A Review on PRP

From the Editor — On January 4, 2018, a well-documented overview of pityriasis rubra pilaris written by Drs. Dingyuan Wang, Vanessa Cui-Lian Chong, Wei-Sheng Chong and Hazel H. Oon was published online by the American Journal of Dermatology. CAUTION: Section 7 on TREATMENT is worth a careful review by all PRP patients and caregivers.

This scientific article is not written for patients or non-medical caregivers. It should, however, be shared with dermatologists.Just ask: “Have you read the online Review on Pityriasis Rubra Pilaris in the American Journal of Dermatology?”

1   Introduction
2  Clinical Manifestations
3  Pathogenesis
4  Associated Conditions
5  Diagnosis
6  Differential Diagnosis
7  Treatment
8  Conclusion

A Review on PRP

PRP Community Resources

Message from the Editor

The onset of my version of PRP began in early August 2012 with the sudden appearance of a small, red spot on my right temple near my hairline. Within a week the “inconsequential” spot had grown in size — enough to prompt a call to my dermatologist.  By early September the side of my face, from my forehead to jaw, had been engulfed by red.

Initially misdiagnosed with seborrheic dermatitis, it took three months, the clinical observations of a more seasoned dermatologist, and a “supporting” biopsy before an “official”  diagnosis of PRP was rendered. My treatment plan was initiated in early December with slow-paced success. It took another seventeen months before I was med-free and symptom free.

Whether you are a PRP patient or caregiver, you are on a journey that few can understand. There is, however, a PRP community of fellow travelers who have faced the same challenges of body, mind and spirit that you face today. The journey from onset to remission should not be undertaken alone.

Bill McCue


Over the past five years there has been a dramatic expansion of resources available to support PRP patients and their caregivers. The PRP community must become more proactive in building awareness of these resources during 2018among two constituencies:

✽  PRP patients and caregivers

✽  Healthcare professionals responsible for the proper treatment of PRP

As a PRP patient or caregiver it is critical that you understand these resources and share there availability with your own dermatologist, general practitioner, podiatrist, opthamologist, ENT specialist, mental health specialist, etc.

1.  Genetic and Rare Diseases Inforation Center  GARD, a program of the National Center for Advancing Translational Sciences, is funded by the National Institutes of Health. The office supports a variety of programs to serve the needs of patients who have any one of approximately 7,000 rare diseases known today. Their rare disease database includes information on each of 597 rare skin diseases including a recently updated overview of PRP. This is a “Must Read” for every PRP patient and caregiver.

❏  Read and share the GARD PRP Report

2.  National Organization of Rare Disorders     On September 20, 2017, NORD replaced the 1,500-word PRP Report (circa 2007) with a significantly more comprehensive, 5,000-word  2017 revision developed by the PRP Community. This overview is written in lay language and a “Must Read” for every PRP patient and caregiver.

❏  Read and share the NORD PRP Report 

3.  PRP Survival Guide     Since its launch in 2013, this ever-expanding repository of information now includes over 400 PRP-specific articles written in lay language on topics specifically related to the diagnosis and treatment of PRP as reported by PRP patients and their caregivers.

❏  Read “How to use the PRP Survival Guide”

4.  PRP Facebook Support Group     Founded in April 2013 by a PRP patient from British Columbia, Canada, membership in this Closed Group has grown steadily. Today over 1,170 PRP patients and caregivers ask questions, share our PRP-related experiences and provide insights only a fellow sufferer can offer. Our mantra: what works fro one doesn’t work for all. We are in this together.

❏  Join the PRP Facebook Support Group

5.  PRP Community in Rare Connect     Established in April 2015 as an initiative of EURORDIS, the international equivalent of the National Organization of Rare Disorders. the PRP Community on RareConnect is a 200+ member, multi-language, non-Facebook option serving adults with PRP and the parents of children diagnosed with juvenile onset.

❏  Join the PRP Community on RareConnect

6.  PRP Alliance     From a domain name registered in February 2013, the PRP Alliance has evolved into a 501(c)(3), nonprofit, patient advocacy organization with a mission is to advocate the timely and accurate diagnosis of pityriasis rubra pilaris (PRP), the implementation of more effective and accessible treatment options, and an increase in PRP-specific research.

❏  Learn more about the PRP Alliance

On November 1, 2017, as part of the annual observance of PRP Awareness Month, the PRP Alliance executed mailing to the dermatology departments of 88 teaching hospitals in the U.S. This was the first salvo in a year-long Rare Skin Disease Referral Initiative seeking to link PRP patients with the PRP resources listed above.

The Last Word

PRP patients and caregivers who join the PRP community discover that they are not alone. YOU are not alone. WE are on this journey together.

Bill McCue, Editor PRP Survival Guide 1500 Commerce Drive Plano, TX 75093-2640 Email: bill.mccue@prpSurvivalGuide.org Website: prpSurvivalGuide.org

 

2018 Path Forward

                 

When moisturizers cake


Editor’s Note… On November 22, 2017, Sharyn B (Gold Coast, Queensland, Australia) posted the following message to the PRP Facebook Community. Members can CLICK HERE see the original, unedited version of Sharyn’s post and feedback.

“I’ve been told to moisturise at least three times a day. I don’t shower everyday (which is excruciating as i love I love my morning wake up shower). The problem I have now is the moisturiser is caking itself on me and I can’t get it if off with the Cetaphil cleanser I’m using. Any tricks or tips?” Here is the feedback she received.

Steven C — Glenrothes, Scotland, UK
Peanut oil

Vivienne P — Australia
I found being constantly covered in moisturizer was a positive. Showers dried out my skin and I dreaded them. I used products called  Dermol 500 and Zerobase Emollient Cream which are also antibacterial.

Sharyn B — Gold Coast, Queensland, Australia
The moisturiser makes me feel better. It’s the covering left over that I can’t get off to begin again freshly,

Gail C — Virginia Beach, VA
I found just the opposite. Shower for at least 15 minutes, BUT DONT DRY OFF! Apply moisturizer while still wet. It helps immensely

Tonya G — McDonough, GA
Same for me. I showered everyday, warm not hot, short 10 minutes, then Aquaphor all over. Sometimes I’d shower morning and evening during the worst of it. Helped greatly.

Gail C — Virginia Beach, VA
Even though Aquaphor is the nastiest stuff, it sure was a lifesaver for me!

Teri R — Ft. Wayne, IN
Are you wet when you put your clothes on then?

Gail C — Virginia Beach, VA
No. By then I am just damp. Oh, another tip. Go to thrift stores and buy clothes that are too big for you. Wash them before you put them on. The goop we put on our skin will ruin any good clothes you have. But you won’t mind throwing away clothes you pay very little for.

Jean N — Boston, MA
I use CeraVe Moisturizer. I don’t dry off. I apply the moisturizer while I am still wet.

Ginny M — Lexington,  SC
Shower! You need to get the old dead skin off. We shower twice a day when we flare. Do not let it cake up. (Editor’s Note: When Ginny says “We” she refers to herself and her three children, all diagnosed with juvenile onset PRP.)

Jan T — Ringwood, NJ
Mineral oil/baby oil helps remove stickier ointments like Aquaphor. When I cut back on baths and showers (which helped my itching immensely), I also switched to less greasy lotions. I could wash them off with a washcloth or sponge bath.

Pam M — Calgary, Alberta, Canada
I resorted to using a back reaching shower brush. Use it gently all over while in the shower.. Then pat dry and apply moisturizer liberally.

Derrick S — Oak Hills, CA
This thread is a PERFECT example of the PRP problem. No two of us are the same. We just guess at what works. If a shower doesn’t hurt or bother you then do it. If you like to moisturize then do that. In short, if it makes you feel better, do it. I personally think this thing runs its course and you should do what ever you like to be as comfortable as you can during the ride.

Jan T — Ringwood, NJ
That’s basically what my nurse told me.

Anita P
I sponge bath daily and shower once a week mostly to wash my hair. I use Dermasil lotion, mainly because it has lanolin in it. I had a lot of fissures when this all started and I used lanolin for them. I was hoping the lanolin would help keep them away. Dermasil doesn’t have buildup and I used it twice a day in the beginning and now only once a day unless I feel dry or a lot of itching because of being dry. I have the lotion in my car too.

Lorna R — Eugene, OR
I took two showers a day, moisturized well after each one. I do not agree with this no bathing for days on end. Skin, body cream build up, it stinks and it is uncomfortable. Take those showers, do what makes you feel good. This is all about comfort levels. I shampoo’d twice a day too.

Ellen H — Rome, GA
I can’t imagine if I had not bathed everyday what it would have been like, When I would shower everyday I could just rub skin off my body. This is some high maintence stuff

Tierney R — Virginia Beach, VA
I just took a shower on Monday, my first and only one in a month. I did NOT stink. My husband gets very close to me and tells me I do not stink, nor do I smell bad. Showering dries my skin out and is one of the reasons I take pain medicine. I believe showering robs the body of its natural oils. Putting lotion on does me no good, it’s more like a glue that holds the flakes down. We’re not all alike.

Anita P
I agree with every word you said, Tierney. And it’s It’s exhausting just a sponge bath with no soap. Just to wipe a little dust off and add a little moisture layer under the “glue”.

Sharyn B — Gold Coast, Queensland, Australia
Thank you Lorna. I do so enjoy my shower it actually takes my mind of all my woes for a short time

Tasha G — Corvallis, OR
I also bathed twice a day during my acute phase and found it so soothing, both physically and mentally. I have never been a long shower person, but just couldn’t resist when life was super hard due to PRP. I also found Epsom salt baths really helped with the itch. I say do what works best for you and be open to exploring various options. The thing about PRP is that it constantly changes, so what works today may not work tomorrow.

Michele L
My grandmother is having a bad flare up. I have been helping her shower and lathering her up with a psoriasis tar coal shampoo all over her body. Then we moisturize before her skin is fully dry. It stops the burning and severe itching. This condition is slowing killing her. It is so hard to see.

Teri R — Ft. Wayne, IN
How do you put lotion on places you can’t reach on your back?

Gail C — Virginia Beach, VA
Get a small paint roller with removable rollers. And a two-foot handle. Put a healthy glob on your back and roll it all over

Derrick S — Oak Hills, CA
That’s a brilliant idea Gail. Likely less irritating to the skin than rubbing it on.

When moisturizers cake

 

Rare Skin Disease Referral Initiative Update

What is the Basic Problem to be Solved?

When a patient is diagnosed with pityriasis rubra pilaris (PRP), the dermatologist — with very rare exceptions — does not refer their patient to the PRP Alliance, PRP Survival Guide, PRP Facebook Support Group, or the PRP Community on RareConnect. Typically, the patient is referred to the internet where search engines (Google, Yahoo) retrieve information that can be redundant, insufficient, outdated, and written for an audience of healthcare professionals— not patients and their caregivers.

With an estimated 574 rare skin diseases inflicting pain and suffering, it is absolutely unreasonable to expect healthcare professionals to keep track of disease-specific patient support organizations and other patient-centric resources for rare diseases like PRP.

What is the Solution?

The solution is actually quite simple. When a patient is diagnosed with a rare skin disease (less than 200,000 effected patients in the US), the standard therapy should include two referrals that are recorded in the patient’s electronic medical record:

✽  Genetic and Rare Diseases Information Center (GARD)
✽  National Organization of Rare Disorders (NORD)

What Do GARD and NORD Accomplish?

Reliable information about rare or genetic diseases is often hard to find. GARD and NORD offer patients with a rare skin disease — and their caregivers — a reliable roadmap to the resources they need.

✽  GARD website

✽  GARD Rare Disease Database  (SEE GARD PRP Report, Revised 11/2017)

✽  GARD Patient, Families and Friends

✽  GARD Information Specialists — Whether by phone, fax or email, the public has access to current, reliable, and easy-to-understand information in English or Spanish. Toll-free Telephone: 888 205-2311; Email: GARDinfo@NIH.gov/

✽  NORD Website

✽  NORD Rare Disease Database — SEE NORD PRP Report

Step One — Jump Start the Campaign

The Rare Skin Disease Referral Initiative is a year-long campaign that began with the mailing of an “Advocacy Packet” to the following:

TEACHING HOSPITALS

✽  Leadership (Chairs) of the dermatology departments at 88 teaching hospitals in the U.S.

ORGANIZATIONS

✽  American Academy of Dermatology
✽  Society of Dermatology Physician Assistants
✽  Dermatology Nurses’ Association

MEDIA

✽  Dermatology Times
✽  The Dermatologist
✽  Dermatology News

The first salvo of “advocacy packets” was funded by members of the the PRP Facebook Support Group and included the following components. Click the link to see what recipients received.

✽  Cover Letter
✽  Factsheet
✽  GARD brochure
✽  NORD PRP Report — reprinted with permission fs

Step Two — AAD Annual Meeting

The next step in the promotion of the Rare Skin Disease Referral Initiative is to take full advantage of the American Academy of Dermatology annual meeting to be held in San Diego, CA. (February 16-19). The PRP Alliance will be attending the four-day event as a member of the Coalition of Skin Diseases with access to dermatologists, dermatology physician assistants, dermatology nurses and 300-plus exhibitors who sell products and services to dermatologists for patient care.

Step Three — The Hard Work of Advocacy

The third step (March-October, 2018) depends on who will join the effort.

✽  Will it be patients and caregivers who tell their dermatologists about GARD and NORD?
✽  Will AAD, DNA and SDPA tell their members?
✽  Will Dermatology Times, The Dermatologist and Dermatology News publish articles about the Rare Skin Disease Referral Initiative?

For the moment, we will focus on Step 2 and see what can make happen in San Diego.

OTR26 Rare Skin Disease Referral Initiative Update

 

PRP Facebook Membership Milestone

From the Editor…
A milestone is defined as “an event or achievement that marks an important stage in a process.” PRP patients and caregivers know about milestones. We already celebrate important milestones in our journey from onset through remission.

✽  Ability to sweat
✽  Return of fingernails to pre-onset glory
✽  Return of hair
✽  A good night’s sleep
✽  Finding a treatment option that is affordable
 Returning to the workplace

Now it’s time for the PRP Facebook Support Group to celebrates its own milestone. On November 3, 2017 — just three days into PRP Awareness Month — Tim W from Mackinaw, IL became the 1,100th member of the PRP Facebook community.

Think about it. Formed in April 2013 by Jonah Grant-Scarfe (Vancouver Island, British Columbia, Canada), it took the PRP Facebook Community nearly two and a half years to reach 500 (September 6, 2015). Membership milestones have continued unabated.

   600 on March 21, 2016 — 166 days
   700 in early October, 2016 — 199 days
   800 on January 2, 2017 — 88 days
   900 on April 15, 2017 — 103 days
   1,000 on July 10, 2017 — 86 days
   1,100 on November 3, 2017 — 116 days

Predictably, the PRP Facebook population ebbs and flows with flows outpacing ebbs. On average in 2017, it takes three months to add 100 members. At that rate, the 1,200 member milestone  before Rare Disease Day (February 28) and 1,500 “mega” milestone by  PRP Awareness Month (November 2018).

How to Join the PRP Facebook Support Group

For those who are seasoned Facebookers, the PRP Facebook Support Group is relatively easy to find.

   Go to Facebook
✽   Click: “Search for people, places and things”
✽   Type: “Pityriasis Rubra Pilaris (PRP)” including the PRP within the parentheses
✽   Click: “Ask to join”

Eleven Reasons to Join

In March 2014, the members of the PRP Facebook Support Group were asked the question: Why should a PRP patient join the PRP Facebook community share? The 11 reasons listed below were the result of the 19 patients and caregivers who responded. They are just as valid for the PRP Community on RareConnect.

1. To better understand pityriasis rubra pilaris through the collective, first-hand experiences of those who have been afflicted.

We are in this together.

2. To provide — and to receive — encouragement and emotional support.  This is especially important in the absence of an existing support system.

We are in this together.

3. To minimize loneliness and feelings of isolation. To know that we are not alone. We are a community of kindred spirits.

We are in this together.

4. To effectively manage expectations and maximize hope.  We want more than our fair share of good news and are willing to work for it.

We are in this together.

5. To lighten the burden placed on loving family members by sharing that burden with the PRP community.

We are in this together.

6. To recognize that everyone has their own version of PRP and what works for one may not work for another.  We can find effective ways to cope with pain, anxiety, stress, depression and frustration.

We are in this together.

7. To promote a better understanding of our disease within the PRP community by sharing our personal experiences with treatments, dermatologists and other health care professionals.

We are in this together.

8. To promote participation in bona fide research projects designed to improve the diagnosis, treatment and understanding of PRP by the medical community. We must find ways to enlighten dermatologists and other healthcare professionals.

We are in this together.

9. To maintain a core value of acceptance, understanding and empathy.

We are in this together.

10. To vent to each other when our pain is too great,  our frustrations unbearable and our fear overwhelming.

We are in this together.

11. To meet a fellow PRP face to face, in the flesh. Isn’t it about time we hugged?

We are in this together.

Another option to to join the PRP Facebook Support Group is to just CLICK HERE.


Have you completed your PRP census form?

As of November 15, 2017, the PRP Community Database had identified  1,000 PRP patients who are members of the members of the PRP Facebook Community. Unfortunately, only 252 have provided their onset date, onset age and current status (active vs.remission). Please complete the PRP Worldwide Census. LEARN MORE.

 

OTR26 — PRP Facebook Membership Milestone

TJU Announces PRP Center of Excellence


From the Editor…

In an open letter to the PRP Community, Drs. Uitto, Keller and Ross announced the establishment of the nation’s first PRP Center of Excellence, located at Sidney Kimmel Medical College at Thomas Jefferson University Philadelphia, PA. What does this mean to the PRP community? We have asked questions based on the comments from the PRP community and will update accordingly.

“It is with great pleasure that we write to you to announce the first PRP Center of Excellence at the Department of Dermatology & Cutaneous Biology of Thomas Jefferson University in Philadelphia, PA. This Clinic seamlessly integrates laboratory and clinical sciences to improve patient care and research to advance our understanding of this devastating disease.”

 Will there be more that one PRP Center of Excellence at TJU?

 Is the Center of Excellence a clinic or more than a clinic?

 What is the difference between laboratory sciences and clinical sciences?

“At the PRP Center of Excellence, each patient will have a personalized consultation to review the prior diagnoses, biopsies and treatments with the world’s leading clinical dermatologists, dermatopathologists and basic science researchers in the field of dermatology. Your appointment will include whole body photography to document one’s progress during care, interdisciplinary review of diagnostic data (biopsy and other tests), and access to direct enrollment into clinical research.”

 How much time is allotted for the “personalized consultation”?

“Together with one of our clinical dermatologists, treatment decisions at the PRP Center of Excellence will be based on evidence-based algorithms that draw from research and expert opinions of a variety of medical disciplines to provide you with the greatest chance of successfully and safely treating your PRP.”

✽  How will the PRP Center of Excellence facilitate access to pharmaceuticals that have yet to be approved for PRP by the Federal Drug Administration?

In addition, at Thomas Jefferson University, patients have access to the largest network of tertiary referral center services in the region. This is particularly important because PRP is often accompanied by other diagnoses that can affect quality of life, one’s health and treatment decisions. It is this attention to the whole body that will allow you to heal. Specifically, patients can obtain appointments with therapists, psychologists and psychiatrists within the same building as the Dermatology Clinic to seek care for the depression and anxiety that can often accompany PRP. Patients will also be able to make appointments with Will’s Eye Hospital at Thomas Jefferson University for specialized ophthalmology care as the incidence of damaging conditions of the eye, such as ectropion (outward turning of the eyelid) and keratoco unctivitis (severely dry, pain lly irritated eyes), is increased in PRP. In addition, patients can schedule appointments with other nationally recognized fields such as Jefferson cardiology, endocrinology, gastroenterology, hematology, neurology, oncology, nutrition and integrative medicine to manage any accompanying health concerns.

✽  As a matter of official treatment protocol, will the PRP Center of Excellence refer PRP patients to the following PRP support resources:

▸  National Organization of Rare Disorders: PRP Report
▸  Genetic and Rare Diseases Information Center: PRP Report
▸  PRP Alliance, Inc.
 PRP Survival Guide
▸  PRP Facebook Support Group
▸  PRP Community on RareConnect

The PRP Center of Excellence marks another achievement of the PRP Community in advancing access, quality and value of care. We look forward to continuing to serve the Community and hope to see you soon! To schedule your consultation with Drs. Keller and Ross, please call (215) 955-9295.

✽  Is there an expectation that PRP patients beyond Southeaster Pennsylvania, e.g., New York, New Jersey, Maryland and Delaware will access the resources of the PRP Center of Excellence?

✽  Is there an opportunity for a Second Opinion regarding diagnosis and treatment?

✽  Will other healthcare professionals be able to “consult” with the PRP Center of Excellence?

Sincerely,

Jouni Uitto, MD, PhD, Professor and Chair
Matthew Keller, MD, Associate Professor
Nicholas Ross, MD, Resident-Physician

The announcement was made to the PRP Facebook Community on November 13 and generated an immediate response including 52 LIKES and 38 COMMENTS including the following (as of 11/14/5:17 PM CST):

Teri R — Ft. Wayne, IN
Awesome

Eric S — San Luis Obispo, CA
That is terrific news! Though I am in remission I am glad to know that there is a place specializing in this condition for future sufferers.

Marion M — Ireland
Wonderful news and it will help so many. Well done to all who made it happen.

Margarita B
…find a medicine that puts an end to the PRP!

Anne E — UK
Fantastic News!

Leslie L
So happy to hear this!!! It is wonderful news!! THANK YOU SO MUCH to all who made this possible!!!Abbie C — San Antonio, TXThis is great.Chris U — England, UK
Great news.

Phil P — England, UK
Congratulations to all from over the Pond. PRP on PRP

Alan C — England, UK
This has the potential to be an excellent resource! Well done to all involved in helping and supporting this development!

Cathy H — China Grove, NC
Wow this is wonderful….

Sandi H
This is great news. Thank you for sharing this!

Marianne B  — San Diego, CA
Please tell me how this is going to help anyone if they don’t live in or near Pennsylvania?

Linda B — England

Or even further afield!

Tierney R — Virginia Beach, VA
Travel. I had to drive 5 1/2 hours and spend the night. I raised money through GoFundMe.

Marianne B — San Diego, CA
I will have to drive two weeks and Linda B  will need a ship.

Burt U — Kenedy, Texas
Outstanding

Guy F — Scaggsville, MD
Excellent news! I was treated by Dr. Keller up until April 2017 with significant clearing and I am participating in TJU’s PRP study. I have nothing but good things to say about TJU Dermatology!

Norma M — Chicago, IL
Wonderful news! It’s a start. More centers of excellence will likely follow

Craig W — Charlotte, NC

Epic news!

Connie D — Maitland, FL
Yippee!!!!!!!

 

If you have any question regarding the PRP Center of Excellence at Thomas Jefferson University, please “Leave a Reply” below and we will find the answer for you.

 

OTR26 — TJU Announces PRP Center of Excellence

Thank you for subscribing…

It was Thursday, October 1, 2015 when I posted the 24th and final issue of On the Road… on the PRP Alliance website. After writing 598 pages of “PRP stuff” I was ready to shift my focus to the PRP Survival Guide. No more self-imposed, end-of-the-month deadlines for me. Besides, I was in remission.

It has been over two years and I am still in remission — but eager to publish a newsletter for the PRP community.

I urge every PRP patient and caregiver who subscribes to On the Road…  to take the time to read what we publish and share your personal insights. PRP patients and caregivers live with PRP day in and day out. You are the subject matter expert for your version of PRP. We need to tell all our stories.

Bill McCue, Editor