What are the treatment options for PRP?


There is no cure for PRP. Therefore, the goal of treatment is to relieve the symptoms.

According to the National Organization of Rare Diseases: “PRP tends to follow a natural waxing and waning course, with episodes in which there is periodic worsening (exacerbation) or cessation (remission) of symptoms.”  Source: https://rarediseases.org/rare-diseases/pityriasis-rubra-pilaris/

From the PRP perspective, there are two major objectives in the treatment of pityriasis rubra pilaris:

✽  relieving symptoms as they present
✽  achieving long-term remission, if possible

The mantra heard within the PRP community is simple but deafening: “What works for one doesn’t work for all.”

TREATMENT OPTIONS

Oral retinoids are derivatives of vitamin A that slow the growth and shedding of skin cells.

 acitretin/Soriatane®
✽  isotretinoin/Accutane®

Immunosuppressants to slow down the body’s immune system. Often used when oral retinoids are ineffective.

✽  methotrexate (oral and injection)
 cyclosporine (oral and injection)

Biologicals with generally fewer side effects, targeted to reduce inflammation. These are injectable or intravenous (IV) medications that affect the immune system.

✽  adalimumab/Humira®
✽  etanercept/Enbrel®
 infliximab/Remicade®
✽  ustekinumab/Stelara®
✽  secukinumab/Cosentyx®
✽  apremilast/Otezla®

Ultraviolet light therapy — especially for Juvenile Onset PRP

Additional therapies

✽  topical creams and ointments
✽  Traditional Chinese Medicine

No meds at all is an option taken by PRP patients concerned with the negative impact of side effects.

OTHER CONSIDERATIONS

✽  Lab tests to monitor the effects of medications on the body and efficient management of drug side effects are important follow-up issues that will concern PRP patients and caregiver  who is under a doctor’s care.
✽  Access to treatment options varies based on age, geography and cost

See Chapter 3 — Treating PRP

SG 03.00.02

How bad is my version of PRP going to get?


Editor’s Note:  While all patients with PRP share a common core of symptoms, the specific symptoms that patients experience may vary dramatically.

PRP causes pink, red, or orange-red scaly patches on the skin — usually everywhere, but not always. But that is just the beginning.

Each PRP patient diagnosed with PRP — and their caregiver — must deal with a unique version of PRP. The intensity of each symptom can range from bothersome to beyond agonizing. How do we cope?

My father was a self-proclaimed optimistic fatalist. He would say, “Whatever is going to happen is going to happen — but it will turn out good in the end.” It worked in Vietnam, with two failed marriages and during my 20-month journey with PRP.

Members of the PRP Facebook and RareConnect communities have shared their stories. We gain strength from the courage of others. We take comfort in knowing that we are not alone on this journey.

There are two facts that seem irrefutable when one looks at the journey ahead with its challenges to body, mind and spirit.

First — no matter how bad you are feeling, someone else feels worse.

Second — there are countless stories of healing and hope. The light at the end of the proverbial tunnel are candles being held by hundreds of PRP patients and caregivers who have traveled the road you travel now.

As an online community we join hands — as best we can — and rejoice in whatever good news there is to share.

Whether a PRP patient or a caregiver, what has been the greatest challenge you have faced? What has helped you most on your PRP journey?

SG 03.00.01 

How can I be sure the diagnosis is correct?

When your dermatologist entered the diagnostic code L44.0 into your medical record, the diagnosis of pityriasis rubra pilaris became official.

If you now question the accuracy of that diagnosis — for any reason — ask your dermatologist to confirm the following:

What clinical observations were made that supported the “official” diagnosis of PRP?

What confirming statement did the dermatopathologist make in support of those clinical observations?

Food for Thought

Every so often a diagnosis of PRP is rescinded in favor of another malady. Just as psoriasis or dermatitis can mimic PRP and delay diagnosis and treatment, PRP can mask other diseases, including Sézary syndrome (Cutaneous T-cell Lymphoma). An adult diagnosed with PRP — unresponsive to standard PRP treatment protocols — should share those concerns with their dermatologist. A possible path forward: a biopsy to specifically ruled out CTCL.

Share your concerns about a misdiagnosis with your dermatologist.

SG 02.04.02

Why is it so difficult to diagnose PRP?

Editor’s Note — Dan M was diagnosed with PRP in July, 2015 and is currently in remission. He recently posted the following to fellow members of the PRP Facebook Support Group

“PRP can mimic other much more common skin  like contact dermatitis, eczema and psoriasis.  A biopsy can be helpful. Unfortunately, PRP is very rare so dermatologists do not recognize it easily. Moreover, dermatopathologists often do not diagnosis PRP under the microscope unless tipped off by the dermatologist who performed the biopsy that PRP is being considered as a possible diagnosis.

“More PRP diagnoses would be made if the physician doing the skin biopsy (1) knew what area would be best to biopsy, and (2) mentioned on the request form that they were suspicious of PRP.

“When I did my skin biopsy I was aware that including a follicular-based papule would increase the probability of getting the correct diagnosis.

“The dermatopathologist who looked at my biopsy called and thanked me for mentioning on the request form that I suspected PRP.  He was very frank in telling me that had I not done that, he would have said the biopsy was non-specific inflammation. He was not very familiar with PRP as it is a rare diagnosis. He went to his textbooks and reviewed what PRP skin biopsies look like and felt that my biopsy was classic for this condition.

“I suspect that a number of PRP skin biopsies are misdiagnosed from the pathologist not being informed that PRP was being considered as a possible diagnosis.”

Did your dermatologist instruct the dermatopathologist to specifically consider pityriasis rubra pilaris as a possible diagnosis?

SG 02.04.01

 

How do PRP symptoms progress?

The PRP timeline varies from sequential to haphazard. For some PRP patients the inflammation spreads like a wildfire. Others watch a slow, methodical movement from “hither to yon” where neither hither nor yon is predictable.
Here are some of the symptoms fellow PRP patients and their caregivers have reported.

✽  The skin on the palms of the hands and the soles of the feet may or may not become red, thick and cracked and may bleed.

✽  The scaly patches that often engulf the entire body may or may not include clear areas (islands of sparing).

✽  While all areas of the body are at risk, ears/hearing, eyes/vision, feet/mobility, hands/dexterity may become critical issues in any treatment.

✽  While the physical discomforts of pain and itching are ever-present at times, we find ways to suppress them, if only temporarily.

✽  Toenails and fingernails may thicken, disappear or remain unchanged.

✽  The scalp may or may not be a cause of distress.

✽  Hair loss, thermoregulation, sensitivity to sunlight, joint pain, the inability to sweat, fatigue and a loss of energy and weight gain/loss may be issues to challenge body, mind and spirit — or not.

The effect of all these symptoms can be depression. If the depression is debilitating, seek the help of a healthcare professional.

How did your symptoms progress? From where  to where — and over what time period?

SG 02.02.03

What are the symptoms of PRP?

When a dermatologist examines a patient who will eventually be diagnosed with PRP, they may see signs and symptoms that suggest a more common skin disorder. When that dermatologist combines medical training, clinical observations and biopsies and makes a diagnosis of psoriasis or atopic dermatitis, it is called a differential diagnosis — not a mistake. A differential diagnosis is how the medical community defines a diagnosis other than the one that is finally established.

The 2014 PRP Worldwide Census identified onset symptoms reported by 477 PRP patients. The range of symptoms — and where they appear — underscores how each case of PRP is as unique as a snowflake. For example:

Words used by PRP patients to describe the onset symptoms

✽  A total of 294 PRP patients used the term rash (201), spot (97) or blemish (19)

✽  A total of 70 used the term itchy, and 57 used patches, shedding, dandruff, bumps, sunburn and peeling.

Words used by PRP patients to describe location of symptoms

✽  Head (168) which includes: scalp, face, forehead, head, eyes, ears and nose

✽  Upper torso (86) which includes chest, back (36), shoulder and torso

✽  Upper extremities (66): hands, arms and fingers

✽  Lower extremities (38): feet and legs

Looking for more symptoms…

Sometimes a PRP patient or caregiver want to compare their version of PRP with others who share this über-rare skin disorder. A search of the internet offers a glimpse of the range of signs and symptoms that are consistent with pityriasis rubra pilaris and a wide range of other possible diagnoses.

Here is a sampling of the signs and symptoms that apply to PRP.

ONSET

❐     Onset of symptoms vary greatly between affected individuals

❐     Onset can be gradual or more rapid

❐     Redness and scaling of the face and scalp are often seen first, followed by redness and thickening of the palms and soles.

❐     Overall, the elbows, knees, backs of the hands and feet, and ankles are most commonly affected..

❐     Lesions may expand and coalesce and eventually cover the entire body.

PATCHES

❐     Color: pink, red or red-orange

❐     Itching

❐     Elbows, knees, hands and/or ankles

❐     Often there is a solitary lesion but within a few weeks multiple patches appear and they join together to form groups of reddish-orange lesions. Over a few weeks these spread downwards and may cover most of the body (erythroderma).

SKIN LESIONS

❐     skin lesions described as “sharply pointed, horn-like, brownish-red to rosy yellow- colored”

PAPULES

Note: a small, raised, solid pimple or swelling, often forming part of a rash on the skin and typically inflamed but not producing pus.

❐     Occur on the back of the wrists, the outside of the forearms, underarm folds, elbows, knees, backs of the hands, and/or fingers?

❐     Grow and connect together they produce dry, scaly, rough, red plaques over large areas of the skin.

RASH

❐     Red scaly rash on head, neck and upper trunk as a red scaly rash.

NAILS

❐     Gray, brittle nails

❐     Thickened and discolored at the free nail edge and may show linear black streaks (splinter hemorrhages).

EXCESSIVE OILINESS OF THE GLANDS

❐     On the scalp (seborrhea)

❐     On face

IMPAIRED VISION

❐     Edges of the eyelids are turned outward (ectropion).

PALMS OF HANDS & SOLES OF FEET

❐     Skin has become red and thickened.

❐     Palms and soles become thickened and yellow colored in PRP.

❐     Cracks may develop which can be painful and make walking and using the hands difficult

HAIR

❐     Hair may thin considerably.

SHIVERING

❐     Shivering, heat and fluid loss

ISLANDS OF SPARING

❐     Small islands of normal skin (islands of sparing) are seen within the areas of the scaly skin.

SYMPTOM Sources used in this post:

www.healthline.com/health/pityriasis-rubra-pilaris

https://rarediseases.org/rare-diseases/pityriasis-rubra-pilaris/

http://www.healthline.com/health/pityriasis-rubra-pilaris#causes3

http://www.dermnetnz.org/topics/pityriasis-rubra-pilaris/

https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris

http://www.bad.org.uk/shared/get-file.ashx?id=116&itemtype=document

https://medlineplus.gov/ency/article/001471.htm

SG 02.02.02

How is PRP diagnosed?

The rendering of an “official” diagnosis of pityriasis rubra pilaris requires two elements: clinical observation AND a biopsy that “supports” a diagnosis of PRP.

Clinical observations of a trained dermatologist

When a yet-to-be diagnosed PRP patient is seen by a dermatologist, the scope of the symptoms may vary from a small persistent spot on the forehead to a torso engulfed in inflammation. Even when PRP was immediately suspected, a dermatologist will be reluctant to enter the diagnostic code L44.0 on a medical chart and make the diagnosis official. A supporting biopsy is essential. [Link to 2017 ICD-10-CM, Diagnosis Code L44.0]

Supporting skin biopsy

In the case of PRP, biopsies are a diagnostic tool that typically “rules out” alternate skin maladies. Multiple biopsies are quite common. As the patient’s PRP symptoms become more recognizable, and the list of alternate diagnoses dwindles, the dermatologist may specifically instruct the dermatopathologist to “look for evidence of PRP”.

PRP patients and caregivers have yet to see a biopsy report that stated: Patient has pityriasis rubra pilaris. You can take it to the bank! Instead we read statements that are less absolute.

The biopsy concludes that the findings are “compatible with” or “support” the clinical observations of a dermatologist.

The “Official” PRP Diagnosis

Based on clinical observations and a supporting biopsy, the dermatologist enters the diagnostic code L44.0 into a medical chart. The journey continues.

There are so many variables impede a timely diagnosis of PRP.

What is the dermatologist’s experience with PRP?

✽  Saw it in med school in a text book
✽  Saw a patient during grand rounds
✽  Saw a patient being treated by another dermatologist

What symptoms are visible to the dermatologist?

✽  Within a week of onset, e.g., a dime-sized spot on a forehead
✽  A month after onset , e.g., side of face engulfed
✽  Inflammation that mirrors psoriasis or atopic dermatitis

How soon is a biopsy performed and what is the outcome?

✽  The biopsy “rules out” other skin maladies. no “smoking gun” to be found in a skin biopsy. There is no “Gotcha” moment.
✽  The biopsy concludes that the findings are “inconclusive with” or “do not support” the clinical observations of a dermatologist.
✽  The biopsy concludes that the findings are “compatible with” or “support” the clinical observations of a dermatologist and “clinical correlation” is recommended.

Misdiagnosis of PRP is a problem

The diagnosis of PRP is too often delayed when the presenting symptoms of red and itchy skin mimic the signs of psoriasis or eczema. Not only does the missed diagnosis delay proper treatment, but inappropriate treatments can also be initiated in the interim. The PRP Patient Registry currently identifies the misdiagnosis of 328 PRPers.

2013 PRP Biopsy Survey

During the summer of 2013, the survey of 487 PRPers gathered responses from 256 who detailed their biopsy experiences.

✽  Dx with no biopsy ordered: 7.8%
✽  Dx with no contradictory biopsy: 45.3%
✽  Biopsy supported Dx: 23.4%
✽  Dx with no confirming biopsy: 23.4%

Return to CHAPTER 2 — Diagnosing PRP INDEX

SG 02.02.00

 

What is the PRP patient population based on Type?

01.05.02  What is the PRP patient population?

Adult Onset PRP (60)

Type 1 — Classical Adult Onset PRP: 55

Type 2 — Atypical Adult Onset PRP: 5

Juvenile Onset PRP (40)

Type 3 — Classical Juvenile Onset PRP: 10

Type 4 — Circumscribed Juvenile Onset PRP: 25

Type 5 — Atypical Juvenile: 5

Other Onset PRP (trace)

Type 6 — HIV-Associated PRP

[  LINK to DermNetNZ chart  ]

http://www.dermnetnz.org/topics/pityriasis-rubra-pilaris