ON to DX Retrospective Study

Editor’s Note: (1)  Comments highlighted in boldface purple reflect  the first 100 responses to the Onset to Diagnosis Survey, (2) There are links to “selected comments”. (3) Question #8 has be “tweaked” for clarity.

YOU are being asked to make history.

For our global PRP community, history begins with a retrospective study from  the patient perspective … our perspective. Our focus in this specific study begins with the onset of PRP symptoms and ends with the diagnosis of pityriasis rubra pilaris.
We want you to look back on your PRP journey and share key datapoints regarding the onset of symptoms and the challenges of diagnosing PRP. In other words, what do you see in your rear view mirror?
As a participant in the PRP Onset to Diagnosis Retrospective Study you will be part of a 600-plus patient cohort — the largest gathering of PRP patients ever assembled for a retrospective (looking back) study.

What do we hope to prove?

(1)  A proper diagnosis of pityriasis rubra pilaris requires the clinical observations of a dermatologist supported by a biopsy report prepared by a dermatopathologist. Prove with data. ,
(2)  When PRP is suspected, the dermatologist should instruct the dermatopathologist to “consider PRP”.  Prove with data.
(3)  PRP is often misdiagnosed as psoriasis, eczema or a host of common skin disorders.  Prove with data.
(4)  The onset of PRP can begin anywhere on the body.  Prove with data.(
(5)  Once  PRP diagnosis is rendered, the dermatologist does not educate the PRP patient. Specifically, there is no referral to the National Organization Rare Disorders and/or the Genetic and Rare Diseases (GARD) Information Center.  Prove with data.
(6)  Dermatologists are unaware of the PRP Survival Guide and the PRP Facebook Support Group.  Prove with data.

When you click the link to the PRP Onset to Diagnosis Retrospective Study (end of this webpage), you will be asked 10 questions. Remember, YOU are the “expert witness” when it comes to detailing your own version of PRP journey,

(1)  Who is completing this questionnaire?
While the questions assume that you are the patient, we know that a caregiver (spouse, partner, parent of a minor child), or a supporter (other family member or friend) may be completing this questionnaire. Which of the three options below is most accurate.

UPDATE: Based on the first 100 respondents:

✔︎  92 patient ✔︎  8 caregivers

(2)  Basic information to get started

a.  Name and location of the PRP patient

Names of PRP patients are never published without prior authorization. Since its creation in 2013, PRP Global Database reports NUMBERS (how many this and that), but never NAMES (who).

The LOCATION is limited to City, State/Province and Country datapoints are used to document the location of PRP patients. Knowing your location is essential to tabulate country-by-country and state-by-state comparisons. We are NOT seeking street addresses, postal codes, or phone numbers.

b.  Valid email address of the person completing this survey

We require a single valid email address for two reasons: First, an email address is the only way we can verify that the information we receive is actually from either the patient or someone who represents the patient.  The second reason is to provide you with updates of the findings. Your email address will  never be shared with third parties –  including future PRP researchers.
UPDATE: Based on the first 100 respondents: 

✔︎  32 email addresses added to the PRP global database.
✔︎  USA (70): Top 2 states — California 11, Florida 7,
✔︎  International (30). Top 2 nations — United Kingdom (11), Canada 8

(3)   ONSET DATE: When did the symptoms of PRP first appear?

Because PRP patients know or remember their exact Onset Date, we only ask for the month and year. Do the best you can, e.g., August 2012, or even the “Summer of 2012”. Onset Date is used to calculate the elapsed time from (a) onset to diagnosis and (b) duration, e.g., onset to remission.
UPDATE: Based on the first 100 respondents:  
✔︎  Median onset date is 2017 
✔︎  1957-2015: 26 (Seasoned travelers)
✔︎  2016: 13
✔︎  2017: 12
✔︎  2018: 16
✔︎  2019: 17
✔︎  2020: 15

(4)   ONSET AGE: At what age did the symptoms of PRP first appear?

The Onset Age for those diagnosed with PRP should just indicate the age in years, e.g., 35, 66, 82, etc. 
However, the Onset Age for children under the age of four (birth to toddler) diagnosed with Juvenile Onset PRP should be more specific, e.g., at birth, at 5 months, at 2-1/2 years. We think that this level of detail for young children may be of interest to PRP researchers who focus on Juvenile Onset PRP.
Based on the first 100 respondents:
✔︎  Median onset age is 58
✔︎  Birth to 19: 9
✔︎  20 to 29: 2
✔︎  30 to 39: 1
✔︎  40 to 49: 16
✔︎  50 to 59: 28
✔︎  60 to 69: 32
✔︎  70 to 79: 10
✔︎  80 or older: 2

(5)   Describe the onset of your version of PRP?

 Specifically, what symptom  signaled that there was a skin problem in need of a healthcare professional? Where was the symptom located? Why did you seek out a healthcare professional?

EXAMPLE: The first PRP symptom to appear was a red spot, the size of a dime. It was located on my right forehead near the hairline. The “spot” did not concern me at first, but within a few weeks it was the size of a quarter. I made an appointment with the dermatologist who had been checking me for the early signs of skin cancer. 

(6)   Describe the FIRST intervention by a dermatologist.

For some PRP patients, the “first intervention” is a  family doctor or general practitioner who is predictably befuddled. At some point, however,  the general practitioner realizes that a referral to a dermatologist is appropriate. Others go straightaway to a dermatologist. What was your path to a dermatologist?

EXAMPLE: I went directly to the dermatologist who had been screening me for skin cancer for many years. She took one look at me and diagnosed “dermatitis”, prescribed a low dose of prednisone and said, “Come back in two weeks.” 

(7)  If PRP was NOT your original diagnosis, what alternative disorders were diagnosed? What did your dermatologist “think” was the skin disorder? Use “Comment” to add ANY misdiagnoses not listed.

● Psoriasis ●  Eczema ●  Seborrheic dermatitis ●  Drug reaction ●  Cutaneous T-cell Lymphoma ●  Virus/viral rash ●  Other (Specify)

Based on the first 100 respondents:

✔︎  Psoriasis: 36
✔︎  Atopic dermatitis (eczema):  14
✔︎  Other dermatitis: 26

The question pertaining to “alternative disorders” showed a predictable and lopsided inclusion of psoriasis, atopic dermatitis (eczema) and other types of dermatitis. The question has been modified for future participants accordingly.

(8)  How many biopsies did it take to get a diagnosis of PRP?

For purposes of this retrospective research study, a single biopsy may involve several tissue samples. Consider it a single medical event rather than the number of tissue samples.

Based on the first 100 respondents:

✔︎  Dx without a biopsy: 7
✔︎  Dx based on one biopsy: 37
✔︎ Dx based on two biopsies:  29
✔︎  Dx based on three biopsies: 14
✔︎  Dx based on four biopsies: 4
✔︎  Dx based on five or more biopsies: 9

Based on the first 100 respondents:

✔︎  Diagnosis supported by a biopsy: 83
✔︎  Diagnosis not supported by a biopsy: 17

The question pertaining to biopsies was poorly written. Simply stated, it was much too difficult to extract numbers to support the following assertion:

A proper PRP diagnosis must be based on  (a) the clinical observations of a dermatologist AND (b) a biopsy reviewed by a dermatopathologist, both of which support a diagnosis of pityriasis rubra pilaris.

(9)  Who was your diagnosing dermatologist?

Please use the “Comment” box to include the name, location, and contact information of the dermatologist or dermatologists who diagnosed PRP. Include website if available, See example:

Dr. Arturo Dominguez, MD University of Texas Southwestern Department of Dermatology Professional Office Building 2 5939 Harry Hines Blvd., 4th Floor, Suite 100 Dallas, Texas75390 Phone: 214-645-2400 https://utswmed.org/locations/pob2/dermatology-clinic/

Based on the first 100 respondents:

✔︎  A total of 97 respondents provided the name of the dermatologist who rendered the PRP diagnosis. Well done.

(10)  What did your dermatologist do to help you learn about PRP?

The general consensus within the PRP global community is that once the diagnosis of pityriasis rubra pilaris was confirmed by a dermatologist, we are expected to LEARN MORE on our own – often with little or no direction. The responses to this question will provide the metrics to document this shortcoming.

Based on the first 100 respondents:

It is clear that PRP patients and caregivers are “on our own” when it comes to learning about PRP. Documenting this fact will provide the basis for an outreach program to teaching hospitals and private dermatology clinics.