Pityriasis rubra pilaris
Encyclopedia
Pityriasis rubra pilaris (also known as "Devergie's disease," "Lichen ruber acuminatus," and "Lichen ruber pilaris") refers to a group of chronic disorders characterized by reddish orange, scaling plaques and keratotic follicular papule
s. Symptoms may include reddish-orange 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 follicle
s (Latin: pilus for hair). For some, early symptoms may also include generalized swelling of the legs, feet and other parts of the body. PRP has a varied clinical progression and a varied rate of improvement. There is no known cause or cure.
It was first described by Marie-Guillaume-Alphonse Devergie
in 1856, and the condition is also known as Devergie's disease.
, classified six forms of PRP in the early 1980s. 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
(familial) 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 50, individuals of any age, race, and nationality can be affected. Women and men seem to be equally affected.
Papule
A papule is a circumscribed, solid elevation of skin with no visible fluid, varying in size from a pinhead to 1 cm.With regard to the quote "...varying in size from a pinhead to 1cm," depending on which text is referenced, some authors state the cutoff between a papule and a plaque as 0.5cm,...
s. Symptoms may include reddish-orange patches (Latin: rubra) on the skin, severe flaking (Latin: pityriasis
Pityriasis
Pityriasis commonly refers to flaking of the skin. The word comes from the Greek πίτυρον "bran".-Classification:Types include:* Pityriasis alba* Pityriasis lichenoides chronica* Pityriasis lichenoides et varioliformis acuta...
), uncomfortable itching, thickening of the skin on the feet and hands, and thickened bumps around hair follicle
Hair follicle
A hair follicle is a skin organ that produces hair. Hair production occurs in phases, including a growth phase , and cessation phase , and a rest phase . Stem cells are principally responsible for the production of hair....
s (Latin: pilus for hair). For some, early symptoms may also include generalized swelling of the legs, feet and other parts of the body. PRP has a varied clinical progression and a varied rate of improvement. There is no known cause or cure.
It was first described by Marie-Guillaume-Alphonse Devergie
Marie-Guillaume-Alphonse Devergie
Marie-Guillaume-Alphonse Devergie was a French dermatologist who was born in Paris. In 1834 he became a physician of Parisian hospitals , and in 1840 succeeded Laurent-Théodore Biett at the Hôpital Saint-Louis, where he practiced medicine until his retirement...
in 1856, and the condition is also known as Devergie's disease.
Classification
Dr. W.A.D. Griffiths, from Great BritainGreat Britain
Great Britain or Britain is an island situated to the northwest of Continental Europe. It is the ninth largest island in the world, and the largest European island, as well as the largest of the British Isles...
, classified six forms of PRP in the early 1980s. 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
Genetic disorder
A genetic disorder is an illness caused by abnormalities in genes or chromosomes, especially a condition that is present from before birth. Most genetic disorders are quite rare and affect one person in every several thousands or millions....
(familial) 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 50, individuals of any age, race, and nationality can be affected. Women and men seem to be equally affected.
See also
- List of cutaneous conditions
- American Osteopathic Dermatology content: http://www.aocd.org/skin/dermatologic_diseases/pityriasis_rubra_p.html
External links
- PRP Support Group - information regarding day-to-day care and medical treatment and having an international membership.
- skin diseases Information