathology

Congenital AbnormalitiesIchthyosisInfant, Newborn, DiseasesKeratosisDarier DiseaseMetabolic DiseasesMetabolism, Inborn ErrorsSkin AbnormalitiesSkin DiseasesSkin Diseases, VesiculobullousSkin Diseases, GeneticIchthyosiform Erythroderma, CongenitalIchthyosis, X-LinkedPemphigus, Benign FamilialHyperkeratosis, EpidermolyticIchthyosis, LamellarGenetic Diseases, InbornGenetic Diseases, X-LinkedSteroid Metabolism, Inborn Errors

Criteria

Note: Accrual into this study has been discontinued as of 03/31/04. Inclusion Criteria: * Diagnosis of one of the ichthyoses, erythrokeratodermas, Darier disease, Hailey-Hailey disease, palmar-plantar keratodermas, pachyonychia congenita, extensive epidermal nevi, or related disorder Exclusion Criteria: * Ichthyosis Vulgaris

Study Centers

These are the hospitals, clinics, or research facilities where the trial is being conducted. You can find the location closest to you and its status.

This study has 1 location

Suspended

University of Washington

Seattle, United StatesSee the location
CompletedOne Study Center