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Pityriasis rubra pilaris

Show Alternative Names
PRP
Pityriasis pilaris
Lichen ruber acuminatus
Devergie disease

Pityriasis rubra pilaris (PRP) is a rare skin disorder that causes inflammation and scaling (exfoliation) of the skin.

Causes

There are several subtypes of PRP. The cause is unknown, although genetic factors and an abnormal immune response may be involved. One subtype is associated with HIV/AIDS.

Symptoms

PRP is a chronic skin condition in which orange or salmon-colored scaly patches with thick skin develop on the hands and feet.

The scaly areas may cover much of the body. Small islands of normal skin (called islands of sparing) are seen within the areas of the scaly skin. The scaly areas may be itchy. There may be changes in the nails.

PRP can be severe. Although it's not life threatening, PRP can greatly reduce quality of life and limit activities of daily living.

Exams and Tests

The health care provider will examine your skin. Diagnosis is usually made by the presence of the unique skin lesions. (A lesion is an abnormal area on the skin). The provider may take samples (biopsies) of the affected skin to confirm the diagnosis and rule out conditions that may look like PRP.

Treatment

Topical creams containing urea, lactic acid, retinoids, and steroids may help. More commonly, treatment includes pills taken by mouth such as isotretinoin, acitretin, or methotrexate. Exposure to ultraviolet light (light therapy) may also help. Medicines that affect the body's immune system are currently being studied and may be effective for PRP.

Support Groups

More information and support for people with PRP disorder and their families can be found at:

When to Contact a Medical Professional

Call your provider if you develop symptoms of PRP. Also call if you have the disorder and symptoms worsen.

Text only

Review Date: 4/14/2021

Reviewed By

Elika Hoss, MD, Senior Associate Consultant, Mayo Clinic, Scottsdale, AZ. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

References

James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Pityriasis rosea, pityriasis rubra pilaris, and other papulosquamous and hyperkeratotic diseases. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 13th ed. Philadelphia, PA: Elsevier; 2020:chap 11.

Patterson JW. Disorders of pigmentation. In: Patterson JW, ed. Weedon's Skin Pathology. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 11.

Disclaimer

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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Pityriasis rubra pilaris on the chest - Illustration Thumbnail

Pityriasis rubra pilaris on the chest

This person has pityriasis rubra pilaris on the chest, an uncommon skin condition characterized by salmon-colored patches with scaling (palmoplantar keratoderma). A characteristic sign of pityriasis rubra pilaris is plugging of the hair follicles with keratin. This picture illustrates areas of uninvolved skin (islands of sparing) surrounded by areas of redness (erythroderma).

Illustration

Pityriasis rubra pilaris on the feet - Illustration Thumbnail

Pityriasis rubra pilaris on the feet

This is a picture of pityriasis rubra pilaris on the feet. It is an uncommon skin condition characterized by thick scales and salmon-colored patches.

Illustration

Pityriasis rubra pilaris on the palms - Illustration Thumbnail

Pityriasis rubra pilaris on the palms

This is a picture of pityriasis rubra pilaris on the palms. This is an uncommon skin condition characterized by salmon-colored patches with scaling (palmoplantar keratoderma).

Illustration

Pityriasis rubra pilaris - close-up - Illustration Thumbnail

Pityriasis rubra pilaris - close-up

Pityriasis rubra pilaris is an uncommon skin condition. It is characterized by salmon-colored patches with scaling, clear areas of normal skin, and thick palms and soles. Hair follicles with keratin plugging is a characteristic sign. Typically the follicle is surrounded with a characteristic orange-red color.

Illustration

Pityriasis rubra pilaris on the chest - Illustration Thumbnail

Pityriasis rubra pilaris on the chest

This person has pityriasis rubra pilaris on the chest, an uncommon skin condition characterized by salmon-colored patches with scaling (palmoplantar keratoderma). A characteristic sign of pityriasis rubra pilaris is plugging of the hair follicles with keratin. This picture illustrates areas of uninvolved skin (islands of sparing) surrounded by areas of redness (erythroderma).

Illustration

Pityriasis rubra pilaris on the feet - Illustration Thumbnail

Pityriasis rubra pilaris on the feet

This is a picture of pityriasis rubra pilaris on the feet. It is an uncommon skin condition characterized by thick scales and salmon-colored patches.

Illustration

Pityriasis rubra pilaris on the palms - Illustration Thumbnail

Pityriasis rubra pilaris on the palms

This is a picture of pityriasis rubra pilaris on the palms. This is an uncommon skin condition characterized by salmon-colored patches with scaling (palmoplantar keratoderma).

Illustration

Pityriasis rubra pilaris - close-up - Illustration Thumbnail

Pityriasis rubra pilaris - close-up

Pityriasis rubra pilaris is an uncommon skin condition. It is characterized by salmon-colored patches with scaling, clear areas of normal skin, and thick palms and soles. Hair follicles with keratin plugging is a characteristic sign. Typically the follicle is surrounded with a characteristic orange-red color.

Illustration

 
 
 
 

 

 
 

 
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