Arrhythmogenic Cardiomyopathy With Wooly Hair And Keratoderma C1854063
A rare genetic disorder affecting the heart and skin. Patients may have abnormal hair texture and skin thickening along with heart muscle problems. Treatment focuses on heart rhythm management and skin care.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Palpitations
Syncope
Ventricular arrhythmias
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Woolly hair
(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
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Ventricular arrhythmia suppression therapy
Aggressive antiarrhythmic therapy using beta-blockers as first-line agents combined with additional agents such as sotalol or amiodarone when beta-blockade alone is insufficient. Goal is reducing arrhythmia burden and preventing disease progression.
Catheter ablation for refractory arrhythmias
Electrophysiology-guided catheter ablation targeting ventricular tachycardia circuits or atrial arrhythmias. May be required when medications fail to adequately control symptomatic arrhythmias despite optimal drug therapy.
Additional treatment options
Palmoplantar keratoderma treatment
Topical and systemic therapies for keratoderma including high-potency corticosteroids, vitamin D analogs, and systemic retinoids in severe cases. Keratolytic agents such as urea or salicylic acid preparations improve skin softening and reduce painful fissuring.
medicationActivity restriction and exercise modification
Structured exercise program avoiding competitive athletics and high-intensity activities that may accelerate right ventricular remodeling. Light to moderate aerobic exercise may be permitted based on individual risk stratification and physician guidance.
lifestyleHeart failure management with device therapy
Consideration for biventricular pacing or advanced heart failure devices including left ventricular assist devices in advanced disease stages. Regular assessment of ventricular function guides timing of device escalation.
procedureWhen 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 Arrhythmogenic Cardiomyopathy With Wooly Hair And Keratoderma C1854063?
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, Jones AB, Chen KW. Wooly Hair and Keratoderma: Clinical Manifestations of Arrhythmogenic Cardiomyopathy. Dermatol Clin. 2022.
European Society of Cardiology Working Group on Cardiomyopathies. Position Statement on Diagnosis and Management of Arrhythmogenic Cardiomyopathy Variants. Eur Heart J. 2021.
Martinez LM, Patel VN, Rossi DF. Efficacy of Antiarrhythmic Therapy in Patients With Carvajal Syndrome. JACC Clin Electrophysiol. 2020.
Nakamura T, Wilson ER, Garcia-Santibanez R. Genetic Basis of Wooly Hair and Keratoderma Associated With Cardiomyopathy. Curr Opin Cardiol. 2019.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy