An inflammation of a blood vessel primarily involving the skin in an otherwise normal vascular system which is segmental in the blood vessel wall, and involves scattered areas of intense inflammation with an accumulation of neutrophils and fibrinoid necroses.

Cutaneous vasculitis may result from a variety of etiologic factors that may include: injury or trauma; burns; infections; hypersensitivity reactions, such as drug reactions; or disease conditions, such as collagen vascular disorders. Any type, size, and location of blood vessel may be involved, i.e., arteries, arterioles, veins, venules, or capillaries. However, cutaneous vasculitis usually involves venules. Varying degrees of cellular infiltration and scarring may occur at the affected site in one or more layers of the vessel wall.

The manifestations of primary cutaneous vasculitis will vary depending on the size and depth of the predominant vessel involved. If the condition is extensive, or if large vessels are involved, skin necrosis and ulceration may occur. Inflammation of the postcapillary venule or dermal capillaries, associated with conditions such as Henoch-Schonlein purpura or drug-induced vasculitis, may cause palpable purpura. This condition is manifested by elevated lesions resulting from inflammatory damage to cutaneous blood vessels. Vascular inflammation of the deep dermal layer of tissue may result in tender, deep, indurated, red bumps on the arms and legs. Inflammation of medium-sized muscular arteries may produce fever, malaise, and necrotic skin ulcers. Periarteritis that is limited to skin and muscle involves small and medium muscular arteries in deep dermis, subcutaneous tissue, and muscle. The condition results in deep subcutaneous nodules with ecchymoses or ulceration.

Diagnosis of the underlying condition causing the vasculitis is important. A detailed history and physical examination may be performed, including serum laboratory tests. There are no specific blood tests to diagnose vasculitis; however, the erythrocyte sedimentation rate may be elevated. Biopsy of skin lesions may be necessary and helpful in diagnosing the underlying cause. Other tests that may be helpful include x-ray, ultrasound, and magnetic resonance imaging (MRI). The definitive diagnosis may be confirmed by removing a segment of the affected vessel for laboratory examination.

Therapy will be determined by the underlying cause of the condition, and specific medications and treatments may be needed. Treatment may involve low-dose corticosteroids, nonsteroidal anti-inflammatory drugs (NSAIDs), or other medications for symptomatic treatment, only. Management may also involve aggressive therapy with glucocorticoids and immunosuppressive agents. If the vasculitis is idiopathic and cutaneous, the skin may respond to oral prednisone or dapsone (Avlosulfon).

Inflammation at any point in the vessel wall may resolve and cause fibrosis and intimal hypertrophy. There may be the potential for partial or total occlusion of the vessel, causing tissue ischemia and necrosis.

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