Q: I suffered from an orphan disease called Type 1 pityriasis rubra pilaris (PRP). I was the first case my dermatologist had seen since medical school. Because of a family history of psoriasis, it took weeks of useless (some deleterious) medications and a biopsy to confirm the diagnosis only to discover there is no real treatment.
My doctor prescribed methotrexate which did some good but Protopic ointment 0.1% really gave the most help. It took 2 ½ years to be declared in remission but two years later I still have slight outbreaks which are controlled by the Protopic. I also took antidepressant medication during the treatment phase.
My questions: 1) As a diabetic is there any connection between type 2 and the PRP? 2) I recently suffered a severe case of cellulitis. Was this connected more to the diabetes or the PRP or is there no connection between them? 3) Since I’m still having small localized outbreaks, should I be concnered about a recurrence of the condition? My dermatologist says no. 4) I’ve always been sun-sensitive but it’s reached the point of idiocy. Proper use of SPF 50 will only allow me 60 minutes of exposure without burning. Will this continue to worsen? 5) I found the treatment phase to be enervating and have really not returned to my previous levels of activity. This has not done my weight any good. Should I “buck-up” and try some (any) physical exercise? I tire very easily and sweating is detrimental. I break out in a PRP-like rash wherever sweat collects in the folds of my skin. I continue to treat this outbreaks with Protopic and use an absorbent powder in these areas. Do you think this wil continue or will I see relief in the future?
My dermatologist called recently to let me know he’s seen another case. This makes three in 12 years – practically an outbreak!
Thank you for any updates you can give me. Orphan diseases don’t get much newsprint and there are only a few websites that give much information.
A: An orphan disease is a rare disease that affects only a very small portion of the population.
Pityriasis rubra pilaris is a rare disease that causes constant inflammation and scaling (peeling) of the skin. The cause is unknown but it is thought that genetics may play a role. It affects men and women equally.
It can appear anywhere on the body with the hands and feet most affected. It presents with reddish orange discoloration. The skin of the soles of the feet and palms of the hands also becomes thickened. On the body, areas of normal skin may be seen within the discolored, inflammed, peeling patches.
There are six classifications of the PRP and include classic adult (type I), atypical adult (type II), classic juvenile (type III), circumscribed juvenile (type IV), atypical juvenile (type V), and the recently recognised, HIV associated (type VI). Type I (classic adult) is the most common form accounting for nearly 50% of all diagnosed cases. It is also the form with the best prognosis with approximately 80% of sufferers achieving remission within three years of onset.
Because of the relative rarity of PRP, more research is still needed and/or being conducted on possible treatment options. Emollients and urea or lactic acid topicals are helpful in reducing dryness and cracking of the affected areas. Methotrexate (which interferes with the growth of certain cells) and isotretinoin and acitretin (both forms of vitamin A) have been found to be beneficial. Calcipotriol, a vitamin D analogue, has been used successfully in three patients but more research is needed. Topical corticosteroid creams may be helpful in relieving symptoms on a short-term basis but the long-term benefits are not known. The Protopic you are currently using is a topical immunosuppressant.
To the best of my knowledge, neither type 2 diabetes nor cellulitis (a bacterial skin infection) are related to PRP, nor is cellulitis the result of diabetes.
I also don’t believe that your sweating is causing patches of PRP to appear, but rather that it is simply the affect of the sweat lying in your skin folds and causing irritation.
As for your energy level, it may be declining due to your weight gain and lack of exercise so I absolutely urge your to start a healthful diet (such as Dr. Gott’s No Flour, No Sugar Diet, of course!). This will kickstart your weight loss and hopefully return some of your energy.
You should also begin an exercise regimen that you can begin slowly, such as walking. Because you tire easily, start off by walking for five to 10 minutes at a time, a few times a day. As your body adjusts to the new activity, your endurance levels should rise and you can slowly increase the time you walk until you can walk for at least 30 minutes to an hour without stopping. You can then try incorporating jogging by jogging for 1-2 minutes and walking for the remainder of the 30 or 60 minute time period or riding a stationary bike. Try lifting some light weights. You can do this at home with items around your house such as gallon jugs of water filled to varying levels to adjust the weight. When you feel confident with that, try joining a gym or attending a weekly exercise class. Whatever you decide to do, you must start doing it now.
As for you worsening sun sensitivity, it may be a result of your PRP. Symptoms are know to worsen following sun exposure. I also suggest you take a look at any medications you are on to determine if one or more carries photo-sensitivity as a side effect.
Speak to your general physician about your concerns. He or she is your best option for information and can work with your other doctors (such as your dermatologist) to help you get the most out of your medical care. There is also an online support group at www.prp-support.org.
