Information on the following diseases can be found in the Related Disorders section of this report:

• Polyarteritis nodosa
• Wegener's granulomatosis
• Churg-Strauss syndrome
• Henoch-Shonlein Purpura

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 following diseases can have similarities to cutaneous necrotizing vasculitis:

Polyarteritis nodosa is a group of systemic necrotizing vasculitis including the original classic polyarteritis nodosa, allergic granulomatosis, and those disorders that have the characteristics of both (an overlap syndrome). (For more information on this disorder, choose "Polyarteritis" as your search term in the Rare Disease Database.)

Wegener's granulomatosis is a rare collagen vascular disorder that begins as a localized inflammation of the upper and lower respiratory tract mucosa, and usually progresses into generalized inflammation of the blood vessels (vasculitis) and kidneys (glomulonephritis). (For more information on this disorder, choose "Wegener" as your search term in the Rare Disease Database.)

Churg-Strauss syndrome is a rare disorder that may affect multiple organ systems, particularly the lungs. The disorder is characterized by the formation and accumulation of an unusually large number of antibodies, abnormal clustering of certain white blood cells (eosinophilia), inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions (granulomatosis).

Many individuals with Churg-Strauss syndrome have a history of allergy. In addition, asthma and other associated lung (pulmonary) abnormalities often precede the development of the generalized symptoms and findings seen in Churg-Strauss syndrome. Asthma, a chronic respiratory disorder, is characterized by inflammation and narrowing of the lungs’ airways, causing difficulties breathing (dyspnea), coughing, the production of a high-pitched whistling sound while breathing (wheezing), and/or other symptoms and findings.

Henoch-Shonlein purpura is one of a group of disorders characterized by discolored purplish or brownish-red skin. These spots may be large or small. Internal bleeding may occur in various areas of the body. This blood vessel disorder may affect the skin, joints, gastrointestinal system, kidneys, and in a very few cases the central nervous system. Little is known about the cause of this form of purpura although it may be an allergic reaction which more often occurs in children than adults. (For more information on this disorder, choose "Henoch-Shonlein" as your search term in the Rare Disease Database.)

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.

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

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.

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