Pityriasis rubra pilaris (also known as PRB, Devergie's disease, Lichen ruber acuminatus, and Lichen ruber pilaris) is a chronic skin disorder characterized by reddish orange, scaling (exfoliation) and inflammation of the skin.
Symptoms may include reddish-orange, salmon-colored or pink patches (Latin-rubra) on the skin, severe flaking (Latin-pityriasis), uncomfortable itching, thickening of the skin on the feet and hands and thickened bumps around hair follicles (Latin-pilus for hair). The colored scaly areas cover much of the body. Small "islands" of normal skin are seen within the areas of pink scaly skin. PRP is not really a single condition but rather a group of unusual eruptions that cause red scaly patches containing dry plugged pores.
PRP most often starts as a patchy rash on the scalp, face or chest. Over a period as short a several weeks it extends downward and often covers much of the body. It spares areas of old scars and injuries and leaves small islands of entirely unaffected skin. Rough, dry plugs can be felt within the rash. The itching is usually severe at first and then later is not as bad as you would think considering how bad the rash looks.
For some, early symptoms may also include generalized swelling of the legs, feet and other parts of the body. There is no known cure.
Pityriasis rubra pilaris is often initially mistaken for another skin disorder, usually psoriasis. It may cover the entire body, or just the elbows and knees. There are many different types of pityriasis rubra pilaris. The cause is unknown, although genetic factors may play a role.
PRP mostly affects adults over 40, but some children are also affected. Sometimes minor burns rashes and infections seem to trigger it. There is no blood test for PRP. It is usually diagnosed when a dermatologist, suspecting the condition, does a biopsy and specifically asks it to be checked for PRP. Sometimes PRP is suspected only after the usual creams, pills and even ultraviolet light treatments used for skin conditions have no effect.
Topical creams containing urea or lactic acid may help. More commonly, treatment includes pills taken by mouth such as isotretinoin, acitretin, or methotrexate. The best treatment is Accutane or Soriatane pills. These are closely related "retinoid" medications. While these have many minor side effects, they do not usually cause any serious harm to the body. A more potent and more effective treatment is methotrexate tablets, but as these can have dangerous side effects they are saved for people who don't improve with the retinoids. Methotrexate may put the PRP into remission, so it goes away and stays away.
At this time, the causes of PRP are still unknown and symptoms can be difficult to diagnose. Frequently, more than one medical professional will be consulted before an accurate PRP diagnosis is made.
Dermatologists have identified both an acquired form and an inherited form of PRP and have described them in medical journals. The acquired form usually shows a spontaneous or gradual remission of symptoms within several years although long-term symptoms may continue for years. The inherited form starts early in childhood with persistent long-term symptoms into adulthood.
Although most people who develop PRP are over age 40, individuals of any age, race, and nationality can be affected. Women and men seem to be equally affected.
Sources:
http://www.nlm.nih.gov
http://www.aocd.org
http://www.aad.org
http://en.wikipedia.org
MC Ortega is the former publicist for the late Walter Payton, Coca-Cola and Dunkin’ Donuts. Ortega is a senior communications and messaging executive specializing in media relations, social media, program development and crisis communications. Also, Ortega is an avid traveler and international shopper. Ortega resides with her partner, Craig, dog, Fionne and extensive shoe collection. Ortega also enjoys jewelry design/production and flamenco dancing.