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Perniosis

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Copyright 1989, 1997, 2005

Synonyms of Perniosis

Disorder Subdivisions

General Discussion

Perniosis is an inflammatory disorder that is triggered by prolonged exposure to cold and damp (humid) conditions. It is a form of inflammation of the small blood vessels (vasculitis) and is characterized by painful, itchy, tender, skin lesions on the lower legs, hands, toes, feet, ears and face. The lesions usually last for two to three weeks. One form of the disorder affects the blood vessels of the thighs.

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 occurring when the weather is cold and the humidity is high; especially during the late fall and winter. It usually lasts for several weeks, but in some cases may persist even into the warmer months.

Perniosis of the thighs is a form of perniosis that more commonly affects young females 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 unclear. Cold weather causes the small veins and arteries close to the skin to tighten or constrict. When the tissues are rewarmed, blood leaks into the tissue and causes the skin to swell. The swelling irritates the nerves, and pain is the result. It is thought by some that the disorder may represent an allergic reaction or hypersensitivity to the cold. Prolonged exposure to the cold, insufficient protective clothing, and circulatory or cardiovascular diseases may also be causative factors. Some cases are believed to be caused by genetic factors. Other suspected causes include nutrition, local infection, hormonal changes, and other systemic diseases.

Affected Populations

Perniosis is seen more often in females than in males. Also, it is seen more often in women who are very thin. Several articles in the medical literature focus on cases of perniosis in anorexic women. Individuals with poor circulation and smokers are affected more frequently than the general population. The disorder is more common in damp climates than in dry ones. Symptoms usually begin before the age of 20 years.

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.)

Standard Therapies

Diagnosis
Diagnosis is the result of physical examination and the close examination of the process of rewarming.

Treatment
Treatment of perniosis consists of protection from cold and warming the affected areas slowly. Individuals with perniosis should avoid scratching or rubbing the affected area to 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.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Perniosis Resources

Organizations:

References

TEXTBOOKS
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:2452.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1656.

Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:350.

JOURNAL ARTICLES
lmahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. 2008 Apr;10(2):128-35.

Souwer IH, Robins LJ, Lagro-Janssen AL. Chilblains from the patient's perspective.
Eur J Gen Pract. 2007;13(3):159-60.

Patra AK, Das AL, Ramadasan P.
Diltiazem vs. nifedipine in chilblains: a clinical trial. Indian J Dermatol Venereol Leprol. 2003 May-Jun;69(3):209-11.

Long WB 3rd , Edlich RF, Winters KL, et al. Cold Injuries. J Long Term Eff Med Implants. 2005;15:67-78.

Price Rd, Murdoch DR. Perniosis (chilblains) of the thigh: report of five cases, including four following river crossings. High Alt Med Biol. 2001;2:535-38.

Cribier B, Djeridi N, Peltre B, et al. A histological and immunohistochemical study of chilblains. J Am Acad Dermatol. 2001;45:924-29.

Weston WL, Morelli JG. Childhood pernio and cryoproteins. Pediatr Drermatol. 2000;17:97-99.

FROM THE INTERNET
Maroon MS, Hensley D. Pernio. emediicne. Last Updated: August 20, 2002. 8pp.
www.emedicine.com/derm/topic322.htm

Zafren K. Cold hands and Feet. emedicine Consumer Health. ©2003. 7pp.
www.emedicinehealth.com/fulltext/6054.htm

Chilblains. New Zealand Dermatological Society. nd. 2pp.
www.dermnetnz.org/reactions/chilblains.html

Report last updated: 2008/04/25 00:00:00 GMT+0