Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Pityriasis rubra pilaris


Other Names for this Disease

  • Familial pityriasis rubra pilaris
  • Pityriasis rubra pilaris--familial type
  • PRP
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

Newline Maker

What are the signs and symptoms of pityriasis rubra pilaris?

Features of this condition vary greatly between affected individuals. The onset is gradual in the familial type and can be more rapid in the acquired type. Redness and scaling of the face and scalp are often seen first, followed by redness and thickening of the palms and soles.[1] Overall, the elbows, knees, backs of the hands and feet, and ankles are most commonly affected.[2] A more widespread eruption consisting of scaling orange-red plaques can be observed on the trunk and extremities. The lesions may expand and coalesce and eventually cover the entire body. When the disease becomes widespread, the nails, mucous membranes and eyes may be affected.[1] The familial type often persists throughout life, but the acquired form may have periods of remission (periods of time where symptoms improve or completely resolve).[1]
Last updated: 9/20/2010

The Human Phenotype Ontology provides the following list of signs and symptoms for Pityriasis rubra pilaris. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Irregular hyperpigmentation 90%
Palmoplantar keratoderma 90%
Abnormality of the fingernails 50%
Pruritus 50%
Abnormality of the oral cavity 7.5%
Eczema 7.5%
Ichthyosis 7.5%
Lichenification 7.5%
Neoplasm 7.5%
Pustule 7.5%
Autosomal dominant inheritance -
Subungual hyperkeratosis -

Last updated: 9/2/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Goldsmith LA, Baden HP. Pityriasis Rubra Pilaris. In: Freedberg, et al., ed. Fitzpatrick's Dermatology in General Medicine, 6th edition. New York, NY: McGraw-Hill; 2003;
  2. Sybert VP. Pityriasis Rubra Pilaris. Genetic Skin Disorders. New York, NY: Oxford University Press; 1997;


Other Names for this Disease
  • Familial pityriasis rubra pilaris
  • Pityriasis rubra pilaris--familial type
  • PRP
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.