Pachyonychia Congenita Management

Introduction

Pachyonychia Congenita is a rare genetic disorder characterized by thickened skin and nails, requiring a comprehensive management approach, as recommended by the American Academy of Dermatology and British Journal of Dermatology 1, 2

The American Academy of Dermatology recommends applying mid to high potency topical steroid ointments to nail folds twice daily, daily dilute vinegar soaks (50:50 dilution) to nail folds twice daily for 10-15 minutes, and topical 2% povidone-iodine for antiseptic effects to manage nail dystrophy 1
Topical povidone iodine 2% can be used for paronychia treatment, and topical antibiotics can be applied if infection is suspected 3

Daily lukewarm baths (30 min or more) can soften hyperkeratotic skin, and gentle removal of scales using sponges, microfiber cloths, or pumice stone can be effective, according to the British Journal of Dermatology guidelines 2
Applying emollients and keratolytics after bathing, such as moisturizing additives, colloidal preparations, baking soda (3-6 g/L), or saltwater baths (0.9% normal saline), can help manage hyperkeratotic skin 2
Keeping hands and feet dry, trimming nails straight across, and wearing properly fitting shoes with adequate toe box are recommended to prevent paronychia and ingrown toenails 3, 4, 5
Applying petrolatum around the nails for lubrication is also suggested 6

Acitretin is the main systemic retinoid used for severe hyperkeratotic disorders, effective for removing scales and thinning hyperkeratosis, but dosage should be determined by a dermatologist experienced in its management, as recommended by the British Journal of Dermatology 2
Caution should be exercised when using oral retinoids due to potential side effects and teratogenicity, as warned by the British Journal of Dermatology 2

Nail splitting or removal is necessary in cases of severe and/or painful nail lesions, pressure hematoma, subungual abscess, or significantly detached nails causing pain or showing signs of infection, according to the Annals of Oncology guidelines 3
The American College of Oncology recommends assessing nail injury to determine the extent of nail detachment, checking for signs of infection, evaluating pain level and functional impairment, and assessing for subungual hematoma, with specific indications for splitting/removal including Grade 2 or higher onycholysis, painful subungual hematoma, and signs of infection beneath the nail plate 3
Partially detached nails should be completely removed if significantly detached or causing pain, followed by cleaning and culturing the nail bed, and applying appropriate antibiotics if infection is suspected, according to the Annals of Oncology guidelines 3
Taxane-induced onycholysis requires partial or total nail avulsion for Grade 2 treatment with painful hematoma or subungual abscess, and interrupting anticancer treatment for Grade 3 or intolerable Grade 2 reactions 3

Referral to podiatry for management of severe nail dystrophy may be necessary, and regular follow-up is essential to monitor treatment response and adjust therapy as needed, according to the British Journal of Dermatology guidelines 2
Avoiding iodine-based antiseptics in long-term use, topical steroids if infection is present, and prolonged use of high-potency topical steroids on the face is recommended, as advised by the American Academy of Dermatology and British Journal of Dermatology 1, 2
Patient-specific factors, such as diabetes or immunocompromised status, and oncology patients on taxane therapy with onycholysis, should be considered when determining the need for nail splitting or removal 3
Diabetic patients with toenail injuries require more vigilant monitoring and earlier intervention due to increased risk of infection, and early referral to a foot care specialist is recommended 7
Patients should be reviewed within 48-72 hours if there are signs of infection, and otherwise, follow up in 1-2 weeks to ensure proper healing 8

Permanent nail deformity, secondary infection, and onycholysis becoming permanent, are potential complications to avoid, highlighting the importance of proper management and follow-up 3
Permanent nail deformity can occur if the matrix is damaged during toenail removal, and secondary infection and permanent nail deformity can occur if the hematoma places prolonged pressure on the nail matrix 3, 5
Onycholysis (nail separation) may become permanent if not properly managed 3
Post-surgical care should include topical antibiotics if indicated, regular dressing changes, and elevation and limited weight-bearing initially 4, 3