• Skip to main content
  • Skip to primary sidebar
  • Skip to footer

Pachyonychia Congenitia Project

Fighting for a cure. Connecting & helping patients. Empowering Research.

Donate
VIEW MENUMENU
  • Pachyonychia Congenita
        • What Is Pachyonychia Congenita?
          • Could I Have PC?
          • Getting Diagnosed
          • Genetics
          • Image Library
          • Glossary
          • Brochures
        • Patient Registry (IPCRR)
          • Patient Registry Data
        • Understanding my Genetic Testing Results
          • PC-K6a
          • PC-K6b
          • PC-K6c
          • PC-K16
          • PC-K17
          • Other Conditions With Painful Palmoplantar Keratoderma
        • Managing and Caring For PC
          • Tips and Tools
          • Living With PC
          • PC & Children
          • Working With Medical Professionals
          • Family & Friends: How To Help
          • Video Library
  • For Professionals
        • For Professionals
          • International PC Consortium
          • Pharmaceutical Partners
          • Refer a Patient
          • Patient Registry (IPCRR)
            • Patient Registry Data
            • Other Conditions With Painful Palmoplantar Keratoderma
          • Advocacy Partners
        • PC Research
          • Apply for a Grant
          • Awarded Grants
          • Published Research Articles
          • Clinical Trials
        • Patient Voices
          • Quality of Life
          • Patient Stories
          • Patient-Focused Drug Development Meeting
  • Ways To Give
        • Ways You Can Give
          • Donate
          • Donate Just Giving UK
          • PC Love Builders
          • Legacy Society (Planned Giving)
          • Combined Federal Campaign
          • Giving Stock
        • Raise Awareness
          • How You Can Help
          • Fundraising Ideas
          • Share Your Story
          • PC Advocates
          • Volunteer
  • News & Events
        • News
          • Current Newsletter
          • Newsletter Archives
          • Sign up for our Newsletter
        • Events
          • Patient Support Meetings
          • IPCC Meetings
          • Video Library
  • About Us
        • About PC Project
        • Medical and Scientific Advisory Board
        • Financial & Legal Information
        • Contact Us

Other Medications & Procedures

November 5, 2016 by Pachyonychia

Below is a list of other medications and procedures that PCers have tried.

Botulinum Toxin and PC

(Botox, Dysport)

COMMENTS: “No one fully understands why Botulinum toxin works for PC at all. It helps people with hyperhydrosis (too much sweat), and so the thought is that it reduces the sweat and may, therefore, reduce the blistering. It doesn’t affect what is really going on with the keratin genes. There is no information on the amount of the drug needed for PC injections versus that used for cosmetic (wrinkles) or other treatments. As far as we know, there is no published information on how the drug is stored or retained in the body following treatment for PC. There are studies for other uses of the drug.”

COST: In Sweden, you pay a yearly fee and then everything is free. In the USA, the cost estimate is over $100,000 for a single treatment because of the need to have a general anesthetic administered in a hospital.

Drug Data

Wikipedia has an excellent article on Botulinum toxin, its purpose, and other uses.

Botox — Allergan, Inc.; [1]
Also see wikipedia article on Allergan

Dysport — Ipsen ispen.com
Dysport website

US National Institutes of Health Articles:
Botox
Dysport

Best Practices

We have learned there is a specific way that works best for Botox. Including:

  • It is not possible to do the injections with only cryosthesia because the pain for PCers is horrible. The injecting physician must employ nerve blocks before injecting the toxin.
  • The dose should be 200 per foot.
  • Other measurements will need to take place.

The following is the best treatment protocol to use for those with PC. The last author has a lot of experience working with botulinum toxin and PC. If your dermatologist would be willing to be instructed, we can connect them.

Title: A treatment protocol for botulinum toxin injections in the treatment of pachyonychia congenita-associated keratoderma.
Journal: Br J Dermatol Authors: Koren A, Sprecher E, Reider E, Artzi O

Abstract:

BACKGROUND:

Severely debilitating plantar keratoderma pain is the most distressing clinical feature of pachyonychia congenita (PC). Several earlier publications reported therapeutic success with botulinum toxin (Btx) plantar injections.

OBJECTIVES:

To describe our 4-year experience during which we administered a total of 30 plantar Btx injections to five PC patients following an optimized protocol.

METHODS:

Five patients with PC (age 21-54 years) who were treated at our medical center from 4/2015 to 6/2018 were included in the study. After an ultrasound-guided nerve block performed by an anesthesiologist, the patients received plantar intradermal injections of Btx A. To ascertain the effect of the treatment, we used a dedicated quality-of-life questionnaire for PC patients (PCQoL) and asked the patients to evaluate the intensity of eight parameters pertaining to their symptoms at baseline and before every treatment session. At study closure, patients were asked to evaluate the maximal improvement in the same eight parameters throughout the study period.

RESULTS:

All patients demonstrated a decrease in PCQoL scores during the follow-up period. All patients showed a significant improvement in PCQoL after the first treatment session and at the last evaluation (P = 0.043). The best improvement scores concerned morning feet burning and long-distance walking (> 500 m). The scores were significantly lower if the intervals between Btx injections were <100 days.

CONCLUSIONS:

Btx treatment of PC-associated keratoderma following an optimized protocol leads to a major change in patients’ quality of life.

Other Patient Experiences

K6a patient: “I have had the treatments every 2-1/2 to 3 months for the past 2 years. It is not 100% (sometimes works better than other times). One time, the anesthesia didn’t work and it was terribly painful. From that time on, I was put to sleep (general anesthesia) to have the treatment because it hurts too much otherwise. My sister also has the treatments, but cannot use the general anesthesia as it makes her sick. My sister waited a little longer this last time as it is so painful to have the treatments. Our cousin has chosen not to have the treatments.

Side Effects: I cannot read fine print very well, my eye doctor thinks I am too young to have this condition and they think it could be from the Botox treatments as my sister is also having this same effect.”

K17 patient: “Here is some information, from my point of view. There are 3 people in my family who have been treated. My sister and two others have done the treatment several times. I have only done it once. It lasted for about 6 months for me, but it took about 3 weeks before I felt any difference in the feet.

I had to start the treatment with only one foot, and the second foot was treated a little more than one month later. The good thing with that was that you could actually SEE the difference: The treated foot had less blisters than the untreated foot!

The treatment was no miracle. My feet didn’t become painless, but it sure reduced the sweat (the feet got really dry…). It took a longer time before I reached the worst “pain level” after a day of work. I got less blisters and my feet produced less calluses during the time the (drug was) in the feet.

And the needles… in Sweden you get anesthetic. There are two types, either you get full anesthetic (you’re “asleep”) or you get a local one, where they block the ankles and the feet are pointed upwards. I did the last one, and I thought that was the worst part of the treatment! Next time I do the treatment I would rather have the full anesthetic, but the good thing with the ankle-anesthetic is that it is possible to walk afterwards without any problems!

(This) is a drug and it might not be the best solution for the body, but eating painkillers all of your life is not good either.”

Early Studies

Although other patients with conditions ‘like’ PC have been included in some treatments, we know of only a few patients with confirmed PC who have been treated. Five were treated in Sweden and one was treated in Ireland. In the study in Sweden, Dysport or Neurobloc (not Botox) was used. We do not know what product was used in Ireland.

From the PC Research Agenda:

Several mechanisms have been suggested to underlie the beneficial effect of botulinum toxin (BTx) in PC. First, BTx has been shown to prevent fluid accumulation within blisters located underneath PC patients’ calluses (1). Second, BTx may block neurotransmitter release by type C fibers and thereby reduce pain (2). Third, BTx may inhibit vasodilation and neurogenic inflammation (3). Unpublished data also show that BTx reduces epidermal thickness in differentiating 3 dimensional skin models (Fried et al, unpublished). To the best of our knowledge, more than 20 PC patients have been treated with plantar injections of BTx with marked improvement in pain and functionality in most cases and varying degrees of impact on callosities. A protocol has been published for clinical use (3).

  1. Swartling C, Karlqvist M, Hymnelius K, Weis J, Vahlquist A. Botulinum toxin in the treatment of sweat-worsened foot problems in patients with epidermolysis bullosa simplex and pachyonychia congenita. Br J Dermatol. 2010 Nov;163(5):1072-6.
  2. Gazerani P, Pedersen NS, Staahl C, Drewes AM, Arendt-Nielsen L. Subcutaneous Botulinum toxin type A reduces capsaicin-induced trigeminal pain and vasomotor reactions in human skin. Pain. 2009 Jan;141(1-2):60-9
  3. Koren A, Sprecher E, Reider E, Artzi O. A treatment protocol for botulinum toxin injections in the treatment of pachyonychia congenita-associated keratoderma. Br J Dermatol. 2020 Mar;182(3):671-677

Filed Under: Care, Medications, Topicals, & Treatments

Primary Sidebar

PC Resources

  • Features of PC
    • Calluses (Keratoderma)
      • Blisters
      • Cracks
      • Itch
      • Neurovascular Structures
    • Cysts
    • Ears
    • Follicular Hyperkeratosis
    • Mouth, Tongue, & Larynx
    • Nails
    • Pain
  • Tools, Aids, & Footwear
    • Shoes, Socks, & Insoles
    • Walking Aids
    • Hand Tools
    • Power Tools
  • Medications & Topicals
    • Oral Medications
    • Topical Medications
    • Other Medications & Procedures
  • Miscellaneous Questions & Answers
  • PC & Children

Footer

PC Project logo in white
PACHYONYCHIA CONGENITA PROJECT

P.O. Box 17850
Holladay, UT 84117

info@pachyonychia.org

801-987-8758

  • Facebook
  • Instagram
  • LinkedIn
  • Twitter
  • YouTube

Sign up for our Newsletter

Loading

Support PC Project

There are many ways you can help improve the lives of those who suffer from Pachyonychia Congenita.

Learn More
  • Contact Us
  • About Us
  • Donate
  • Patient Registry

Copyright © 2023 PC Project. All rights reserved. Pachyonychia Congenita Project is a 501(c)(3) under federal tax guidelines. Using this site means you accept its terms as outlined in the disclaimer and privacy policy.