Perniosis

Perniosis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Perniosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Chilblains
  • Cold Induced Vascular Disease
  • Erythema, Pernio
  • Pernio

Disorder Subdivisions

  • None

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.

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

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

Resources

NIH/National Heart, Lung and Blood Institute

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use . How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.