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$Unique_ID{BRK04111}
$Pretitle{}
$Title{Pityriasis Rubra Pilaris}
$Subject{Pityriasis Rubra Pilaris Devergie Disease Lichen Acuminatus Lichen
Psoriasis Lichen Ruber Acuminatus Pityriasis Pilaris Lichen Planus Pityriasis
Rosea Psoriasis}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
475:
Pityriasis Rubra Pilaris
** IMPORTANT **
It is possible the main title of the article (Pityriasis Rubra Pilaris)
is not the name you expected. Please check the SYNONYMS listing on the next
page to find alternate names, disorder subdivisions, and related disorders
covered by this article.
Synonyms
Devergie Disease
Lichen Acuminatus
Lichen Psoriasis
Lichen Ruber Acuminatus
Pityriasis Pilaris
Information on the following disorders may be found in the Related
Disorders section of this report:
Lichen Planus
Pityriasis Rosea
Psoriasis
General Discussion
** IMPORTANT **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Pityriasis Rubra Pilaris is a mildly itchy chronic skin disorder which is
possibly caused by an inherited metabolic defect. Initially, the disorder is
characterized by elevated spots (papules) on the skin. These spots grow and
become connected, producing red plaques over large areas.
Symptoms
Pityriasis Rubra Pilaris is initially characterized by skin lesions described
as mildly itchy, sharply pointed, horn-like, brownish-red to rosy yellow-
colored papules. These papules usually occur on the back of the wrists, the
outside of the forearms, underarm folds, elbows, knees, backs of the hands,
and fingers. When the papules grow and connect together they produce dry,
scaly, rough, red plaques over large areas of the skin. Gray, brittle nails
and excessive oiliness of the glands on the scalp (seborrhea) and face may
also occur. Often the edge of the eyelids are turned outward (ectropion).
Causes
Pityriasis Rubra Pilaris is suspected to be a hereditary disorder transmitted
as a autosomal dominant trait. Symptoms may be triggered by a metabolic
defect. An acquired form of the disease also exists which may be caused by a
deficiency of vitamin-A. (Human traits including the classic genetic
diseases, are the product of the interaction of two genes for that condition,
one received from the father and one from the mother. In dominant disorders,
a single copy of the disease gene (received from either the mother or father)
will be expressed "dominating" the normal gene and resulting in appearance of
the disease. The risk of transmitting the disorder from affected parent to
offspring is 50% for each pregnancy regardless of the sex of the resulting
child.)
Affected Population
Pityriasis Rubra Pilaris is a rare disorder which affects children and
adults. Males and females are affected in equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to those of Pityriasis
Rubra Pilaris. Comparisons may be useful for a differential diagnosis:
Lichen Planus is a recurrent, itchy, inflammatory eruption characterized
by small separate, angular spots that may combine into rough scaly patches.
It is often accompanied by lesions in the mouth. Women are most commonly
affected, although some cases have begun during childhood. The exact cause
of this disorder is unknown, though some minerals such as bismuth, arsenic,
gold, or exposure to certain chemicals used in developing color photographs
may cause an eruption indistinguishable from Lichen Planus. (For more
information on this disorder, choose "Lichen Planus" as your search term in
the Rare Disease Database.)
Pityriasis Rosea is a self-limited, mild, inflammatory skin disorder
characterized by scaly lesions found most commonly on the trunk. The
disorder is possibly due to an unidentified infectious agent. It may occur
at any age but is seen most frequently in young adults. In temperate
climates, incidence is highest during spring and autumn.
Psoriasis is a common chronic and recurrent skin disorder characterized
by dry, well-circumscribed silvery gray scaling spots (papules) or plaques on
the scalp, elbows, knees and/or the buttocks. (For more information on this
disorder, choose "Psoriasis" as your search term in the Rare Disease
Database.)
Therapies: Standard
Pityriasis Rubra Pilaris may respond to treatment with vitamin-A, either
orally or in alcohol as an ointment. Retinoid drugs such as isotretinoin
which are chemically related to vitamin A, and the antineoplastic drug
methotrexate may also be prescribed. The lesions often get better or worse
without treatment, waxing and waning naturally.
DDAVP in the injectable form is standard treatment for von Willebrand
Disease. It is an antidiuretic peptide manufactured by Rorer
Pharmaceuticals.
Therapies: Investigational
Research on Pityriasis Rubis Pilaris is being conducted at New York
University Medical Center. For information about this research, please
contact:
Irwin M. Freedberg, M.D.
NYU Medical Center
Dept. of Dermatology
550 First Ave.
New York, NY 10016
(212) 340-5245
This disease entry is based upon medical information available through
January 1990. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Pityriasis Rubra Pilaris, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Foundation for Ichthyosis and Related Skin Types (F.I.R.S.T.)
P.O. Box 20921
Raleigh, NC 27619-0921
(919) 782-5728
(800) 545-3286
The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
For genetic information and genetic counseling referrals, please contact:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
CHILDHOOD-ONSET PITYRIASIS RUBRA PILARIS WITH IMMUNOLOGIC ABNORMALITIES: D.
Shvili, et al.; Pediatr Dermatol (May 1987: issue 4(1)). Pp. 21-23.
PITYRIASIS RUBRA PILARIS, VITAMIN A AND RETINOL-BINDING PROTEIN: A CASE
STUDY: P.C. van Voorst Vader, et al.; Acta Derm Venereol (Stockholm) (1984:
issue 64(5)). Pp. 430-432.
ISOTRETINOIN TREATMENT OF PITYRIASIS RUBRA PILARIS: C.H. Dicken; Journal
Am Acad Dermatol (February 1987: issue 16(2 Part 1)). Pp. 297-301.