Disease Information for Pityriasis rubra pilaris

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Clinical Manifestations
Signs & Symptoms
Facial Malar flush/rash
Red/erythema skin discoloration
Dandruff
Erythema with skip areas/sign
Erythematous generalized rash
Erythroderma
Facial erythema
Facial rash
Fissuring palms/soles
Follicular hyperkeratosis
Hyperkeratosis/palms and soles
Koebner phenomenon/post-mechanical stimulation/rash lesion
Papule
Papulosquamous Lesion
Papulosquamous rash
Pruritic/itching rash
Rash
Rash hand/foot areas
Rash, erythematous/Sunburn-like
Scaling/Skin finding
Scalp rash
Scalp round scaly patches/broken hairs
Associated Diseases & Rule outs
Associated Disease & Complications
Neonatal Erythroderma
Pityriasis rubra pilaris
Seborrhea/Seborrhea dermatitis
Disease Mechanism & Classification
Class
CLASS/Primary organ/system disorder (ex)
CLASS/Dermatologic/Subcutaneous (category)
CLASS/Skin disorder (ex)
Process
PROCESS/Idiopathic/unclassified/unknown (category)
PROCESS/Reference organ/system (category)
PROCESS/Uncommon disease
Treatment
Drug Therapy - Indication
RX/Etretinate (Tegison)
RX/Isotretinoin (Accutane)
RX/Oral Retinoids
Definition

A chronic skin disease characterized by small follicular papules, disseminated reddish-brown scaly patches, and often, palmoplantar hyperkeratosis; The papules are about the size of a pin and topped by a horny plug;-------------------------------- Pityriasis rubra pilaris (PRP) is a rare condition that is often initially mistaken for another skin disorder, usually psoriasis; PRP is not really a single condition, but rather a group of unusual eruptions that cause red scaly patches containing dry plugged pores; It may cover the entire body, or just the elbows and knees;

PRP mostly affects adults over 40, but some children are also affected; The cause of PRP is unknown; 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; 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 ("salmon") 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 later is not as bad as you would think considering how bad the rash looks; 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; [AOL website 2005]

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External Links Related to Pityriasis rubra pilaris
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Wikipedia
Merck
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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