Keratoderma with Woolly Hair Type II

Keratoderma with woolly hair type II is a rare inherited “cardio-cutaneous” syndrome. It affects the skin, hair, and heart. Babies are usually born with tightly curled, woolly scalp hair. During the first years of life they develop palmoplantar keratoderma (thick, rough skin on the palms and soles). In later childhood, the teen years, or young adulthood, many develop a left-sided dilated or arrhythmogenic cardiomyopathy—a disease of the heart muscle that can cause heart enlargement, weakness, rhythm problems, fainting, heart failure, or sudden cardiac death if untreated. The condition is most often caused by harmful variants (mutations) in the DSP gene (desmoplakin), a critical protein that ties cells together in the skin and heart through structures called desmosomes. When desmoplakin does not work correctly, skin blisters or thickening and heart muscle scarring can occur, especially under physical stress. orpha.net+2PMC+2

Keratoderma with woolly hair type II—better known as Carvajal syndrome—is a rare inherited condition that affects the skin, hair, and heart. Babies typically have very tightly curled (“woolly”) scalp hair from birth. During the first year of life, thick skin builds up on the palms and soles (palmoplantar keratoderma). As children grow, some develop a serious heart problem called dilated left-dominant arrhythmogenic cardiomyopathy, which can cause irregular heartbeats, heart weakness, and, rarely, sudden cardiac death without treatment. The root cause is usually a change (variant) in the DSP gene (desmoplakin), a key protein that ties skin and heart cells together via desmosomes. When desmosomes are weak, skin and hair become fragile, and heart muscle is more prone to scarring and rhythm problems. Most families inherit it in an autosomal recessive pattern. orpha.net+3PMC+3MedlinePlus+3

Although the classic inheritance is autosomal recessive (both copies of the gene altered), some families show autosomal dominant patterns (one altered copy) with overlapping features. The heart phenotype is often left-dominant arrhythmogenic cardiomyopathy (LDAC) with distinctive scarring on cardiac MRI. PMC+1

Other names

• Carvajal syndrome

• Dilated cardiomyopathy with woolly hair and keratoderma (DCWHK)

• Desmoplakin-related cardio-cutaneous syndrome

• Keratoderma with woolly hair, type 2 (KWWH2)

• Left-dominant arrhythmogenic cardiomyopathy with PPK and woolly hair (DSP-cardiomyopathy with PPK) VisualDx+1

Why this happens

Desmoplakin anchors the inside of the cell to the desmosome, a cell-to-cell “rivet” that lets tissues resist stretching. Skin (palms/soles) and heart muscle are constantly under mechanical stress. DSP variants weaken these rivets, so cells detach or die under stress. In the skin, repair leads to thick keratin layers (keratoderma). In the heart, repeated injury triggers inflammation and fibrous/fatty scarring, especially in the left ventricle. Scar tissue conducts electricity poorly, causing arrhythmias; it also makes the heart pump less effectively, causing cardiomyopathy. Exercise and infections may precipitate “hot-phase” episodes that mimic myocarditis. PMC+2PMC+2

Types

1. Classic recessive Carvajal (KWWH type II): woolly hair at birth, palmoplantar keratoderma in infancy, left-sided dilated/arrhythmogenic cardiomyopathy in later childhood/teens. orpha.net

2. Autosomal dominant DSP-cardiomyopathy with skin/hair features: similar but may be milder or variable; sometimes dental anomalies (hypo/oligodontia) are present. Medical Journals

3. Left-dominant arrhythmogenic cardiomyopathy (LDAC) due to DSP: prominent left-ventricular fibrosis on MRI, arrhythmias, chest-pain “hot phases”; skin/hair signs may be subtle or absent. PMC+1

4. Cardio-cutaneous spectrum with overlapping desmosomal genes: biology overlaps with Naxos disease (JUP) and DSC2-related disease; type II refers specifically to DSP/Carvajal. PMC+1

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Causes

Note: The root cause is a pathogenic variant in DSP; the items below include genetic drivers and common triggers/worseners of skin/heart problems in this syndrome.

1. Pathogenic variants in DSP (desmoplakin): the fundamental cause; truncating or missense changes disrupt desmosomes. PMC

2. Autosomal recessive inheritance: both parents carry one altered DSP copy; child inherits both. orpha.net

3. Autosomal dominant inheritance: a single altered copy can be enough in some families. PMC

4. Variants affecting the C-terminal tail of desmoplakin: impair filament anchoring → fragile tissues. PMC

5. Variants affecting the “head” domain: destabilize desmosome assembly. PMC

6. Mechanical stress (endurance exercise): increases cell injury in heart/skin; can hasten fibrosis/arrhythmias. PubMed

7. Adolescence growth spurts: higher mechanical load unmasks cardiomyopathy. PubMed

8. Febrile viral illnesses (myocarditis-like “hot phases”): trigger chest pain, troponin rise, and new scars. PMC

9. Frequent tachyarrhythmias: worsen heart muscle function over time. PubMed

10. Pregnancy (hemodynamic stress): may reveal latent cardiomyopathy. PubMed

11. Hypertension (added wall stress): speeds remodeling in a vulnerable myocardium. PubMed

12. Alcohol binges: pro-arrhythmic and cardiodepressant effects aggravate risk. PubMed

13. Electrolyte imbalance (low potassium/magnesium): lowers arrhythmia threshold. PubMed

14. Certain QT-prolonging drugs: can provoke dangerous rhythms in scarred myocardium. PubMed

15. Dehydration/heat stress: raises heart rate and irritability of myocardium. PubMed

16. Severe emotional stress: catecholamine surges increase arrhythmia risk. PubMed

17. Skin friction/pressure on palms/soles: promotes callus thickening (keratoderma). (Clinical consensus)

18. Harsh detergents/solvents: irritate already fragile skin barrier. (Dermatology practice consensus)

19. Consanguinity in high-risk regions: increases recessive disease likelihood. orpha.net

20. Coexisting desmosomal gene variants (rare): can modify severity. AHA Journals

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Symptoms

1. Woolly hair from birth: tight, fine curls; hair may be fragile. orpha.net

2. Thick skin on palms/soles (keratoderma): rough, yellowish, painful cracks. orpha.net

3. Blisters or fissures on hands/feet: after friction or heat. orpha.net

4. Foot pain with walking or standing: from thick, cracked skin. (Clinical)

5. Early fatigue or shortness of breath on exertion: heart pump weakness or arrhythmia. PMC

6. Chest pain episodes (“hot phases”): look like myocarditis; troponin may rise. PMC

7. Palpitations (pounding or fluttering heartbeat): due to ventricular arrhythmias. PubMed

8. Lightheadedness or fainting (syncope): from fast or unstable rhythms. PubMed

9. Swelling of legs or abdomen: signs of heart failure in advanced disease. PubMed

10. Reduced exercise tolerance: early functional clue. PubMed

11. Irregular pulse found by smartwatch/monitor: extra beats or runs. (Clinical)

12. Family members with sudden cardiac death or cardiomyopathy: inherited risk clue. PubMed

13. Cracks around fingers/heels: painful, may get infected. (Dermatology consensus)

14. Toenail or fingernail thickening (sometimes): from chronic keratoderma stress. (Clinical)

15. Anxiety around exertion: due to prior palpitations/fainting; important psychosocial symptom. (Clinical)

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Diagnostic tests

Physical examination

1. Full skin exam (palms/soles/hair): confirms woolly hair and keratoderma; looks for fissures, infections, nail changes. This anchors the “cutaneous” part of the syndrome. orpha.net

2. Cardiovascular exam at rest and after walking: checks heart rate, rhythm, murmurs, heart size signs, edema; documents functional class. PubMed

3. Family history three-generation pedigree: sudden deaths, fainting, cardiomyopathy, skin/hair signs; guides genetic testing. PubMed

4. Exercise counseling screen: assesses activity level because high-intensity exercise can worsen cardiomyopathy risk. PubMed

5. Dermatology photography/dermoscopy: baseline images to track severity and response to treatment. (Clinical)

Manual / bedside tests

6. Grip-and-walk tests (6-minute walk): simple functional check for exertional capacity and symptoms. (Clinical)

7. Holter/event monitor placement teaching: patient learns how to capture palpitations; essential in arrhythmic diseases. PubMed

8. Blood-pressure and orthostatic checks: look for instability that can mimic arrhythmias. (Clinical)

9. Foot pressure/orthotic assessment: identifies pressure points that worsen fissures and pain. (Clinical)

Laboratory & pathological tests

10. High-sensitivity troponin during chest-pain “hot phases”: detects injury; in DSP disease, troponin may spike with MRI scar evolution. PMC+1

11. NT-proBNP: gauges heart strain/heart-failure status. PubMed

12. Electrolytes, thyroid, iron studies: find reversible contributors to arrhythmia or heart failure (e.g., low potassium). PubMed

13. Genetic testing panel (DSP and related desmosomal genes): confirms the diagnosis, informs family screening. AHA Journals

14. Skin biopsy with immunohistochemistry/EM (selected cases): can show reduced/altered desmoplakin staining and help rule out other PPKs. PMC

Electrodiagnostic tests

15. 12-lead ECG: looks for T-wave inversion, low QRS voltages, premature ventricular beats; baseline for follow-up. PubMed

16. 24–72-hour Holter or longer ambulatory monitoring: quantifies PVC burden, NSVT, and symptom-rhythm correlation. PubMed

17. Exercise ECG test (in selected, low-risk patients): may provoke arrhythmias; used with caution and expert oversight in ACM. PubMed

Imaging tests

18. Echocardiography: checks chamber size and pump function; looks for left-ventricular dilatation or regional wall motion changes. PubMed

19. Cardiac MRI with late gadolinium enhancement (LGE): hallmark test in DSP disease; shows ring-like subepicardial/mid-wall fibrosis typical of LDAC and tracks scar progression after “hot phases.” PMC

20. Signal-averaged ECG / advanced mapping or EP testing (specialist use): characterizes arrhythmic substrate when planning ICD or ablation. PubMed

Non-pharmacological treatments (therapies & everyday care)

These measures do not replace cardiology/dermatology care. They help reduce skin symptoms, protect the heart, and lower rhythm risk. Individual plans should be set by specialists.

1. Regular emollients (moisturizers)
   Daily thick creams/ointments soften thick skin and reduce cracks. Apply after bathing and before sleep. Consistent use limits pain and keeps walking/hand use easier. DermNet®+1

2. Keratolytic skin care at home
   Urea or salicylic-acid preparations (per clinician guidance) gently thin hard skin and relieve pressure. Use cautiously on fissures and avoid overuse in children. DermNet®+1

3. Protective footwear and insoles
   Soft, roomy shoes, gel insoles, and friction-reducing socks lower shear forces on soles, decreasing fissures and pain from keratoderma. BAD Patient Hub

4. Callus debridement by a clinician
   Periodic careful trimming of thick callus by dermatology/podiatry improves comfort and reduces skin cracking and infection risk. Medical Journals Sweden

5. Avoid skin irritants
   Limit harsh soaps; use lukewarm water and fragrance-free cleansers to preserve skin barrier and reduce fissures. BAD Patient Hub

6. Topical occlusion techniques
   Applying emollients/keratolytics under cotton gloves/socks at night increases penetration and softening, improving outcomes. DermNet®

7. Nail care and pressure relief
   Careful trimming, protective gloves, and avoiding repetitive friction reduce painful thickening or nail changes sometimes seen alongside PPK. BAD Patient Hub

8. Activity modification & exercise guidance
   High-intensity endurance/competitive sports can raise arrhythmic risk in desmosomal cardiomyopathy. Structured low-to-moderate exercise plans with cardiology input are safer. JACC

9. Cardiac rhythm surveillance
   Scheduled ECG, Holter/patch monitoring, and echocardiography or cardiac MRI help detect early arrhythmias or ventricular changes so therapy can be started promptly. Oxford Academic

10. Family genetic counseling & testing
    Because most cases are autosomal recessive (and some DSP conditions can be dominant), counseling helps relatives understand carrier risks and plan screening. MedlinePlus

11. School/work accommodations
    Allowing rest breaks for foot pain, flexible footwear policies, and avoiding heat/excess friction reduces symptoms and improves participation. BAD Patient Hub

12. Wound care education
    Prompt cleaning and petrolatum dressings for fissures lower infection risk; see clinicians for spreading redness or fever. BAD Patient Hub

13. Sun/heat management
    Heat and sweating aggravate PPK discomfort. Cool environments, breathable socks, and antiperspirants (medical advice) can help. BAD Patient Hub

14. Psychological support
    Visible hair/skin differences and activity limits can affect mood. Counseling and patient groups help coping and adherence. National Organization for Rare Disorders

15. Vaccinations (routine)
    Keeping routine vaccines up-to-date lowers infection-related cardiac stress; discuss influenza/COVID-19 shots with clinicians. heartrhythmjournal.com

16. Dental and surgical planning alerts
    Tell providers about DSP-related cardiomyopathy so anesthesia and fluids/arrhythmia precautions are used. heartrhythmjournal.com

17. Household safety for syncope risk
    If prone to fainting, use shower chairs, avoid heights alone, and teach family CPR/when to call emergency services. heartrhythmjournal.com

18. Wearable medical ID
    Lists diagnosis, arrhythmia risk, and medications (e.g., beta-blocker); this speeds emergency care. heartrhythmjournal.com

19. Avoid QT-prolonging or pro-arrhythmic drugs
    Pharmacists/clinicians should cross-check new medicines for arrhythmia risk. heartrhythmjournal.com

20. Pregnancy planning
    Pre-pregnancy cardiology review helps assess heart function and plan monitoring during pregnancy and delivery. heartrhythmjournal.com

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Drug treatments

There is no medicine that fixes the gene change. Treatment targets the heart (to prevent arrhythmias/heart failure) and the skin (to reduce pain and cracking). Dosing is individualized—below are common roles, not personal prescriptions.

1. Beta-blockers (e.g., metoprolol)
   Class: β-adrenergic blocker. Purpose: reduce adrenaline effects, suppress ventricular arrhythmias, protect the heart. Mechanism: slows heart rate and reduces excitability. Side effects: fatigue, low blood pressure, dizziness. Timing/Dose: daily, titrated to effect. Evidence underpins use in arrhythmogenic cardiomyopathy (ACM). AHA Journals+1

2. ACE inhibitors (e.g., enalapril)
   Class: RAAS blocker. Purpose: treat/remodel dilated cardiomyopathy. Mechanism: lowers afterload and harmful neurohormonal activation. Side effects: cough, kidney effects, high potassium. Dose: daily as tolerated. AHA Journals

3. ARBs (e.g., losartan)
   Class: RAAS blocker alternative when ACEI not tolerated. Purpose/mechanism: similar cardiac protection. Side effects: dizziness, high potassium. Dose: daily. AHA Journals

4. ARNI (sacubitril/valsartan)
   Class: neprilysin inhibitor + ARB. Purpose: for symptomatic heart failure with reduced EF. Mechanism: enhances natriuretic peptides, reduces RAAS. Side effects: low BP, high potassium. Dose: twice daily, uptitrate. AHA Journals

5. Mineralocorticoid receptor antagonists (e.g., spironolactone)
   Class: aldosterone blocker. Purpose: improve outcomes in HFrEF. Mechanism: antifibrotic, diuretic. Side effects: high potassium, gynecomastia. Dose: daily with labs. AHA Journals

6. Loop diuretics (e.g., furosemide)
   Class: diuretic. Purpose: relieve fluid overload symptoms. Mechanism: increases urine output. Side effects: low potassium, dehydration. Timing: PRN/daily per congestion. AHA Journals

7. SGLT2 inhibitors (e.g., dapagliflozin)
   Class: SGLT2 inhibitor. Purpose: reduces HF hospitalizations and improves symptoms in HFrEF. Mechanism: natriuresis, metabolic effects. Side effects: genital infections, volume depletion. Dose: daily. AHA Journals

8. Amiodarone
   Class: antiarrhythmic. Purpose: suppress ventricular arrhythmias when needed. Mechanism: multi-channel blockade. Side effects: thyroid, lung, liver, skin issues—requires monitoring. Dose: loading then maintenance. AHA Journals

9. Sotalol
   Class: class III antiarrhythmic + β-blocker. Purpose: rhythm control in selected patients. Risks: QT prolongation—specialist oversight. heartrhythmjournal.com

10. Mexiletine
    Class: class IB antiarrhythmic. Purpose: adjunct for refractory ventricular ectopy. Risks: GI/neurologic side effects. heartrhythmjournal.com

11. Ivabradine
    Class: If-channel inhibitor. Purpose: rate control in HFrEF when β-blocker inadequate. Mechanism: slows sinus node. Side effects: luminous phenomena, bradycardia. AHA Journals

12. Topical retinoids (e.g., tazarotene)
    Class: vitamin-A derivative. Purpose: thin hyperkeratosis. Mechanism: normalizes keratinization. Side effects: irritation; avoid on cracks. Use: nightly per dermatologist. DermNet®

13. Oral retinoids (e.g., acitretin, isotretinoin)
    Class: systemic retinoid. Purpose: reduce severe, disabling PPK thickness. Mechanism: regulates epidermal differentiation. Side effects: teratogenicity, lipid and liver changes, dryness. Use: specialist-guided courses with lab checks. PubMed+1

14. Topical vitamin D analogues (calcipotriol)
    Class: vitamin-D analogue. Purpose: soften plaques. Mechanism: modulates keratinocyte growth. Side effects: irritation. DermNet®

15. Topical keratolytics (urea/salicylic acid, clinician-approved strengths)
    Class: keratolytic. Purpose: reduce thick scale/fissures. Risks: stinging/over-thinning if overused. DermNet®

16. Topical anti-inflammatories (short courses of corticosteroids for fissure margins/itch)
    Purpose: calm inflammation and pain to aid healing alongside emollients. Risks: skin thinning if overused. PMC

17. Antiperspirants/Botulinum toxin for painful hyperhidrosis (select cases)
    Purpose: reduce sweat-related maceration and friction pain. Note: evidence mainly from PPK case series. Medscape

18. Antibiotics (topical/oral) for secondary infection when indicated
    Purpose: treat cellulitis or infected fissures; not for routine use. BAD Patient Hub

19. Analgesics (acetaminophen first-line)
    Purpose: manage foot/hand pain from fissures; avoid NSAIDs if cardiology advises. heartrhythmjournal.com

20. Electrolyte/magnesium repletion when on diuretics/antiarrhythmics
    Purpose: reduce arrhythmia risk; lab-guided. heartrhythmjournal.com

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Dietary “molecular” supplements

No supplement has been proven to fix the DSP gene or cure Carvajal syndrome. Any supplement should be discussed with your clinicians to avoid drug interactions and arrhythmia risks.

1. Omega-3 fatty acids – may modestly aid cardiovascular health; avoid high doses if bleeding risk. Evidence is general to heart health, not DSP-specific. AHA Journals

2. Vitamin D – low levels are common in chronic skin conditions; supplementation per labs can support general health and may help skin barrier. PMC

3. Urea-based topical therapy – not a dietary supplement but a “molecular” keratolytic; strong clinical role for PPK symptom relief. DermNet®

4. Zinc (if deficient) – correct deficiency that can worsen skin healing; lab-guided only. BAD Patient Hub

5. Iron (if deficient) – supports energy; only if true deficiency to avoid overload. AHA Journals

6. CoQ10 – sometimes used in heart failure adjunctively, but evidence is mixed; discuss with cardiology. AHA Journals

7. Electrolyte solutions – maintain potassium/magnesium balance when on diuretics; dose guided by labs. heartrhythmjournal.com

8. Protein-adequate diet – supports wound healing in cracked skin; dietitian can tailor for heart failure sodium limits. AHA Journals

9. Topical salicylic acid – again not “dietary,” but as a molecular keratolytic it’s highly useful for PPK with correct strength. DermNet®

10. Multivitamin (standard dose) – reasonable if diet is limited; avoid mega-doses that could interact with medications. AHA Journals

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Immunity-booster / regenerative / stem-cell” drugs

No approved stem-cell or gene-editing treatment exists for Carvajal syndrome as of September 23, 2025. Care focuses on established heart-failure/arrhythmia therapies and skin management. Heart transplantation is a life-saving surgical option when end-stage heart failure occurs. Experimental ACM research targets pathways like GSK-3 and desmosomal biology in models, but this is not clinical standard yet. Always be cautious with “regenerative” claims marketed online. Oxford Academic+1

If you see products claiming to “regenerate heart muscle” for Carvajal syndrome specifically, discuss with your cardiologist; current best-evidence care remains guideline-directed HF therapy and arrhythmia prevention. heartrhythmjournal.com

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Procedures / surgeries

1. Implantable cardioverter-defibrillator (ICD)
   Purpose: prevents sudden cardiac death by detecting and stopping dangerous rhythms. Considered when arrhythmic risk is high based on genetics, history, and testing. heartrhythmjournal.com+1

2. Catheter ablation of ventricular arrhythmias
   Purpose: reduce recurrent ventricular tachycardia when drug therapy/ICD shocks persist. It targets scar-related circuits. May need repeat procedures. AHA Journals

3. Cardiac resynchronization therapy (CRT) for select patients
   Purpose: improve pumping when the heart is wide-dyssynchronous and weak. Not everyone qualifies. AHA Journals

4. Heart transplantation
   Purpose: definitive option for refractory end-stage cardiomyopathy despite maximal therapy, improving survival and quality of life. AHA Journals

5. Dermatologic debridement / limited procedures
   Purpose: relieve painful keratoderma areas (mechanical debridement; rarely nail procedures). Not curative—aims to improve function and reduce fissures/infection. Medical Journals Sweden

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Practical prevention & safety tips

1. Build a care team (cardiology + dermatology + genetics). NCBI

2. Sports moderation—avoid high-intensity endurance/competitive athletics without cardiology clearance. JACC

3. Regular cardiac follow-up (ECG/Holter/Echo/MRI as advised). Oxford Academic

4. Prompt infection care for skin fissures to avoid cellulitis/stress on the heart. BAD Patient Hub

5. Emollient routine morning and evening. DermNet®

6. Footwear/sock hygiene to reduce friction/maceration. BAD Patient Hub

7. Medication checks for QT/arrhythmia risk before starting anything new. heartrhythmjournal.com

8. Family counseling/testing for early diagnosis and monitoring. MedlinePlus

9. Heat/sweat control to limit skin breakdown. BAD Patient Hub

10. Emergency plan (ICD card or medical ID; caregivers know when to call for help). heartrhythmjournal.com

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When to see a doctor urgently

• New palpitations, fainting, chest pain, or shortness of breath—these can signal dangerous rhythms or heart failure. Seek emergency care. heartrhythmjournal.com

• Rapid swelling, weight gain, or inability to lie flat—possible fluid overload. AHA Journals

• Painful, red, or draining skin fissures—could be infection needing antibiotics. BAD Patient Hub

• Pregnancy or planning pregnancy—pre-pregnancy cardiology review is important. heartrhythmjournal.com

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What to eat and what to avoid

What to eat:

• Balanced meals with fruits, vegetables, whole grains, legumes, and lean proteins; enough protein helps skin repair. AHA Journals

• Low-sodium choices (read labels); many heart-failure patients target ~2 g sodium/day if advised by clinicians. AHA Journals

• Adequate fluids per cardiology advice; not too much, not too little. Electrolyte-aware if on diuretics. AHA Journals

• Healthy fats (olive oil, nuts, fish with omega-3s). AHA Journals

What to avoid:

• Very salty foods (packaged soups, chips, cured meats) that worsen fluid retention. AHA Journals

• Stimulants (excess caffeine/energy drinks) that may trigger palpitations. heartrhythmjournal.com

• Unverified “regenerative” supplements promised online; they don’t fix DSP and may interact with medications. Oxford Academic

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Frequently asked questions

1. Is Carvajal syndrome the same as Naxos disease?
   No. Both cause woolly hair and keratoderma, but Naxos (type I) is usually right-sided ARVC (JUP), while Carvajal (type II) is left-dominant/dilated ACM (DSP). NCBI

2. How common is it?
   Very rare worldwide; exact prevalence is unknown. MedlinePlus

3. How is it inherited?
   Most families show autosomal recessive inheritance; some DSP conditions can be dominant. Genetic counseling helps clarify risks. MedlinePlus+1

4. What heart problems occur?
   Arrhythmias and left-dominant dilated arrhythmogenic cardiomyopathy with risk of heart failure or sudden cardiac death if untreated. PMC

5. What tests are used?
   ECG, Holter/patch monitoring, echocardiography, and cardiac MRI to look for scarring and function; plus genetic testing. Oxford Academic

6. Can I play sports?
   Discuss with your cardiologist. Competitive/high-endurance sports can raise arrhythmic risk in desmosomal ACM. JACC

7. Are there curative medicines?
   No. Treatment focuses on arrhythmia prevention, heart-failure therapy, and skin care. AHA Journals

8. Who needs an ICD?
   People at higher risk based on arrhythmias, fainting, imaging, or family history; the decision is individualized with specialists. heartrhythmjournal.com

9. What helps the keratoderma?
   Daily emollients, keratolytics, topical retinoids, and in severe cases oral retinoids under specialist care. DermNet®+1

10. Do topical treatments thin the skin too much?
    They can if overused—follow dermatologist guidance and adjust strength/frequency. Medical Journals Sweden

11. Will hair become “normal”?
    Woolly hair texture is usually lifelong. Hair care focuses on gentle grooming to avoid breakage. orpha.net

12. Should relatives be tested?
    Yes—cascade genetic testing and periodic cardiac screening help detect problems early. NCBI

13. What about pregnancy?
    Pre-pregnancy evaluation and close monitoring during pregnancy are advised for people with DSP-related cardiomyopathy. heartrhythmjournal.com

14. Are stem-cell or gene therapies available now?
    Not in clinical practice for this condition as of September 23, 2025; research is ongoing. ScienceDirect

15. Where can I read more?
    See MedlinePlus Genetics for overviews; GeneReviews and specialty cardiology guidelines for management. MedlinePlus+2NCBI+2,

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Last Updated: September 23, 2025.

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