Pityriasis rubra pilaris (PRP) is a rare and chronic skin disorder that often has a sudden onset. Symptoms include reddish orange discoloration scaling, and severe flaking of the skin. Dr. W.A.D. Griffiths has classified six forms of PRP. At this time, the cause of PRP is unknown, although it is thought to be related to abnormal vitamin A metabolism, and a cure is also unknown. The disease affects persons of all ages, races, and nationalities. Both sexes are affected equally. Many support group members report a varied clinical progression of the disease. Pityriasis rubra pilaris is a skin disorder that causes constant inflammation and scaling of the skin. It is generally accompanied by elevated erythematous spots (papules) on the skin. The papules are the most important diagnostic feature, being more or less acuminate, reddish brown, about pinhead size, and topped by a central horny plug.
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. The rash has an orange-red color and the palms and soles become thickened. Rough, dry plugs can be felt within the rash. The itching is usually severe at first, and then slowly lessens in severity despite the persistence of the rash. 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 serious side effects including liver and blood toxicity, and are reserved for people who don't improve with the retinoids.
Pityriasis rubra pilaris is the name given to a group of rare skin disorders that present with reddish-orange coloured scaling patches with well defined borders. The disease may affect entire body or just restrict to some parts such as the elbows and knees, palms and soles. The disease generally produces elevated spots on the skin. These spots grow and become connected, producing red plaques over large areas containing dry plugged pores. It is found to be having association with abnormal metabolism of vitamin A. It could take as long as one to three years for an individual to fully recover from this skin disorder. It may occur in any race, and both male and female are equally affected.
The two most common forms of the disease are juvenile classic, characterized by autosomal dominant inheritance and onset in childhood, as compared to the adult classic, characterized by no apparent genetic transmission. Atypical forms exist in both age groups. Sunlight can trigger a flare. Diagnosis is by clinical appearance and may be supported by biopsy. Differential diagnosis includes seborrheic dermatitis (in children) and psoriasis when disease occurs on the scalp, elbows, and knees.
PRP is generally sporadic, but as mentioned above, the juvenile form has a genetic predisposition. It is also believed that certain fungal infections may contribute to PRP. Signs and symptoms include seborrhea of the scalp, scaling and lesions across the face, an orange-reddish rash, and severe pruritus (itchiness).
Treatments for PRP include steroid creams and ointments to treat the rash and severe itching. Methotrexate is a strong drug used for suppressing the immune system may be used as a treatment for pityriasis rubra pilaris, but has many toxicities. Accutane and Soriatane which are retinoid medications, remain the best medical treatment. There is also a derivative of vitamin A known as acitretin which is also useful in PRP but the treatment has some severe side effects.
Most importantly, this is a chronic skin disorder which may affect the quality of the patient's life, particularly because of the rash, scaling and itching affecting the face. This skin disorder requires the expertise of a dermatologist to diagnose this rash, prescribe the appropriate medications and monitor the patient for improvement and side effects.
