From the Editor…
If pityriasis rubra pilaris is, in fact, an autoimmune disease, then joining the American Autoimmune Related Diseases Association would seem like an appropriate path forward. It was on this basis that I approached the AARDA in 2015 intent upon having the PRP Alliance become a member organization. It became clear, however, that it was the view of ARRDA that PRP was NOT an autoimmune disease. It should be noted that the National Psoriasis Foundation agrees.
According to the AARDA…
“The American Autoimmune Related Diseases Association is dedicated to the eradication of autoimmune diseases and the alleviation of suffering and the socioeconomic impact of autoimmunity through fostering and facilitating collaboration in the areas of education, public awareness, research, and patient services in an effective, ethical, and efficient manner.
“AARDA is the only national nonprofit health agency dedicated to bringing a comprehensive focus to autoimmunity, the major cause of over 100 serious chronic diseases. Approximately 50 million Americans — or one in five people — suffer from autoimmune diseases. Women are more likely than men to be affected; some estimates say 75% of those affected are women. Still, autoimmunity is rarely discussed as a women’s health issue.
“An autoimmune disease develops when a person’s immune system mistakenly identifies healthy cells as foreign cells and attacks them. There are over 100 known autoimmune diseases. Most of these are known by their singular names, but the public is generally unaware of their autoimmune nature. This makes it difficult to raise awareness of autoimmune diseases as a whole.
Many people also confuse the term autoimmune with acquired immune deficiency syndrome (AIDS), or they think these diseases are a form of cancer.
Editor’s Note: Skin disorders considered to be autoimmune include:
✽ Psoriatic arthritis
✽ Bechet’s disease
✽ Bullous pemphigoid
✽ Dermatitis herpetiforms
✽ Lichen planus
✽ Lichen sclerosus
✽ Psoriatic arthritis
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?
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
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.
…find a medicine that puts an end to the PRP!
Anne E — UK
OTR26 — TJU Announces PRP Center of Excellence
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
From the Editors: During the summer of 2013, a total of 487 PRP patients were polled by email and given an opportunity to participate in a first-of-its-kind PRP Biopsy Poll. Remarkably, within a 3-week period, a total of 256 invitees (52.6%) shared their biopsy experiences and/or recollections.The primary objective of the PRP Biopsy Poll was to better understand the role played by biopsies in the diagnosis of PRP from the perspective of a PRP patient
2013 PRP Biopsy Poll
It was a simple poll with only two questions.
(1) How many biopsies have you had that supported a PRP diagnosis?
(2) How many biopsies have you had that failed to confirm PRP?
Based on the results, however, a benchmark for a future biopsy-related survey was established.
✽ Diagnosis with no biopsy ordered: 7.8%
✽ Diagnosis with no contradictory biopsy: 45.3%
✽ Biopsy supported diagnosis: 23.4%
✽ Diagnosis with no confirming biopsy: 23.4%
2018 PRP Biopsy Survey
Since participation in the 2018 PRP Biopsy Survey will require a completed PRP Census form, we will have more dots to connect.
✽ Elapsed time from onset to diagnosis
✽ Misdiagnoses that preceded the PRP diagnosis
✽ Adult onset versus juvenile onset (based on onset age)
✽ Duration based on onset date and current status
Questions to ask dermatopathologists
While we are surveying PRP patients and caregivers, we should recruit an advisory committee of dermatopathologists. The following questions might be a good place to start.
✽ How should a biopsy be performed to increase the likelihood of a timely PRP diagnosis? Is there a right way? Wrong way? Better way?
✽ What specific signs or indicators should a dermatopathologist be looking for as they review the biopsy slides?
✽ Are the characteristics of PRP really so elusive that they cannot be easily seen?
✽ How important is it for the dermatologist who performs the biopsy to specifically instruct the dermatopathologist to “consider” or “look for” PRP.
The ongoing status of the 2018 PRP Biopsy Survey will be update in future issues of the PRP community newsletter — On the Road….
For additional information about diagnosing PRP, go to Chapter 2 of the PRP Survival Guide.
PRPA Plans Biopsy & Efficacy Surveys: OTR025.4
November 1, 2017
Every patient organization needs an Awareness Day they can call their own. We celebrate Rare Disease Day on the last day of February and share the spotlight with 7,000 other rare diseases. However, PRP Awareness Month gives us 30 days to crow like a banty rooster, shake our tail feathers and get more than our fair share of attention. — Bill McCue
My first PRP Awareness Day was November 28, 2012 and quite “unofficial” by any standards. It began with a phone call from my dermatologist’s office. I was told that the red spot on my forehead on August 8 — nearly four months prior — had been the onset of pityriasis rubra pilaris. Like most PRP “newbies”, I spent the next few days searching the internet and learning what little there was to learn about PRP. What I found was redundant, insufficient, outdated, and typically written for an audience of healthcare professionals.
On November 1, 2013, I celebrated PRP Awareness Day once again. This time I sent out over 1,500 emails to PRP patients and caregivers and called the effort the PRP Worldwide Census. It had taken me several months during the previous summer to review over 29,000 emails in the PRP Support Group Archive going back to November, 1997. As I collected email addresses I also harvested “core” information about the onset, diagnosis, and treatment of PRP. It took six months to solicit 500 responses. That was the genesis of the PRP Community Database.
November 6, 2014 was the first “official” observance of PRP Awareness Day. The date was selected to commemorate the signing of the Rare Diseases Act of 2002 that set the threshold for a rare disease. A rare disease or disorder is defined in the U.S. as one affecting fewer than 200,000 Americans. Using the European standard, however, the U.S. threshold would be only 160,000. Today there are more than 7,000 known rare diseases, and it is estimated that about 30 million Americans are affected by them.
November 6-8, 2015 was the first “official” observance of PRP Awareness Weekend since the 6th fell on a Friday. The “Big Event” would be a global Meet & Greet. Whether face to face or using technology (FaceTime, Skype and ooVoo) we pitched the idea to the PRP Facebook Support Group. Other than a few face-to-face Meet & Greets and a spike in Facetime encounters, the effort missed it’s mark. Lesson learned.
November 1-30, 2016 was the first “official” observance of PRP Awareness Month. Perhaps all we needed was 30 days rather than a weekend or a day. Sunday, November 6th was selected to host a 24-hour GoToMeeting gathering. Only the predictable and dependable cadre of seasoned PRP travelers stopped by to chat.
An important lesson has been learned over the past five years. If you run the same flag up the flagpole, the same people are going to salute. We’re going to try something different.
✔︎ Revive the PRP Community newsletter
✔︎ Launch the PRP Worldwide Census
✔︎ Execute the Rare Skin Disease Referral Initiative
✔︎ Initiate PRP Surveys
✔︎ Recruit participants for PRP research
✔︎ Promote legislative advocacy
✔︎ Support the PRP Alliance.
PRP Awareness Month Begins Today: OTR025.1
From the Editors: The PRP Community Database currently tracks 1,622 PRP patients who are now members of the PRP Facebook and/or RareConnect Communities, or are unaligned but have a valid email address. The 2017 PRP Worldwide Census begins today with a simple goal: confirm the data we have and add data we are missing.
If you are a PRP patient or caregiver, you have something we need.
✽ We can’t force you to give it to us.
✽ We don’t have the funds to buy it from you.
✽ And we would never try to steal it from you.
All we can do is ask you to share and hope you say “Yes”.
Sharing data: Are you a skeptic?
Most PRP patients/caregivers appreciate the importance of sharing data to help PRP researchers better understand pityriasis rubra pilaris. Because your information is personal and private, it is important that we safeguard any data you share. We hope that our data collection methodology encourages your enthusiastic participation in the upcoming PRP Worldwide Census.
Confidential Data — While the PRP Alliance is not required to comply with federal regulations regarding the security of protected health information (HIPAA Security Rule), the PRP Community Database is in full compliance. The data we collect about your PRP journey and the data for other PRP patients (active and in remission) is both encrypted and password protected. Moreover, the data cannot be accessed via the Internet.
De-identified Data — When shared with PRP researchers or published on the PRP Survival Guide website, your data is de-identified. Simply stated, the PRP Community Database reports numbers, not names. When your PRP-related data is published or shared, that data is disassociated with your name. There is complete and total anonymity.
The Value of Data in the Aggregate — Our PRP-related data has the greatest value when combined with the data of others. It is the aggregation of data that will ultimately enlighten PRP researchers.
Sharing of Data — The PRP community will be notified when information contained in the PRP Community Database is shared with PRP researchers, e.g., Thomas Jefferson University, Yale University School of Medicine, etc. Such notification will include the reason why the de-identified data is being shared.
What is “Core” data?
The following datapoints represents core data. With this information we can build a profile of the worldwide PRP community.
✽ Elapsed time between onset and diagnosis
✽ Elapsed time between onset and remission
✽ Onset age by decade of life
✽ Biopsies as a diagnostic tool
✽ Survey PRP patients in remission
✽ Survey PRP children based n onset age
Name: Bill McCue
Even though we ask for the FIRST NAME and LAST NAME, we NEVER publish NAMES. WE ONLY report NUMBERS, e.g., How many “this” and how many “that”.
Location: Plano, TX
We are NOT looking for street addresses or ZIP Codes/Postal Codes)
The email address is the only way we can verify that the information we receive is from a valid source. We will send a confirming email to the email address of the person completing the PRP Census. If the email confirmation is undeliverable, the data will not be added to the database.
Onset Age: 66 years, 2 months
At what age did your PRP symptoms first appear?
Onset Date: August 8, 2012
What was the approximate date when your PRP symptoms first appeared? Because few people know the exact date Onset Date, only month and year is required, Do the best you can, e.g., August 8, 2012; August 2012, or even the “Summer of 2012”.
Misdiagnosis: seborrheic dermatitis
What misdiagnoses preceded your “official” PRP diagnosis? We are only concerned with the misdiagnoses of your dermatologist, not your GP. Options include psoriasis, eczema, seborrheic dermatitis, allergic reaction and other (specify)
Biopsy History: 3 biopsies inconclusive. 4th biopsy supported PRP
What role did biopsies play in your diagnosis of PRP?
Diagnosis Date: November 28, 2012
The Diagnosis Date should be as accurate as possible, e.g., November 28, 2012, November, 2012; end of November, 2012. Do the best you can.
Current Status: In remission since April 4, 2012
Don’t worry about definitions. Given the choice between “Active” and “In Remission”, what is your current status? If “in remission”, what is your date of remission
Treatment Options (oral/injection): acitretin
All we want to know are the PRP-related drugs you were prescribed from the list retinoids, immunosuppressants and biologicals. Whether or not the drugs were successful will be the subject of a future survey. We are NOT collecting data on itch and pain relief, topical ointments, creams and lotions, sleep aids, etc.
We are a global community. Each country has their own way of protecting a patient’s medical information. For purposes of the PRP Census, we comply with U.S. HIPAA regulations. Even with assurances of confidentiality, data collection has never been an easy task.
Confidential Data – The data we collect about your unique PRP journey – and the data for all PRP patients (active and in remission) – is encrypted, password-protected, and not connected to the Internet.
If you have any questions, Leave a Reply below. If not, your PRP Worldwide Census awaits.
1,621 Invited to Share PRP "Core" Data: OTR025.2
I have been collecting PRP-related information from PRP patients and their caregivers since November, 2013. In the beginning it could take six requests before I received a completed PRP census form.
On the 1st and 15th of ever month the NUMBERS will be posted here. The list will show the November 1, 2017 numbers and the most current numbers.
The numbers below are based on the “core data” shared by PRP patients and caregivers worldwide. The PRP Community Database is the most complete representation of our patient population. Thank you for sharing your information.
Bill McCue, Founder/President
PRP Alliance, Inc.
Recap as of November 1, 2017
Numbers in parentheses ( ) indicate “not providing data”
✽ Total number of PRP patients reporting: 1,629
✽ Reporting email addresses: 1,033 (596)
✽ Reporting location: 1,140 (489)
✽ Reporting onset date: 715 (914)
✽ Reporting onset age: 651 (978)
✽ Reporting diagnosis date: 694 (935)
✽ Reporting current status: 656 (973)
✽ Active: 394
✽ Remission: 262
✽ Remission date: 64 (198)
✽ Reporting standard drug therapies: 67 (1,562)
Let’s start with a little history.
When James Shooter was admitted to St. Bartholomew’s Hospital in London, England in 1828, he unwittingly became the world’s first recorded patient with what the medical community would eventually call pityriasis rubra pilaris.
Fast forward 169 years. The PRP community has only been around since late 1997 when a group of PRP patients and caregivers traded emails on AOL. It was Jean-Luc Deslauriers, a Canadian from New Brunswick, who got the ball rolling. Our own Tierney Ratti and Ginny Maxwell’s mother were there when the PRP Support Group was established. For 16 years PRP patients and caregivers traded an average of 150 emails each month. It was the only game in town for a while until technology caught up.
In June 2008, Jonah Grant-Scarfe, another Canadian, founded the PRP Facebook Support Group. In September 2013 it transitioned from a Public Forum into a Closed Group with about 100 members. By year’s end Tierney was the Administrator and the rest is history, Today the PRP Facebook community is nearing 1,100 members and growing. Each month a hundred discussions are started with thousands of comments and replies. The PRP Facebook community has earned the moniker: “The Land of Chat”.
That’s an easy question to answer. It takes money to do the things we want to do. Are the projects and activities worth the effort — worth your donation? That’s a decision we want you to consider. Here’s how we intend to use your donation.
#1 — 2017 PRP WORLDWIDE CENSUS
The first PRP Worldwide Census kicked off on November 1, 2013 with emails being sent to over 1,500 PRP members of the PRP Support Group. Unfortunately, within 24 hours, more than 500 email addresses were purged as “undelverable”. By the end of May, however the PRP Community Database had updated records for over 1,000 PRP patients.
As of November 17, 2017, the PRP Community Database currently tracks 1,627 PRP patients of which 1,002 (62%) are members of the PRP Facebook Support Group. The challenge we face is finding the missing data.
✽ Missing locations: 468 (29%)
✽ Missing onset dates: 868 (54%)
✽ Missing onset ages: 933 (58%)
✽ Missing diagnosis dates: 889 (55%)
✽ Missing current status: 927 (57%)
We can do a better job. The focus of the 2017 PRP Worldwide Census is to fill in the data gaps for all the datapoints including:
✽ Onset date
✽ Onset age
✽ Biopsy history
✽ Diagnosis date
✽ Treatment (oral or injection — from a list of drugs)
✽ Dermatologist of record
✽ Current Status (active/remission)
The PRP Alliance will use the donations we receive to support the 2017 PRP Worldwide Census.
#2 — DIAGNOSING PRP
During the summer of 2013, a total of 487 PRP patients/caregivers were polled by email and given an opportunity to participate in a first-ever PRP Biopsy Poll. The primary objective of the PRP Biopsy Poll was to better understand the role played by biopsies in the diagnosis of pityriasis rubra pilaris from the PRP patient perspective. There were only two questions:
✽ How many biopsies have you had that supported a PRP diagnosis?
✽ How many biopsies have you had that failed to confirm PRP?
Within a three-week period a total of 256 PRP patients/caregivers responded with the following results:
✽ Diagnoses made strictly on the basis of clinical observations — no biopsies were ordered: 7.8%
✽ The results of the biopsy provided sufficient information to support a diagnosis: 45.3%
✽ Non- conclusive biopsies were ignored when the clinical observations were “consistent” with a PRP diagnosis: 23.4%
✽ PRP diagnosis made without the benefit of a confirming biopsy: 23.4%
✽ The PRP Biopsy Poll also suggests that when a dermatologist instructs a pathologist to consider PRP, the results nearly always confirm PRP.
It is time to revisit the issue of biopsies and the timely diagnosis of PRP. Your donation will support the 2017 PRP Biopsy Survey.
#3 — TREATING PRP
When a patient is diagnosed with PRP, the dermatologist typical prescribes medications but rarely refers the patient to the PRP Alliance, PRP Facebook Support Group, the PRP Community on RareConnect or to the PRP Survival Guide. Instead, the PRP patient is referred to Dr. Google, Dr. Yahoo or Dr. Bing to find answers and support on their own. On November 1, the PRP Alliance will begin a year-long campaign to change that practice. The PRP Dermatology Referral Initiative will advocate that patients diagnosed with PRP — or any rare skin disorder — be referred to the following websites:
The PRP Alliance has the support of both organizations in this effort.
PRP Treatment Efficacy Survey
The PRP community has an opportunity to collect information that could whet the appetite of PRP researchers. We hear the mantra every day: What works for one doesn’t work for all. But let’s quantify what works and what doesn’t, e.g., acitretin, methotrexate, Stelara, Cosentyx, Humira, Remicade, Otezla, Taltz, etc. The PRP Worldwide Census will identify drugs that have been prescribed, the PRP Treatment Efficacy Survey will collect information on what worked and what didn’t.
Your donation will support both the Dermatology Referral Initiative and the PRP Treatment Efficacy Survey.
#4 — RESEARCHING PRP
Prior to 2012 the PRP community had lamented the lack of PRP research. In October 2012, researchers at Thomas Jefferson University began genetic research with 50 participants. Their focus was on the genetic mutation of CARD14. During the summer of 2015, Dr. Jouni Uitto, Chair of the Department of Dermatology and Cutaneous Biology expanded that research. The PRP community provided 105 participants and TJU published their finding in 2016. Today both genetic and clinical research continues in Philadelphia.
The PRP community has been officially invited to participate in three major PRP research projects underway at Yale University School of Medicine. The funding for this research comes from the National Institutes of Health and the Foundation of Ichthyosis and Related Skin Types (FIRST). We are one of the “Related Skin Types”,
✽ Genetic research where PRP is hereditary, e.g., familial PRP
✽ PRP Patient Registry
✽ PRP Burden of Skin Disease research
Your donation will support the efforts of the PRP Alliance to recruit participants in PRP research at TJU and Yale
#5 — BUILDING PRP AWARENESS
The PRP community newsletter was published during the period April, 2014 to October, 2015. A total of 24 issues (598 pages) of PRP-related information was shared. The newsletter was suspended when the focus shifted to the PRP Survival Guide. It’s time to bring back the newsletter. It’s time to revive: On the Road… Our Journey from Onset through Remission .
The newsletter will mirror the organization of the PRP Survival Guide.
✽ Chapter 1 — The Basics
✽ Chapter 2 — Diagnosing PRP
✽ Chapter 3 — Treating PRP
✽ Chapter 4 — Daily Life
✽ Chapter 5 — PRP Parents and Kids
✽ Chapter 6 — Remission
✽ Chapter 7 — PRP Research
✽ Chapter 8 — PRP Advocacy
Your donation will underwrite the email distribution of On the Road… to the PRP community worldwide.
If you’ve been wondering how to help or if you’ve been wishing you could do something more, we welcome your tax deductible contribution.
If you needed some “specifics” to justify a donation, we hope that the information we provided here was enough.
The PRP Alliance is a 501(c)(3) tax-exempt nonprofit corporation. Our Employer Identification Number is 47-3868578. We will make sure that your donation receives a proper receipt for your tax records.