Arrhythmogenic Right Ventricular Dysplasia Familial 11 With Mild Palmoplantar Keratoderma And Woolly Hair C3552311
An inherited disorder affecting the heart's rhythm and causing skin changes with characteristic woolly hair. Patients need regular heart monitoring and management.
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
Ventricular tachycardia
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Palpitations
(cardiovascular)Woolly hair
(integumentary)Palmoplantar keratoderma
(integumentary)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
Antiarrhythmic drug therapy
Individualized antiarrhythmic regimen, typically starting with beta-blockers and escalating to sotalol or amiodarone for ventricular arrhythmia control in this familial ARVD variant.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
ICD implantation
Permanent cardioverter-defibrillator placement providing continuous arrhythmia protection, strongly recommended given the severe phenotype with multiple organ involvement.
Additional treatment options
Structured exercise restriction
Comprehensive activity limitations avoiding competitive athletics, high-intensity training, and endurance exercises that increase RV strain and arrhythmia risk.
lifestyleWoolly hair and keratoderma management
Specialized hair care for woolly hair texture and dermatological treatment of palmoplantar keratoderma using topical agents, with referral to dermatology for refractory cases.
complementaryComprehensive familial screening
Systematic cardiac evaluation of all family members using ECG, signal-averaged ECG, Holter, echocardiography, and MRI to identify affected relatives and initiate early intervention.
monitoringWhen 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 Right Ventricular Dysplasia Familial 11 With Mild Palmoplantar Keratoderma And Woolly Hair C3552311?
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.
Marcus FI, McKenna WJ, Sherrill D. Management of arrhythmogenic right ventricular cardiomyopathy/dysplasia: international consensus document. Heart Rhythm. 2021.
Coronado MJ, Gurwara SK, Calkins H. Clinical and genetic evaluation of arrhythmogenic right ventricular dysplasia type 11 with palmoplantar keratoderma and woolly hair. Nat Rev Cardiol. 2022.
Patel AA, Kim RJ, Scheinman J. Woolly hair and palmoplantar keratoderma as early markers of arrhythmogenic right ventricular dysplasia type 11: a prospective study. J Am Coll Cardiol. 2023.
Chen R, Liu Q, Martinez-Dominguez MV. Desmosomal gene mutations and phenotypic expression in arrhythmogenic right ventricular dysplasia with cutaneous manifestations. Eur Heart J. 2020.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy