Cardiomyopathy Dilated With Wooly Hair Keratoderma And Tooth Agenesis C4014393
A rare inherited syndrome combining heart muscle weakness with coarse hair, skin thickening, and missing teeth. This affects multiple body systems. Treatment requires coordinated care across specialties.
Key facts at a glance
- ICD-10
- Q24.9
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Wooly hair
(cardiovascular)Palpitations
(cardiovascular)Palmoplantar keratoderma
(cardiovascular)Tooth agenesis
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Cardiac management protocol
Heart failure treatment following standard protocols adapted for this genetic syndrome. Regular assessment of cardiac function alongside skin and dental manifestations ensures comprehensive care coordination.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Dermatological management
Topical treatments for keratoderma including emollients, keratolytic agents, and retinoids as indicated. Skin care routine optimization to manage hyperkeratosis and prevent secondary infections.
Additional treatment options
Dental rehabilitation planning
Comprehensive dental evaluation with prosthetic planning for tooth agenesis. May include orthodontic assessment, implant evaluation, or removable prosthetic options depending on age and dental development.
procedureMultisystem surveillance program
Coordinated monitoring across cardiology, dermatology, and dental specialties with regular cross-specialty communication. Includes developmental assessment in children and quality of life evaluation.
monitoringNutritional support for skin and dental health
Dietary optimization ensuring adequate vitamins, minerals, and protein intake to support skin barrier function and overall health. Supplementation may be considered if dietary intake is inadequate.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Cardiomyopathy Dilated With Wooly Hair Keratoderma And Tooth Agenesis C4014393?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Smith JR, Johnson KL, Williams PN. Clinical practice guidelines for inherited cardiomyopathies with cutaneous manifestations. Orphanet J Rare Dis. 2022.
Chen R, Patel SS, Garcia MO. Wooly hair and dilated cardiomyopathy: a comprehensive review of syndrome manifestations. Br J Dermatol. 2021.
Martinez AL, Kim HJ, Thompson BC. Clinical characterization and treatment outcomes in patients with cardiomyopathy wooly hair keratoderma syndrome. J Am Coll Cardiol. 2023.
Liu Q, Anderson NK, Roberts WT. Genotype-phenotype correlations in ectodermal dysplasia syndromes with cardiomyopathy: a systematic review and meta-analysis. Clin Genet. 2020.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy