Vasculitis, Cutaneous

Vasculitis, Cutaneous

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Vasculitis, Cutaneous 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

  • Dermal Necrotizing Angiitis
  • Cutaneous Leukocytoclastic Angiitis
  • Hypersensitivity Vasculitis

Disorder Subdivisions

  • None

General Discussion

Cutaneous necrotizing vasculitis (CNV) is characterized by inflammation and tissue damage (necrosis) of blood vessel walls (lumen) and associated skin (cutaneous) lesions. CNV may be a primary disease process or occur as a result of, or in association with, a number of different underlying disorders (e.g., certain infections, certain autoimmune disorders) or other factors (e.g., allergic reaction or hypersensitivity to certain medications, toxins, or inhaled environmental irritants). It is important to determine whether there is an underlying disorder that leads to the CNV before treatment is started.



CNV is one of a larger group of disorders involving inflammation and blood vessels known as the vasculitides or the vasculitic syndromes. These syndromes range from modest disorders limited to the skin to more serious ones that may involve various organ systems.

Symptoms

Cutaneous necrotizing vasculitis is a not uncommon disorder characterized by an inflammation of the blood vessel walls and skin lesions. These skin lesions may be flat and red (macules), nodules or more substantial hemorrhages under the skin (purpura). They may occur on many areas of the body but are seen most often on the back, hands, buttocks, the inside areas of the forearm and the lower extremities. These skin symptoms may occur only once or at regular intervals. They will usually last for several weeks and may leave darkened spots and scarring. In some cases there may be wheel-like lesions that cause intense itching (urticaria), or ring-shaped lesions and ulcers. Blister-like lesions (vesicles, bullae) may develop in severe cases. There may also be fever, generalized discomfort (malaise), muscle or joint pain.

Causes

The exact cause of cutaneous necrotizing vasculitis is unknown. One review suggests that 45-55% of cases are of unknown origin (idiopathic), 15-20% of cases are a response to infection, another 15-20% are the result of connective tissue diseases, 10-15% are the result of reactions to drugs and/or medications, and about 5% are responses to the presence of cancer cells.



Some lesions may be caused by an allergic reaction or hypersensitivity to certain medications such as sulfa or penicillin, other drugs, toxins, and inhaled environmental irritants. Skin manifestations may also occur because of a fungal infection, parasites or viral infections, while in some instances the cause may be due to an autoimmune disorder. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.

Affected Populations

Cutaneous necrotizing vasculitis affects males and females in equal numbers. It affects children as well as adults. CNV is more common than the other inflammatory vascular disorders.

Standard Therapies

Diagnosis

The results of a skin biopsy demonstrate the presence or absence of CNV. Since a number of the more severe vasculitides involve the skin as well as other organ systems, additional tests of other organs must be prescribed in order to be sure that CNV is limited to the skin.



Treatment

Treatment of cutaneous necrotizing bvasculitis depends on the cause and symptoms. Removing the irritating agent (e.g., drug) and treating the underlying infection will usually eliminate the symptoms of this disorder. The drugs prednisone, cyclophosphamide, pentoxifylline and azathioprine have proven to be successful in treating the autoimmune form of Vasculitis. 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 website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) 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



Research is being conducted on the use of high dose intravenous gammaglobulin for some forms of cutaneous vasculitis. Plasmapheresis may be of benefit in some cases. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is retransfused into the patient. This therapy is still under investigation to analyze side effects and effectiveness. More research must be conducted to determine long-term safety and effectiveness of these treatments.

References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:437-38; 930.



Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:386-91.



Ryan TJ. Cutaneous Vasculitis. In: Champion RH, Burton JL, Ebling FJG. Eds. Textbook of Dermatology. 5th ed. Blackwell Scientific Publications. London, UK; 1992:1893-961.



JOURNAL ARTICLES

Liao YH, Su YW, Tsay W, et al. Association of cutaneous necrotizing eosinophilic vaculitis and deep vein thrombosis in hypereosinophilic syndrome. Arch Dermatol. 2004;141:1051-53.



Manoharan S, Muir J. Gallbladder vasculitis associated with cutaneous leukocytoclastic vasculitis. Australas J Dermatol. 2004;45:216-19.



Calamia KT, Balabanova M. Vasculitis in systemic lupus erythematosis. Clin Dermatol. 2004;22:148-56.



Ramos-casals M, Anaya JM, Garcia-Carrasco M, et al. Cutaneous vasculitis in primary Sjogren syndrome: classification and clinical significance of 52 patients. Medicine (Baltimore). 2004;83:96-106.



Willcocks L, chelliah G, BrownR, et al. Cutaneous vasculitis - a case for laparotomy. J Rheumatol. 2003;30:1621-23.



Heron E, Fiessinger JN, Guillven L. Polyarteritis nodosa presenting as acute leg ischemia. J Rheumatol. 2003;30:1344-46.



FROM THE INTERNET

Chung L, Fiorentino D. Cutaneous Vascilitis. Orphanet encyclopedia, March 2005. 14pp.

www.orpha.net/patho/GB/ik-cutaneous-vasculitis.pdf



Chung L, Fiorentino D. Cutaneous Vascilitis. Orphanet. March 2005. 2pp.

www.orpha.net//consor/chi-bin/OC_Exp.php?Lng=GB&Expert=889



Cutaneous vasculitis. DermNet NZ. nd. 5pp.

www.dermnetnz.org/vascular/vasculitis.htm



Roane DW, Griger DR. An Approach to Diagnosis and Initial Management of Systemic Vasculitis. American Family Physician. 1991;60:14pp.

www.aafp.org/afp/991001ap/1421.html

Resources

Vasculitis Foundation

PO Box 28660

Kansas City, MO 64188

USA

Tel: (816)436-8211

Fax: (816)436-8211

Tel: (800)277-9474

Email: vf@vasculitisfoundation.org

Internet: http://www.vasculitisfoundation.org



American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



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/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



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.

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