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$Unique_ID{BRK04098}
$Pretitle{}
$Title{Perniosis}
$Subject{Perniosis Chilblains Pernio Cold Induced Vascular Disease Erythema
Pernio Thigh Perniosis Vasculitis Raynaud's Syndrome Chilblain Lupus
Erythromatosus Urticaria Cold}
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
736:
Perniosis
** IMPORTANT **
It is possible that the main title of the article (Perniosis) is not the
name you expected. Please check the SYNONYM listing to find the alternate
names and disorder subdivisions covered by this article.
Synonyms
Chilblains
Pernio
Cold Induced Vascular Disease
Erythema, Pernio
Disorder Subdivisions
Thigh Perniosis
Information on the following diseases can be found in the Related
Disorders section of this report:
Vasculitis
Raynaud's Syndrome
Chilblain Lupus Erythromatosus
Urticaria, Cold
General Discussion
** REMINDER **
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.
Perniosis is a vascular disorder caused by prolonged exposure to cold
damp weather. It is characterized most often by skin lesions on the lower
legs, hands, toes, feet, ears and face.
Symptoms
Perniosis is an inflammation of the small blood vessels caused by an abnormal
reaction to the cold. It is characterized by a bluish-red discoloration of
the skin that can cause pain, intense itching, burning, and swelling of the
skin especially as the body becomes warmer. A dark-blue discoloration
(acrococyanosis) may be seen on the fingertips or extremities. The lesions
usually occur on the fingers, toes, lower legs, heels, ears and nose. In
severe cases there may be blister-like lesions (bullae) which may ulcerate if
rubbed or irritated. Perniosis is a seasonal disorder usually occuring when
the weather is cold and the humidity is high; late fall and winter. It
usually lasts for several weeks, but in some cases may persist even into the
warmer months.
Thigh Perniosis is a form of Perniosis that more commonly affects young
women who wear tight fitting pants. It is characterized by red or bluish
patches (plaques) on the skin. These plaques are distributed on the outside
of the thighs and can cause swelling, burning, itching and occasionally
ulceration. Exposure to a warm temperature and wearing looser fitting
thermal insulated clothing will usually relieve or avoid the symptoms of this
abnormal reaction to the cold.
Causes
The exact cause of Perniosis is unknown. It may be caused by an allergic
reaction or hypersensitivity to the cold. Prolonged exposure to the cold,
insufficient protective clothing, circulatory or cardiovascular diseases may
also be causative factors.
Affected Population
Perniosis is seen more often in females than in males. Children, adults with
poor circulation, and smokers are affected more often than other people. It
is more common in colder climates and rarely seen in the United States.
Related Disorders
Symptoms of the following disorders can be similar to those of Perniosis.
Comparisons may be useful for a differential diagnosis:
Raynaud's Disease (or Raynaud's Phenomenon) is a vascular disorder. It is
characterized by spasms of arterioles occuring with exposure to cold,
occuring especially in the fingers and toes. Occasionally other areas of the
body such as the nose and tongue may be affected. The intermittent attacks
of pallor or bluish color of the fingers or toes are precipitated by exposure
to cold and intensified by emotional upsets. Raynaud's may occur alone or be
secondary to other conditions. (For more information on this disorder,
choose "Raynaud" as your search term in the Rare Disease Database).
Chilblain Lupus Erythromatosus is a chronic and persistent form of Lupus
Erythromatosus. It may be preceded by facial lesions, but most commonly
affects the fingers, calves and heels of the feet. It is a disorder that
affects mostly women and may progress to Systemic Lupus Erythromatosus which
can affect the internal organs. (For more on this disorder, choose (Lupus"
as your search term in the Rare Disease Database.)
Vasculitis is a common disorder characterized by an inflammation of the
blood vessel walls. This inflammation causes a narrowing of the inside of
the vessel and can obstruct the flow of blood to the tissues (ischemia). The
lack of blood may cause damage to the tissues (necrosis), possible formation
of blood clots (thrombosis), or a weakening or ballooning which can possibly
cause a rupture of the vessel wall (aneurysm). (For more information on this
disorder, choose "Vasculitis" as your search term in the Rare Disease
Database.)
Cold Urticaria is a chronic, reactive skin disorder. It is probably the
most common form of physical urticaria. Major symptoms may include abnormal
reddening of the skin (erythema), hives, and itching after exposure of the
skin to cold temperatures. (For more information on this disorder, choose
"Urticaria, Cold" as your search term in the Rare Disease Database.)
Therapies: Standard
Treatment of Perniosis consists of warming the affected areas slowly and not
scratching or rubbing the affected skin. This will help avoid further damage
to the skin. Corticosteroid creams may help relieve the intense itching. The
calcium channel blocker drug nifedipine (Adalat) can be an effective
treatment in relieving the symptoms of cold related Perniosis. Other
treatment is symptomatic and supportive.
Therapies: Investigational
This disease entry is based upon medical information available through August
1989. 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 Perniosis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Heart, Lung and Blood Institute
9000 Rockville Pike
Bethesda, MD 20892
(301) 421-8453
References
THE MERCK MANUAL, 15th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck, Sharp &
Dohme Laboratories., 1987. Pp. 2361.
THE TREATMENT OF CHILBLAINS WITH NIFEDIPINE: THE RESULTS OF A PILOT
STUDY, A DOUBLE-BLIND PLACEBO-CONTROLLED RANDOMIZED STUDY AND A LONG-TERM
OPEN TRIAL. M. Rustin, et al.; BR J DERMATOL (February 1989, issue 120
(2)). Pp. 267.
CHRONIC PERNIO. A HISTORICAL PERSPECTIVE OF COLD-INDUCED VASCULAR
DISEASE. J. Jacob, et al.; ARCH INTERN MED, (August 1986, issue 146 (8)).
Pp. 1589-1592.