$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.