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.
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.
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.
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 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.
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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.
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.
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.
Chung L, Fiorentino D. Cutaneous Vascilitis. Orphanet. March 2005. 2pp.
Cutaneous vasculitis. DermNet NZ. nd. 5pp.
Roane DW, Griger DR. An Approach to Diagnosis and Initial Management of Systemic Vasculitis. American Family Physician. 1991;60:14pp.
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