2017 NORD PRP Report Revision — Second Draft

TO:    PRP-savvy Dermatologists and other Healthcare Professionals

FROM:    Bill McCue, Editor, PRP Survival Guide

SUBJECT:   2017 Revision of the NORD PRP Report

The National Organization of Rare Disorders maintains a Rare Disease Database to provide brief introductions to more than 1,200 rare diseases — including pityriasis rubra pilaris. These rare disease reports are primarily geared toward patients and families. There is also an important secondary audience, e.g.,  physicians, researchers, nurses, students, journalists and others who might request and benefit from NORD information.

The NORD website enjoys one million visitors per month, 85% of whom access the rare disease database. The PRP community is indeed fortunate to have had “our disease” listed for nearly two decades.

NORD works very hard to keep their Rare Disease Database as up-to-date and accurate as possible. Their efforts include having rare disease reports periodically revised and reviewed by MDs with expertise on the topic. The NORD PRP report was first published in 1988 and subsequently reviewed in 1989, 1999 and most recently in 2007.

The PRP Facebook and RareConnect communities have had the opportunity to review and comment on the FIRST DRAFT of the 2017 Revised NORD PRP Report. That effort has resulted in the SECOND DRAFT.

We invite you to review this SECOND DRAFT?  I you want to make a comment, either use  “Leave a Reply” at the bottom of this webpage or send an email to editor@prpSurvivalGuide.org.

Pityriasis Rubra Pilaris


Devergie’s Disease
Lichen Acuminatus
Lichen Ruber Acuminatus
Pityriasis Rubra Pilaire (Fr.)
Lichen Ruber Pilaris(1) (2) (3)

Subdivisions of PRP

Type 1 — Classical Adult Onset
Type 2 — Atypical Adult Onset
Type 3 — Classical Juvenile Onset
Type 4 — Circumscribed Juvenile Onset
Type 5 — Atypical Juvenile Onset
Type 6 — HIV-associated(1) (2) (3)


Pityriasis rubra pilaris (PRP) is a group of rare skin disorders that cause inflammation and shedding of the skin.  The name means scaling (pityriasis), redness (rubra) and involvement of the hair follicles (pilaris).(4)

Typically, PRP appears first as a small spot somewhere on the body and then spreads elsewhere.(5)

It will impact different parts of the body in different ways for unpredictable periods of time. (5) The inflammation may cover the entire body or just parts of the body such as the elbows and knees, palms and soles. (6) The disease may progress and leave distinct areas of uninvolved skin, the so-called “islands of sparing” or “skip areas”.(7)

The disorder may start in childhood or adulthood. It affects males and females equally.(8)

There are several types of PRP, which are classified based on age of onset, body areas affected, and whether other associated conditions are present. PRP is usually sporadic (occurring randomly) but some forms may be inherited.(9)

With an estimated 800-plus “active” patients in the U.S. and less than 1900 in Europe, PRP is an ultra-rare skin disorder. The rarity of PRP notwithstanding, the signs and symptoms of PRP often mimic those of eczema (31.6 million patients) and psoriasis (8 million patients).(10) (11)

The treatment of PRP typically involves a combination of systemic and topical therapies combined. Topical therapies can help with the symptoms and may be enough for people with mild PRP. Topical treatments are usually combined with systemic therapy for PRP that affects a large part of the body. Most people need systemic therapy to control the condition.

Currently there are no treatments approved by the US Food and Drug Administration (FDA) or the European Medicines Agency (EMA) for use in PRP.(12)

PRP patients and their caregivers quickly learn that every case of PRP is unique.(4)


When James Shooter was admitted to St. Bartholomew’s Hospital in London, England in 1828, he unwittingly became the world’s first patient with what the medical community would eventually call pityriasis rubra pilaris. In 1828, however, Mr. Shooter’s skin disorder did not yet have a name. Seven years passed before Claudius Tarral, a French dermatologist, wrote about the case in Traite theorique et pratique des maladies de la peau (Treatise on Skin Diseases) in 1835. Tarral saw it as a variant of psoriasis.(13) (14)

Alphonse Devergie

It would take another 21 years for Marie Guillaume Alphonse Devergie, a dermatologist and forensic doctor at St. Louis Hospital in Paris, to publish the most complete description of PRP. In fact, it was considered to be the “original description” of PRP by the medical community. Devergie’s article was published in the Gazette Hebdomadaire de Medecine et de Chirurgie in 1856.(14)

While Devergie saw the skin disorder as a combination of “follicular lesions and psoriasis palmaris, pityriasis capillitii and pityriasis rubra”, it would take yet another 21 years before another Frenchman and dermatologist by the name of Richaud to recognize PRP as a distinct entity.   Richaud published “Etude sur le pityriasis pilaris” in 1877.(15)

Ernest Bernier

When Ernest Besnier presented nine cases in a 120-page article published in 1889 — 12 years after Richaud, 43 years after Devergie, and 54 years after Tarral, he forever fixed the name of the disease as pityriasis rubra pilaris. Besnier too, was a Frenchman and dermatologist. He was also the medical director of the St. Louis Hospital in Paris — the same hospital as Devergie.(16) The name comes from three Latin words: pityriasis (scalelike skin), rubra (red) and pilaris (hair follicles).(2)

Like many rare disease communities, the PRP community laments the snail’s pace at which PRP research progresses. What should we expect —  it took 61 years just to get the name right.

Signs & Symptoms

The terms “sign” and “symptom” are not redundant. A SIGN is subjective, eg, pain, itching, fatigue. The PRP patients must share that information with the dermatologist, dermatology physician assistant or dermatology nurse.  In contrast, a SYMPTOM can be observed by others, including healthcare  professionals. The following is a list of SIGNS and SYMPTOMS that define the PRP experience.

Pre-onset Signs|

Mild signs include dandruff and crusty scalp, limited red patches or scaling of the skin, eg, dime-sized red spot” on forehead. Duration varies from patient to patient. At some point patient determines that intervention of a healthcare professional is warranted, eg, red spot has doubled in size in less than two weeks.

Onset of Symptoms

Depending on the advance of inflammation, a general practitioner or dermatologist will see symptoms including pink, red, or orange-red scaly patches on your skin. The patches are usually itchy. You may have the scaly patches only on some parts of your body. They most often occur on the elbows, knees, hands, feet, and ankles. The skin on the palms of your hands and the soles of your feet may also become red and thickened. The scaly patches may eventually spread over the entire body.(17)

Cracks may develop which can be painful and make walking and using the hands difficult. The nails may become thickened and discolored at the free nail edge and may show linear black streaks (splinter hemorrhages). The hair may thin considerably.(6)  Shivering, heat and fluid loss may occur if the rash covers large areas of skin.(4) The onset of PRP may be further exacerbated if a misdiagnosis of psoriasis or eczema, for example, results in an improper treatment option.(5)

Acute Stage Signs and Symptoms

The dermatologist will be able to see the symptoms of a body engulfed by dry, red, and flaking skin, swollen feet and legs, and cracked and bleeding feet. There may be serious issues related to impaired mobility, eyes and vision, and dexterity. The PRP patient will see signs of PRP from a different perspective, eg, pain of motion, unrelenting itch, inability to sweat, overheating, the impact of cold, heat and sleep deprivation. The Acute Stage poses the greatest challenge to body, mind, and spirit and can last less than a month or months longer. This is the time in the PRP journey that PRP patients and caregivers should seek support from patient support groups. It is also a time to address issues of depression.(5)

Management Stage

After the Acute Stage, the journey of a PRP patient takes on a new focus — mitigating symptoms. All the potential irritants are waiting on the roadside, eg, joint pain, clogged ears, disability claims, etc. While 90% of the PRP patient population can look forward to full remission within one to four or five years, the timetable is not certain. Those diagnosed with Atypical Adult Onset and Atypical Juvenile Onset, the chronic versions of PRP, must develop long-term coping skills. For everyone, the daily routine associated with medications, moisturizing, and dealing with the unpleasantries of this skin disorder cannot be ignored.

Remission & Healing Milestones

There does not appear to be an official definition of remission as it applies to pityriasis rubra pilaris. For some it means no meds, no signs, and no symptoms. Others are told they are in remission by their dermatologist during their last clinic visit. Other believe that sustained improvement with an acceptable quality of life is all that is required for a declaration of remission. The PRP community, however, has adopted a more celebratory approach with recognition of healing milestones, eg, the return of sweat, the first trip to Walmart for groceries, dark hardwood floors that don’t need hourly vacuuming. These milestones are signs of healing that PRP patients and caregivers feel and symptoms that everyone else observes.


The specific underlying cause of PRP is unknown, although genetic factors, an abnormal immune response, or vitamin A deficiency may be involved.  (18), (19), (20), (21), (22)

PRP is an autoinflammatory disease according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), “a relatively new category of diseases that are different from autoimmune diseases. However, autoimmune and autoinflammatory diseases share common characteristics in that both groups of disorders result from the immune system attacking the body’s own tissues, and they also result in increased inflammation.“(23) When your body is attacked — perhaps by a virus or other germs — your immune system defends you. It “sees” and kills the germs that might hurt you.

“But when the system doesn’t work right, this process can cause harm. Immune cells can mistake your body’s own cells as invaders and attack them. This “friendly fire’ can affect almost any part of the body. It can sometimes affect many parts of the body at once. This is called “autoimmunity’ (meaning “self-immunity’).

“The part of the immune system that orchestrates all of this develops as a person grows and is known as the acquired immune system. It “remembers” foreign antigens, or proteins, so that it can fight them if they come back. It employs white blood cells called lymphocytes.

“But the body also has an innate (inborn) immune system that is more primitive. It employs types of white blood cells called granulocytes and monocytes to destroy harmful substances. In autoinflammatory diseases, this innate immune system causes inflammation for unknown reasons. It reacts, even though it has never encountered autoantibodies or antigens in the body.

“Autoinflammatory disorders are characterized by intense episodes of inflammation that result in such symptoms as fever, rash, or joint swelling. These diseases also carry the risk of amyloidosis, a potentially fatal buildup of a blood protein in vital organs.”(23)

Affected Populations

Based on conversations within the PRP community, we can say with metaphysical certitude that PRP isn’t a punishment for misbehavior or forgetting to put the seat down on a toilet (loo in some parts of the world). There are thousands of perfectly wonderful people who have — or had — PRP. Moreover, there are many very bad people who don’t have it. Who then gets the short end of this rare disease stick?

Prevalence: In March 2003, Dr Andrew Griffiths delivered a “Dowling Oration” to members of the British Association of Dermatology assembled in Liverpool, England. Dr Griffiths reflected on 35 years of diagnosing, treating and researching pityriasis rubra pilaris. He unilaterally fixed the PRP prevalence rate at one in 400,000. While the methodology used by Dr Griffiths is subject to debate, dermatologists worldwide have accepted his estimates.(1)

Age: Pityriasis rubra pilaris is a rare disorder that may develop during childhood or adulthood. Juvenile Onset accounts for 45% of the “active” patient population while Adult Onset accounts for 55%.(6)

Although PRP may occur at any age (10), it most commonly affects those in their first, second, fifth, or sixth decades of life.(24)(2)

Gender: PRP appears to occur in males and females in relatively equal numbers. However, in childhood, the male to female ratio is 3:2.(25)

Race: Persons of any race may be affected.(2)(7)

Acquired or Inherited: PRP is usually sporadic (occurring randomly) but some forms may be inherited.(6)(2)

Related Disorders

Symptoms of the following disorders can be similar to those of pityriasis rubra pilaris. Comparisons may be useful for a differential diagnosis:

Psoriasis: According to the National Psoriasis Foundation, “psoriasis is an immune-mediated disease that causes raised, red, scaly patches to appear on the skin. It typically affects the outside of the elbows, knees or scalp, though it can appear on any location. Some people report that psoriasis is itchy, burns and stings. Psoriasis is associated with other serious health conditions, such as diabetes, heart disease and depression.” More information is available at the NPF website.(26)

Atopic dermatitis (Eczema & Dermatitis): There are different types of eczema that collectively affect more than 30 million Americans: atopic dermatitis, contact dermatitis, dyshidrotic eczema, hand eczema, neurodermatitis, nummular eczema and stasis dermatitis. For more information contact the National Eczema Foundation.(27)

Allergic reaction: Most allergic reactions are minor, such as a rash from poison ivy, mosquito or other bug bites, or sneezing from hay fever. The type of reaction depends on the person’s immune system response, which is sometimes unpredictable. For more information about allergic reactions, go to eMedicineHealth.(28)

Pityriasis rosea: Pityriasis rosea (PR) is a benign rash, a common skin disorder observed in otherwise healthy people, most frequently children and young adults. It can easily mimic types of similar skin eruptions including lichen planus, psoriasis, and pityriasis rubra pilaris. Pityriasis rosea has a very specific and recognizable rash. It does, however, begin with a “herald mark”. Follow the link for additional information at Medscape.(29)

Fungal infection: Fungal infections of the skin are also known as ‘mycoses’. They are common and generally mild. However, in very sick or otherwise immune suppressed people, fungi can sometimes cause serious disease. Fungi are parasites or saprophytes, ie, they live off living or dead organic matter. Mycologists identify and classify fungi according to their appearance by microscopy and in culture, and by the method of reproduction, which may be sexual or asexual.(30)

Lupus: Lupus is a chronic autoimmune disease that can damage any part of the body (skin, joints, and/or organs). “Chronic” means that the signs and symptoms tend to last longer than six weeks and often for many years. In lupus, something goes wrong with the immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). Normally our immune systems produce proteins called “antibodies” which protect the body from these invaders. “Autoimmunity” means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”). As a result, it creates autoantibodies that attack and destroy healthy tissue. These autoantibodies cause inflammation, pain, and damage in various parts of the body.(31)

Cutaneous T-cell lymphoma: On occasion, the diagnosis of a PRP patient has been changed to cutaneous T-cell lymphoma. The PRP community recommends that biopsy be performed to rule out CTCL. Inclusion of CTCL is an appropriate warning to PRP patients, caregivers and dermatologists.(32)


In the FINAL DRAFT, the references under the NORD-required headings: Textbooks, Journal Articles and From the Internet.

(1)   Griffiths WA. Edited version of the Dowling Oration delivered to the British Association of Dermatologists in Liverpool, England. March 2003. http://prpsurvivalguide.org/wp-content/uploads/2017/05/Dowling-Oration-2003-Liverpool-England.pdf Accessed August 7, 2017.

(2)  Paravina M, Ljubenovic M, Milosavljevic M, et.al. Pityriasis rubra pilaris: A report of two cases and literature review. https://www.degruyter.com/downloadpdf/j/sjdv.2015.7.issue-4/sjdv-2015-0016/sjdv-2015-0016.pdf. Serbian J Dermatol Venereol. 2015; 7 (4): 181-194 DOI: 10.1515/sjdv-2015-0016. Accessed August 7, 2017.

(3) ICD!)Data.com.  http://www.icd10data.com/ICD10CM/Codes/L00-L99/L40-L45/L44-/L44.0 Accessed August 7, 2010.

(4) British Association of Dermatologists. Pityriasis rubra pilaris leaflet. http://www.bad.org.uk/shared/get-file.ashx?id=116&itemtype=document  Updated May 2016. Accessed August 7, 2017.

(5) PRP Survival Guide. Chapter 1 — The Basics.  http://prpsurvivalguide.org/2017/05/19/yayaya/?trashed=1&ids=3 Accessed August 7, 2017.

(6) DermNet New Zealand. Pityriasis rubra pilaris.  www.dermnetnz.org/topics/pityriasis-rubra-pilaris/ Updated October, 2015. Accessed August 7, 2017.

(7)  Medscape.com. Pityriasis rubra pilaris. http://reference.medscape.com/article/1107742-overview Updated April 17, 2017. Accessed August 7, 2017.

(8)  Healthline.com. Pityriasis rubra pilaris.  http://www.healthline.com/health/pityriasis-rubra-pilaris#Introduction1 Reviewed June 9, 2016. Accessed August 7, 2017.

(9)  Genetic and Rare Disease Information Center. Pityriasis rubra pilaris. https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris Updated August 1, 2017. Accessed August 7, 2017.

(10)  National Eczema Association. Eczema Facts. https://nationaleczema.org/research/eczema-facts/ Accessed August 7, 2017.

(11)  National Psoriasis Foundation. About the National Psoriasis Foundation. https://www.psoriasis.org/about-us Accessed August 7, 2017.|

(12)  Genetic and Rare Diseases Information Center. Pityriasis rubra pilaris. https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris#ref_7262 Accessed August 7, 2017.

(13)  Tarral C. Observation CXVIII. In Rayer P :‘Traite theorique et pratique des maladies de la peau’, Paris: Bailliere:1835: 2,158-9.

(14)  Devergie MGA. Pityriasis rubra pilaris, maladie de peau non decrite par les dermatologists. Gazette Hebdomadaire de Med. et de Chirurg. 1856;3:197-201.

(15)  Richaud A,  Féré C. Etude sur le pityriasis pilaris [dissertation]. Paris: A Parent; 1877.

(16)  Besnier E. Observations pour server a l’histoire clinique du pityriasis rubra pilaire (pityriasis pilaris de Devergie et de Richaud). Ann Dermatol 2nd series 1889;10: 253-87, 398-427, 485- 544.

(17)  Healthline.com. Pityriasis rubra pilaris. http://www.healthline.com/health/pityriasis-rubra-pilaris#causes3 Reviewed June 9, 2016. Accessed August 7, 2017.

(18) Healthline.com. Pityriasis rubra pilaris. http://www.healthline.com/health/pityriasis-rubra-pilaris#types2 Reviewed June 9, 2016. Accessed August 7, 2017.

(19)  Genetic and Rare Diseases Information Center. Pityriasis rubra pilaris.  https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris Accessed August 7, 2017

(20)  Medscape.com. Pityriasis rubra pilaris.   http://reference.medscape.com/article/1107742-overview#a5 Updated April 17, 2017. Accessed August 7, 2017.

(21)  MedlinePlus, US National Library of Medicine. Pityriasis rubra pilaris.  https://medlineplus.gov/ency/article/001471.htm Updated July 5, 2017. Accessed August 7, 2017.

(22)  New Medical.net. Pityriasis rubra pilaris skin condition. http://www.news-medical.net/health/Pityriasis-Rubra-Pilaris-Skin-Condition.aspx Updated May 26, 2016. Accessed August 7, 2017.

(23)  National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). Understanding autoinflammatory diseases. https://www.niams.nih.gov/Health_Info/Autoinflammatory/default.asp January 2017. Accessed August 7, 2017.

(24) Judge MR, McLean WHI, Munro CS. Disorders of keratinization. In: Burns T, Breathnach S, Cox N, Gri ths C, editors. Rook’s textbook of dermatology. 8th ed. Oxford: Blackwell Publishing Ltd; 2010. p. 19.1-19.122.

(25)  Sehgal VN, Srivastava G. (Juvenile) Pityriasis rubra pilaris. https://www.ncbi.nlm.nih.gov/pubmed/16650174 Int J Dermatol 2006;45(4):438-46. Accessed August 7, 2017. |

(26)  National Psoriasis Foundation.  https://rarediseases.org/organizations/national-psoriasis-foundation/  Accessed 2017.

(27) National Eczema Association. https://nationaleczema.org Accessed 2017

(28)  eMedicineHealth. Allergic reaction. http://www.emedicinehealth.com/allergic_reaction/page2_em.htm#what_causes_an_allergic_reaction ; 2017

(29)  eMedicine Medscape. http://emedicine.medscape.com/article/1107532-overview ; Reviewed April 12, 2016. Accessed August 7, 2017.

(30)  DermNet New Zealand. Introduction to fungal infections. https://www.dermnetnz.org/topics/introduction-to-fungal-infections/ 2003. Accessed August 7, 2017.

(31)  Lupus Foundation of America. http://www.resources.lupus.org/entry/what-is-lupus Accessed August 7, 2017.

(32)  Cutaneous Lymphoma Foundation. https://www.clfoundation.org; Accessed August 7, 2017.

(33) Committee on the Development of the Third Edition of the Reference Manual on Scientific Evidence, Committee on Science, Technology, and Law Policy and Global Affairs, FEDERAL JUDICIAL CENTER, National Academic Press, Washington, DC, page 214. https://www.fjc.gov/sites/default/files/2015/SciMan3D01.pdf Accessed August 7, 2017.

(34) International Center for Toxicology and Medicine. From symptoms to liability: The distinct roles of differential diagnosis and causation assessment. http://www.ictm.com/Downloads/dri-causation.pdf. Accessed August 7, 2017

(35)  MedicineNet.com. Medical definition of differential diagnosis.  http://www.medicinenet.com/script/main/art.asp?articlekey=2991 Reviewed May 13, 2016. Accessed August 7, 2017.

(36)  PRP Survival Guide. Chapter 7 — PRP Research. http://prpsurvivalguide.org/2017/05/21/chapter-6-prp-research/ Updated August 1, 2017; Accessed August 7, 2017.

2017 NORD PRP Report Revision — Second Draft

OPEN ACT — H.R. 1223 and Senate Bill 1509

From the Editor…

The OPEN ACT (Orphan Product Extensions Now, Accelerating Cures and Treatment) is on the radar of the PRP community. The official designations are H.R. 1223 in the U.S. House of Representatives and S. 1509 in the U.S. Senate.

The proposed legislation would provide an incentive for biopharmaceutical companies to repurpose medicines that treat common disease for rare diseases. We think it has the potential to double the number of FDA-approved treatments for rare disease (and much more quickly than traditional drug development since the process of adding a new indication is much less intensive than the review of a new potential therapy). I currently take medicine off-label for my rare disease, which means my doctor and I had no clinical data to look at when considering whether it was a good treatment option and my insurance company refuses to cover its use off-label. OPEN ACT could help on both points.

The EveryLife Foundation for Rare Diseases has prepared the following overview of OPEN ACT (HR 1223

Please Support the OPEN ACT (HR 1223 and S. 1509 )
Orphan Product Extensions Now, Accelerating Cures and Treatments

Congress should incentivize the repurposing of potentially life-saving approved drugs for rare diseases and pediatric cancers. Similar incentives have been critical in the development of new medicines for underserved patient populations and could lead to hundreds of safe, effective and affordable rare disease treatments within the next five years. The OPEN ACT is sponsored in the House (H.R. 1223) by Representatives Bilirakis (R-FL) and Butterfield (D-NC) and sponsored in the Senate (S. 1509) by Senators Orrin Hatch (R-UT) and Robert Menendez (D-NJ).


Despite advances made by the Orphan Drug Act, 95 percent of the 7,000 rare diseases still have no treatment approved by the Food and Drug Administration (FDA). Most rare disease patients are prescribed treatments off-label, at times with little clinical evidence and variable effectiveness. As a result, obtaining reimbursement for off-label treatments or procedures can be challenging for patients. Biopharmaceutical companies seldom consider repurposing approved therapies to treat rare diseases because there is little incentive for them to do so.


The OPEN ACT would establish a six-month marketing exclusivity extension, providing an incentive to a sponsor to repurpose an already approved therapy for a rare disease. The sponsor company would need to demonstrate that the repurposed therapy is safe and effective in treating the rare disease and obtain a rare disease indication from FDA on the drug label. The OPEN ACT is modeled on the highly successful Best Pharmaceuticals for Children Act (2002) that has led to more than 500 labeling changes for pediatric populations.


Scientific literature shows that a single-targeted drug is likely to have multiple therapeutic uses and that biopharmaceutical companies can repurpose drugs for the treatment of different diseases. Repurposing drugs is faster, cheaper, and presents fewer risks than traditional drug development. For complex rare diseases with small patient populations, the current economic model of drug development often lacks financial viability. Utilizing targeted economic incentives has a proven track record of encouraging industry stakeholders to invest in the development of drugs for diseases with unmet need.


The OPEN ACT would leverage the investment already made by biopharmaceutical companies into the development of approved therapies by providing an economic incentive to explore ways to bring more treatments for rare diseases to the marketplace through the process of repurposing drugs, resulting in:

✽   Potentially hundreds of well-tested therapies approved and on the label for rare disease patients in the next five years.

✽  Major market drug prices, resulting in a reduction in the average cost of rare disease drugs.

✽  Fewer rare disease patients using untested and potentially ineffective drugs off–label.

✽  A surge in biotech investment, new jobs, and grants to research universities to conduct repurposing trials.

Among the 229 supporters…

The supporters highlighted in boldface type are members of the Coalition of Skin Diseases.

✽  American Behcet’s Disease Association
✽  Autoinflammatory Alliance
✽  Cutaneous Lymphoma Foundation
✽  Debra of America
✽  EveryLife Foundation for Rare Diseases
✽  Foundation for Ichthyosis & Related SkinTypes, Inc.
✽  International Pemphigus and Pemphigoid Foundation (IPPF)
✽  National Organization of Rare Disorders (NORD)
✽  PRP Alliance, Inc. (PRPA)
✽  Sturge-Weber Foundation

Click HERE for the full list of Open Act supporters

✽  As of March 3, 2017.  H.R. 1223 has been referred to the Subcommittee on Health

✽  As of June 29, 2017. Senate Bill 1509 has been read twice and referred to the Committee on Health, Education, Labor, and Pensions.

According to Stephanie Fischer, Senior Director, Patient Engagement and Communications, neither bill has been marked-up or the subject of a hearing. The health committees have been busy with healthcare reform and FDA user fee reauthorization.

OPEN ACT — H.R. 1223 and Senate Bill 1509

Shipping Blood Samples — Dallas to Philly

From the Editor…

On July 25, 2017, I received a message from Dr. Nick Ross at Thomas Jefferson University. He reminded me that I had not sent in my blood samples for the ongoing PRP Research Study. I was mortified. Nah. Actually, at 71, very little mortifies me. At best I was embarrassed that yet another senior moment had become public. I told Nick I would submit the blood samples tomorrow. Sometimes “tomorrow” means nine days later. Oops.


On Thursday, August 3rd, I did a little “recon” in the area near my home to evaluate the available shipping options. I started with FedEx (Preston and Highland). They wanted to charge me $30 and offered no help or insights. In fact, the sales person seemed concerned that I might be dealing with bio-hazard materials. Imagine her reaction if I had been standing there four years ago in my radiant glory — a six-foot, three-inch, 250-pound shedding strawberry. Forget FedEx.

My next stop was the UPS Store (Park and Preston). The owner recognized me and we chatted. The shipping of blood samples brought a look that we see when a clueless healthcare professional can’t figure out what the heck is going on with our skin. Terminal Befuddlement Syndrome (TBS). When he told me it would cost $70 to ship my blood samples to Philadelphia, I told him, “See ya in December.”

That left the U.S. Postal Service. I told Mike, the postal clerk, what I needed and he handed me the Express Mail box, and Express Mail envelope, the Express Mail form and said “$28.50 and it will be there by noon if you bring it back here by 3:00 PM. No problem. I walked out of the post office knowing that my blood samples would be in Philadelphia by noon, the next day.


I didn’t make an appointment with my family doctor. In sales it is called a “Cold Call.” I asked for Charlene, the Office Manager and explained my need for blood samples to her. She went “backstage” to check with Dr. Kaplan while I sat in the waiting room. In less than five minutes I was sitting in a different chair and having two “lavender” tubes filled with my blood. I was given the two vials and a biomedical bag. On the way out I paid $5 for the “procedure” and took two lollipops for being a good boy.

It should be noted that Dr. Adam Kaplan, a General Practitioner, is well versed on the subject of PRP and quite familiar with the ongoing research at Thomas Jefferson University.


The packaging of the blood samples was a no-brainer. Each vial was wrapped separately in a paper towel. I recommend Brawny because of the lumberjack on the package. Each vial/towel was then rolled in a SMALL sheet of bubble wrap. And finally, both vial/towel/bubble wrap units were put in a waterproof biomedical bag and stuffed into the Express Mail box,

I filled out the Express Mail form and returned to the Post Office. The postal clerk put the Express Mail box into the Express Mail envelope and affixed the form. I received the receipt and tracking number and paid $28.50.

I hope EVERY PRP patient in the US will consider or reconsider participating in the ongoing PRP research underway at Thomas Jefferson University. I urge ALL those who have submitted their blood samples in support of the PRP Research Study to share your reasons.

Shipping Blood Samples — Dallas to Philly

Another Biological Approved for Psoriasis

From the Editor…

Janssen Announces U.S. FDA Approval of TREMFYA™ (Guselkumab) for the Treatment of Moderate to Severe Plaque Psoriasis

The following message was posted by Ginny M (Lexington, SC) to the PRP Facebook Community (08/02/2017). “My dermatologist mentioned this new biologic to me last week. I have been on Stelara for seven years and had to increase to 90mg recently. The side effects are weighing me down. I can’t imagine getting insurance approval for Tremfya™ any time soon.

The news spread to Ringwood, NJ.

Jan T — Ringwood, NJ
Tremfya has been studied in a head-to-head study with Humira. It is effective in patients who did not respond well to Stelara (which is also made by Janssen) Tremfya specifically targets IL-23 like Stelara does. head-to-head comparisons will be very useful.Ginny M
Great summary of the article!Jan T
My insurance won’t put a drug on its formulary until it’s been on the market a year (and probably wouldn’t approve this off-label anyway.)

Ginny M
I was in the same boat with Stelara eight years ago.

Jan T
It’s a tough situation. Part of me wishes the earlier cheaper drugs worked better and without the effects on the liver. Often the old tried-and-true drugs actually are just as good (and certainly cheaper) than the new highly touted drugs. New isn’t always better. Still, if a doctor and patient feel the drug is worth a try, the insurance companies should approve. Proper treatment saves money in the long run.

Ginny M
Methotrexate worked well for me for many years. However, the side effects, blood work every eight weeks and three liver biopsies were alot. Remicade worked well, but those side effects were over the top. So I am a huge fan of the new biologic drugs. They are narrowing in and targeting specific areas. I did very well on 45mg for 6 years but the 90mg of Stelara is too much.

Jan T
I’m going to read up on this one in the journals. Obviously the market for psoriasis drugs is very strong.

Carol L — Jacksonville, FL
Since there’s now something new on the market, maybe you will be allowed to try TALTZ. It’s been miraculous for me ! 1 and 1/2 years of almost complete clearing. Isolated light outbreaks on chest, and of course hands and feet never have been completely clear.

Jan T
More specific than Stelara in targeting the immune pathway thought to be involved in psoriasis. (Humira has a different mechanism of action than either of those.) More specificity means Tremfya will have less of an impact on immune system functions that are not thought to be involved in psoriasis. However, and this is my thought: how close is PRP to psoriasis with respect to the immune system pathways involved? A target more specific in the treatment of psoriasis may or may not translate to better treatment for PRP. Fingers crossed.


Another Biological Approved for Psoriasis

Daily Life Chat — Setbacks

From the Editor…

Members of the PRP Facebook and RareConnect communities understand the importance of a support group. The following is an exchange between 16 members of the PRP Facebook Support Group.

Nana B — New York City
This week has been a nightmare for me. Just when I think I’m getting better, boom, everything falls apart. My skin is unbearably itchy. I am hot and red one moment and freezing cold the next. My fingernails and fingertips hurt so much I can’t hold a glass or a cup for too long. My feet and ankles are so swollen that it is difficult to walk. I’m trying so hard to stay positive but it is very difficult.

Martin W — Wolverhampton, England, UK
I know but we have to keep going. I know it’s so frustrating. How long have you had PRP? What improvements have you had so far?

Nana B
5 months. I’ve been on methotraxate for 2 months. I was approved for the injection drugs, but the copays are way out of my reach and I don’t qualify for assistance since I am on Medicare which makes no sense. My skin is not as red and my hands were showing soft skin. All of that has changed now.

Dolores P — Montreal, Quebec, Canada
I know the feeling , after 8 months taking the first medications my skin was getting better but was affecting my organs so the doctors had to change my med , so everything went back to the beginning , all the improvements was gone.

Deborah B — Kannapolis, NC
Have a good cry or fit….. get the grief, fear, pain, anger out if your system!! Not good to hold the emotions inside (or so I am learning!). Then things should settle down inside your mind, body, spirit. Glad you are in this group. Glad you are sharing.

Nana B
Thank you so much. I did have a good cry last night.

Eileen S — Philadelphia, PA
It’s a constant struggle.

Tami C — Minnesota
I know how you feel.

Sonnia R — Ontario, Canada
So sorry …the up and down cycle of this condition can be depressing ! I know some of us, me included have found relief from extreme itching fits by taking an over the counter allergy medication. I took Benadryl…if your on other meds perhaps ask your pharmacist if this OK for you. If your feet are puffy try to sit with them elevated for a while to get some relief….

Nana B
I live on Benadryl. It’s the new M&M’s. lol

Anita R
— Pearl, MS
So sorry it got worse. Took me 18 months to get better. Pray yours won’t take that long.

Jan T — Ringwood, NJ
That was me up until about a month ago! I’m a lot better and I hope you will be too! Hang in there!!

Richard L — Lansdale, PA
Likewise Nana. The endless cycle of good and bad days keeps chipping away at optimism and revives and deepens the depression.

Carol L — Jacksonville, FL
Thinking of you, we have all been there! Try very hard to focus on you, and feeling better. It’s important that you take each day at a time, and remember to moisturize! Reach out to any of us, we are here for each other

Alan B — Lakeland, FL
I’ve been there with you. The itching was maddening at one point … I used Serna lotion (with menthol camphor )and placed in refrigerator also used some gel cooling pads I got from my wonderful sisnlaw and laid them on itchy areas … if you’re not already on antidepressants ask your doctor about Doxepin it’s mild helps with itching and is suppose to help you sleep too. Have faith and know you will get better everyday one step closer to healing.

Vivienne P — Australia
Nana Bendana so sorry you are having a set back. It’s really maddening just when things were improving. I found oat baths helped me with the itching. It can’t hurt and is worth a try.

Bronagh M — Belfast, Northern Ireland, UK
Hang in there nana you will get there just try and be patient .

Debbie S  — Casper, WY
Ohhhh, I feel for you. Hopefully this will pass soon! My hubby in hospital for swelling, what a trip we are on!

Anita P — Ohio
I’m so glad I found this group… It wonderful to know that I’m not alone in this journey but saddened too. Why me/us? It seems like a great place to jus t say HEY!

The unedited exchange  between Nancy B and her fellow PRP Facebookers may be accessed by fellow members o of the PRP Facebook Community at https://www.facebook.com/groups/15865278115/permalink/10155382765638116/


Daily Life Chat — Setbacks

2017 PRP Resources Survey

From the Editor…

Several members of the PRP Facebook Support Group have recently attempted to conduct surveys using the Facebook polling feature. They — and many others over the past four years — are to be applauded for their curiosity and initiative. No matter how basic or complex the poll or survey, there are three basic tasks to perform.

SELECT the appropriate topic
When it comes to PRP, selecting the right topic is easy. Just about any topic applies to someone in our PRP family.

ASK the right questions. Asking the right questions can be a challenge. I have posted a number of surveys in the past four years and almost immediately wish I had asked one or two additional questions.

USE the information gathered Using the information is the easiest part of the equation. All the findings can be published in the PRP Survival Guide and shared with PRP patients, caregivers and the healthcare professionals who manage our treatment.

PRP Resources Survey

The PRP Resources Survey has concluded and here are the results:

(1)  To which support group(s) do you currently belong? (123 responses)

 PRP Facebook Support Group: 120 — 97.5%
✽  PRP Community on RareConnect: 44 — 35.7%
✽  PRP Support Group: 32 — 26.0 %

Objective: Increase the number of PRP patients and caregivers who are members of both the PRP Facebook and RareConnect communities. How close can we get to 66%

(2)  Have you visited the new PRP Survival Guide? (124 responses)

✽  Yes: 44 — 34.3%
✽  No: 80 — 62.5%

Objective:  Find ways to increase traffic from the PRP Facebook and RareConnect communities  to the PRP Survival Guide. A major push is in the works for PRP Awareness Month (November 2017)

(3) The PRP Survival Guide is divided into eight chapters. Please indicate the importance or relevance to each chapter to you.

 Important:  Chapters 5 (PRP Parents and Kids) scored lowest because PRP patients diagnosed with Adult Onset have no interest in issues related to those diagnosed with Juvenile onset. Conversely, all the other chapters are of interest to PRP parents and their children.
✽  Very Important:  Chapters 1, 2
✽  Critically Important: Chapters 3, 4, 6, 7, 8

Objective: Ensure that all eight chapters are developed as co-equals in importance.

The PRP Survival Guide is now working with Constant Contact to access their online marketing tools and other resources, including survey support. What topics would you like surveyed in the future?

2017 PRP Resources Survey 

PRP Community Update: August 1, 2017

From the Editor…

While the “harvesting” of information about PRP patients began in May 2013, it wasn’t until the following November when the first PRP Worldwide Census was undertaken.

Collecting “core data” from PRP patients has always been a challenge. Over the past four years, the following data has be harvested from PRP patient and caregivers. Over half the following “core data” was entered by PRP patients and caregivers on census forms and surveys. The remaining data was extracted from PRP Facebook posts and comments. (updated August 1, 2017)

✽  PRP Facebook Support Group:  1,023 members
✽  PRP Community on RareConnect: 207 members
✽  Nonaligned: 609

PRP Facebook Posts
✽  Total posts: 174
✽  July 1-15: 89
✽  July 16-31: 84

✽  US: 674
✽  International: 527
✽  N/A: 350

Core Data
✽  Onset date: 718
✽  Onset Age: 655
✽  Diagnosis date: 698
✽  Current status: 660 (active: 397; remission: 263)
✽  Complete core data snapshot: 509
✽  No data:  287 (name only)

PRP and Diagnostic Coding

From the Editor…

When the clinical observations of a dermatologist are supported by a biopsy prepared by a dermatopathologist, a diagnosis of pityriasis rubra pilaris is  rendered and the diagnostic code L44.0 entered into the patient’s medical record.

The International Classification of Diseases (ICD) is an essential element in every medical record. The ICD code is important for health insurance reimbursement, administration, epidemiology (the cause, incidence, distribution and control of diseases in specific populations).   Of the approximately 7,000 rare diseases, only about 500 have a specific ICD code. (1)

Codification is a process where a disease is assigned an individual code so that it can be easily recognized within a health information system. “A health information system is a group of IT tools, databases and procedures related to patient cases and diseases and is used to make decisions to improve treatment, research, care and healthcare management. Codification allows healthcare professionals to store and search for disease information in a faster and easier way.” (2)

“Effective codification can make it easier to identify and diagnose a patient’s disease. Effective codification of each individual rare disease can help to ensure that no rare disease, and as a result no rare disease patient, is lost within a healthcare system. The data retrieved through codification can also be used in much-needed rare disease clinical research and also for national healthcare services to better perform epidemiological studies and to better plan their services.” (2)

The ICD-10 code for pityriasis rubra pilaris is L44.0

“Different versions of the ICD code exist worldwide. The United States currently uses the ICD-10-CM, a Clinical Modification of the WHO (World Health Organization) standard for diagnoses adapted for insurance reimbursement and billing purposes. This version allows for further breakdown of a code, which increases diagnosis specificity. (1)

The ICD-10 codes for diseases of the skin and subcutaneous tissue fall in the bucket labeled “L00-L99. Most recognizable to the PRP community would be our kindred spirits diagnosed with dermatitis and eczema (L2 to L30) and psoriasis (L40.0). (3)

Pityriasis rubra pilaris falls in the category of “Other papulosquamous disorders”. A papulosquamous disorder is a condition which presents with both papules and scales, or both scaly papules and plaques. (3)

More information in the PRP Survival Guide about PRP and Diagnostic Codes
✽    ICD Coding for Rare Diseases from GARD
✽    Does your rare disease have a code?
✽    Were dermatologists ready for ICD-10?

(1) Genetic and Rare Diseases Information Center, Coding for Rare Diseases, updated 6/24/2015; https://rarediseases.info.nih.gov/guides/pages/123/icd-coding-for-rare-diseases

(2)   Does Your Rare Disease Have a Code?, Eva Berryman, Junior Communications Manager, EURORDIS, http://www.eurordis.org/news/does-your-rare-disease-have-code

(3) ICD10Data.com; http://www.icd10data.com/ICD10CM/Codes/L00-L99/L40-L45/L44-/L44.0

Itch Relief in the Wild

From the Editor…

There are many ways to find itch relief. The following video, posted by Jan T (Ringwood, NJ), illustrates the legendary Vertical Tree Rub (VTR).

PRP & Nurse Case Managers

From the Editor…

I received a call from Carla, a heath care professional who identified herself as a Nurse Case Manager for BlueCross Blue Shield of Arizona. She was calling on behalf of a patient insured by BCBS who was recently diagnosed with pityriasis rubra pilaris. With just a hint of desperation she asked if there are ANY resources available to her the patient. I think my response went something like this: “Carla, you have stumbled upon the Mother Lode of PRP support.” She received an informed overview of PRP resources that included:
✽ PRP Facebook Support Group
✽ PRP Community on RareConnect
✽ PRP Survival Guide
✽ PRP Alliance, Inc.
Then it was my turn to ask Carla some questions. She told me that every insurance provider has Nurse Care Managers to help patients navigate benefits, treatment options, etc. Then and there I decided that the PRP community should learn more about Nurse Case Managers — and Nurse Case Managers at insurance companies should know more about us.

From CMSA …

“Today, more than 100,000 case managers practice in the United States, most of them nurses. That’s a tenfold increase from 1990, when there were 10,000 case managers. This profession is appealing to many nurses because it allows them to use their critical thinking skills and act as passionate patient advocates.” (1)

Since 1990, the Case Management Society of America, which has 7,500 members in the United States, has been the official organization for case managers in the United States and abroad.  (1)

According to CMSA, “case managers are advocates who help patients understand their current health status, what they can do about it and why those treatments are important.  In this way, care managers are catalysts by guiding patients and providing cohesion to other professionals in the health care delivery team, enabling their clients to achieve goals more effectively and efficiently. (2)

ED. NOTE: There are CMSA chapters throughout the U.S. The Dallas/Fort Worth Chapter is on my radar.

From Nursing Explorer …

Nursing Explorer is an education and career resource website created to provide comprehensive and up-to-date information for nursing students and professional nurses.

According to NursingExplorer.com, an education and career resource website created to provide comprehensive and up-to-date information for case management nurses are registered nurses who

✽  coordinate all aspects of the care of individual patients
✽  ensure proper utilization of services and resources
✽  provide assistance within, between, and outside of facilities
✽  obtaining resources
✽  work with patients, families and other professionals
✽  assess, plan, implement, and evaluate patient care and the use of resources. ✽  they monitor quality of care to ensure that infection control (3)

From Nurses.com …

In a recent interview, Kathleen Moreo, RN, and president of the Case Management Society of America, describes this dynamic profession and offers advice for how nurses can join this exciting field.

Q.  Why are more nurses seeking to become case managers?

A.  In today’s healthcare environment, nurses see patients who are lost in the healthcare maze and find that no one is addressing family dynamics.

Q.  What attributes do the best case managers possess?

A.  First and foremost, the best case manager will be an excellent patient advocate.  A nurse who is a good educator will also do well as a case manager because educating patients and their families to empower them is a big part of what we do.

Collaboration is another big attribute. Case managers must know how to get physicians and other members of the healthcare team to work together with them.

Q.  What is the definition of case management?

A.  Case management is a collaborative process which assesses, plans, implements, coordinates, monitors and evaluates options and services to meet an individual’s health needs through communication and available resources to promote quality, cost-effective outcomes. 

Q.  In what kinds of settings do case managers work?

A.  Some work over the phone; others are out in the community visiting patients’ home and meeting with their families. Some work for insurance companies or a vast array of providers; still others are independent contractors. (4)

From VeryWell.com …

Has your health insurance company assigned you a case manager?

“A health insurance company’s case manager receives information from hospital case managers, home health care companies, physician’s offices, social workers and other health care providers. Depending on the insurer and the location, nurse case manager may even visit a patient in the hospital.

Is the patient getting medically necessary care, quality care, and that the care is being delivered as efficiently and economically as possible.? The goal is to  anticipate the patient’s future health care needs and try to put in place mechanisms to meet those needs as efficiently as possible.” (5)

Path Forward

Here are some questions for the PRP community to pander:

✽  Since every insurance provider has case managers in their employ, how many PRP patients/caregiver have actually been assigned a nurse case manager? Sounds like we need to ask. A survey?

✽  If there are over 100,000 case managers in the U.S., how many are as smart as Carla at BlueCross BlueShield in Arizona  who found the PRP Alliance in the NORD rare disease database? Since the PRP Alliance is a member of the National Organization of Rare Disorders, should we take steps to inspire to NORD to consider an advocacy effort to educate CSMA’s 100,000 members?

(1)  Case Management Society of America; http://www.cmsa.org

(2)  Case Management Society of America; http://www.cmsa.org

(3)  Nursing Explorer ; http://www.nursingexplorer.com/careers/case-management-nurse

(4)  Professional Perspectives: Case Management–Coordinating Care from Start to Finish; Kelly Healthcare Resources, Larisa Hubbs, author;   https://www.nurses.com/doc/professional-perspectives-case-management-coo-0001

(5)  VeryWel.com; What does a case manager do?; https://www.verywell.com/what-does-a-case-manager-do-1738560

PRP & Nurse Case Managers