Acrokeratosis Verruciformis

Acrokeratosis Verruciformis
Acrokeratosis Verruciformis
Acrokeratosis Verruciformis

Acrokeratosis Verruciformis (AKV) is a rare genetic skin disorder that causes wart-like (verrucous) lesions, typically on the hands, feet, elbows, knees, and forearms. It is usually inherited in an autosomal dominant pattern and caused by mutations in the ATP2A2 gene, which is also involved in Darier disease. Lesions are generally benign, but they can be cosmetically distressing or prone to secondary infection or irritation.

🔬 Overview of Treatment

There is no definitive cure for AKV, but several treatment options are available to manage and reduce the appearance of lesions.


🔹 1. Topical Treatments

These are often first-line therapies for mild or localized cases:

  • Topical retinoids (e.g., tretinoin, adapalene)

    • Help normalize keratinization and reduce hyperkeratotic lesions

  • Salicylic acid (keratolytic agent)

    • Helps exfoliate thickened skin

  • Urea creams (keratolytic and moisturizing)

    • Help reduce scaling and soften skin

  • 5-fluorouracil cream

    • Occasionally used for persistent lesions due to its antiproliferative effects


🔹 2. Systemic Treatments

For widespread or resistant lesions:

  • Oral retinoids (e.g., acitretin, isotretinoin)

    • Effective in reducing keratinization

    • Require careful monitoring for side effects (e.g., liver function, lipid profile)


🔹 3. Procedural Treatments

Used for isolated or bothersome lesions:

  • Cryotherapy (liquid nitrogen)

    • Freezes and destroys the lesion

  • Laser therapy (e.g., CO₂ laser, Er:YAG)

    • Ablative lasers can remove thickened lesions with good cosmetic outcomes

  • Electrosurgery or curettage

    • Physical removal, usually reserved for stubborn or solitary lesions


🔹 4. Photodynamic Therapy (PDT)

  • A newer option with limited but promising results

  • Involves applying a photosensitizer followed by light exposure to target abnormal cells


🔹 5. Genetic Counseling

Since AKV is often inherited, family screening and genetic counseling are recommended, especially in familial cases.


🔹 6. Monitoring & Follow-up

  • Long-term dermatologic follow-up is important

  • Rare reports of squamous cell carcinoma arising in AKV lesions suggest a need for vigilance, especially if lesions change or ulcerate


⚠️ Note:

  • Treatment results can be variable, and relapse is common.

  • A dermatologist should tailor treatment based on the severity, location, and symptoms.

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