Symptoms
Cutaneous vasculitis is a term which describes inflammation of the blood vessel wall in the dermis and the subcutis, and, as such, is not a diagnosis but a cutaneous reaction pattern that can result from multiple aetiologies.
Causes
Vasculitis presents several diagnostic challenges. Firstly, patients could present with protean clinical manifestations with a wide spectrum ranging from isolated cutaneous vasculitis to multisystem involvement. Secondly, there are several medical conditions that could mimic the presentation of vasculitis.
Prevention
Keywords: vasculitis, diagnosis Vasculitis means inflammation of the blood vessel wall. Any type of blood vessel in any organ could be affected. Clinical manifestations arise because:
Complications
In summary, patients in whom vasculitis is suspected need detailed medical evaluation (box 6). Because treatment of most primary forms of vasculitis consists of potentially toxic immunosuppressive therapy, exclusion of conditions that could mimic or cause vasculitis (especially infection and drug exposure) should take priority.
What is cutcutaneous vasculitis?
What are the diagnostic challenges in the workup of variable vasculitis?
What is vasculitis?
What are the treatment options for vasculitis?
What is the treatment for cutaneous vasculitis?
Treatment of Cutaneous Vasculitis Colchicine, hydroxychloroquine, dapsone, or a short course of low-dose corticosteroids can be tried. Sometimes, stronger immunosuppressants (eg, azathioprine, methotrexate) are used, particularly if lesions ulcerate or if corticosteroids must be taken indefinitely to control symptoms.
What is vasculitis and how is it treated?
Vasculitis, an inflammation of blood vessels, can affect vessels of all size. If blood flow is reduced or stopped, tissues can begin to die. Treatment, which may include medication, depends upon which vessels and organs are affected.
How do you treat vascular rash?
Like NSAIDs, oral steroids are used to manage skin rashes and joint pain. Most people respond to a short course of steroids, such as prednisone or methylprednisolone. If your internal organs are affected, or if you have severe skin lesions, your healthcare professional might recommend intravenous (IV) corticosteroids.
How do you approach a case of vasculitis?
A thorough history and detailed physical examination supplemented with a few simple investigations such as urine dipstick and chest radiography should be sufficient in most patients to assess extent of involvement with vasculitis.
What is cutaneous vasculitis?
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).
What does prednisone do for vasculitis?
Currently, treatment for ANCA-associated vasculitis typically involves use of high doses of glucocorticoids (“steroids”) like prednisone to tamp down the inflammation that breaks down blood vessels.
How is cutaneous vasculitis diagnosed?
A skin biopsy can be helpful to confirm the diagnosis of cutaneous vasculitis. Biopsy of a lesion that is 24 to 48 hours old is most likely to yield the relevant diagnostic information. Features include inflammatory infiltrate around blood vessels, disruption or destruction of the vessel walls and fibrinoid necrosis.
How is small vessel vasculitis treated?
What is the treatment for cutaneous small vessel vasculitis?If an underlying cause is found, remove the trigger (for example, stop the drug) and treat associated disease(s)Rest — exercise often induces new lesions.Apply compression and elevate the affected limb(s)Use simple analgesics and NSAIDs for pain.More items...
What are cutaneous vessels?
The small vessels in the skin affected are located in the superficial dermis and include arterioles (small arteries carrying blood to capillaries), capillaries, and venules (small veins receiving blood from capillaries).
What is the main cause of vasculitis?
The exact cause of vasculitis isn't fully understood. Some types are related to a person's genetic makeup. Others result from the immune system attacking blood vessel cells by mistake.
What is the most common vasculitis?
Giant cell arteritis is the most common type of primary systemic vasculitis with an incidence of 200/million population/year.
What is vasculitis of the legs?
Vasculitis means inflammation of the blood vessels. It causes swelling and can help the body deal with invading germs. But in vasculitis, for some reason the immune system attacks healthy blood vessels, causing them to become swollen and narrow.
What is the clinical manifestation of a blood vessel?
Clinical manifestations arise because: Systemic inflammatory response resulting from release of chemical mediators from inflamed blood vessels gives rise to various non‐specific systemic manifestations.
Is vasculitis self limiting?
Some are benign and self limiting, while others have the potential to threaten vital organ function and life. It follows that a rational approach is required during evaluation of patients with suspected vasculitis. Keywords: vasculitis, diagnosis.
Is secondary vasculitis rare?
Most forms of secondary vasculitis are extremely rare with the possible exception of rheumatoid vasculitis .20Vasculitis is seldom the initial presenting manifestation when it occurs in the setting of rheumatoid arthritis or systemic lupus erythematosus, and is thus readily diagnosed by features of the parent illness.
Can vasculitis mimic infection?
Several conditions could mimic vasculitis7,8,9and need to be considered in the differential diagnosis depending on clinical presentation. Firstly, infection is a great mimic of vasculitis (see box 1). Several clinical and laboratory features are common to both vasculitis and infection.
Is vasculitis a primary disease?
Thirdly, vasculitis could occur as a primary disorder or be secondary to various medical conditions. It becomes important to differentiate them, as treatment of some forms of vasculitis such as those that are secondary to infection or drugs, is different from that of primary vasculitis.
Can polyarteritis nodosa be labelled as a small vessel?
By definition, patients with small vessel involvement such as glomerulonephritis or pulmonary capillaritis should not be labelled as polyarteritis nodosa.47Renal involvement can however still occur in polyarteritis nodosa because of hypertension or involvement of medium sized renal arteries leading to renal infarction.
What is cutaneous vasculitis?
Cutaneous vasculitis associated with cryoglobulinemia, hypergammaglobulinemic vasculitis (HGV), urticarial vasculitis, and purpura without cryoglobulins are the most common findings in pSS with cutaneous involvement.85 Generally, patients with cutaneous vasculitis, generally classified as leukocytoclastic vasculitis or lymphocytic vasculitis, have more severe disease and more diffuse extraglandular involvement than those without this manifestation. 86 About half of such patients have a single episode, mainly involving the lower extremities, whereas the other half of these patients are characterized by recurrent episodes requiring corticosteroids and immunosuppressive therapy. 85 In particular, HGV represents a typical vasculitis in pSS related to benign B cell proliferation and characterized by lower extremity recurrent purpura, polyclonal hypergammaglobulinemia, and Ig deposition along the blood vessel walls. In pSS, HGV must be differentiated from cryoglobulinemia, a cutaneous vasculitis characterized by higher rates of systemic manifestations, including PNS and renal involvement, complement activation, leukopenia, serum monoclonal component, and, of more relevance, by a higher risk of lymphoma. 87
What is the primary lesion in cutaneous vasculitis?
The primary lesion in cutaneous vasculitis is a purpuric papule (palpable purpura). Necrosis sometimes follows; it is heralded by the appearance of a dark gray color in the center of a lesion, followed by slough. In the absence of necrosis, lesions evolve by flattening and fading. The flattening may occur surprisingly quickly, so a lesion that is palpable on the first day may be flat by the second. As lesions fade, hemosiderin remains, leaving the affected skin brown. In gonococcemia, lesions are distinctive in that they are pustular as well as purpuric, sparse, and distributed distally on the extremities (Fig. 17.13 ). Lesions of vasculitis are most often located on the lower extremities, but they may be generalized in patients with extensive disease.
What is the most common cause of purpura?
Cutaneous vasculitis (CV) is one of the most common causes of nonthrombocytopenic purpura and, because of its frequent association with significant underlying medical disease, is of interest to the hematologist and the internist. That CV is often associated with diseases of rheumatic origin led early investigators such as William Osler and Austin Flint to refer to this as purpura rheumatica. We now frequently refer to CV also by its chief clinical attribute ( palpable purpura) or its histologic hallmark ( leukocytoclastic vasculitis ). In leukocytoclastic vasculitis (LCV), the smallest vessels in the skin are encased by sheets of neutrophils in various stages of disintegration, often referred to as “nuclear dust” ( Fig. 11‐12 ). Heavy infiltration by leukocytes is what actually gives rise to the perception of palpability in palpable purpura ( Fig. 11‐13 ). It is hypothesized that this pathologic event results from leakage of immune complexes through the vessel wall into the subendothelial area. These complexes then induce the egress of leukocytes. Subsequently, the neutrophils disintegrate, releasing their proteolytic enzymes into the area around the adjacent vessel, causing digestion and disruption of the endothelial membrane, with subsequent egress of red cells ( Fig. 11‐14 ). When LCV is acutely severe, it is called necrotizing vasculitis.
Is cutaneous vasculitis uncommon?
Cutaneous vasculitis is uncommon. Of new patients seen in the authors' dermatology clinic, 0.3% had cutaneous vasculitis. It was more common in their hospital practice: 2% of the dermatology consultations were for vasculitis.
What is the cause of vasculitis?
Vasculitis involves inflammation of the blood vessels. The inflammation can cause the walls of the blood vessels to thicken, which reduces the width of the passageway through the vessel. If blood flow is restricted, it can result in organ and tissue damage. There are many types of vasculitis, and most of them are rare.
What are the complications of vasculitis?
Complications of vasculitis include: Organ damage. Some types of vasculitis can be severe, causing damage to major organs. Blood clots and aneurysms. A blood clot may form in a blood vessel, obstructing blood flow. Rarely, vasculitis will cause a blood vessel to weaken and bulge, forming an aneurysm (AN-yoo-riz-um).
How do you know if you have vasculitis?
General signs and symptoms of most types of vasculitis include: Fever. Headache. Fatigue. Weight loss. General aches and pains. Other signs and symptoms are related to the parts of the body affected, including: Digestive system. If your stomach or intestines are affected, you may experience pain after eating.
What are the causes of a person's immune system reaction?
Possible triggers for this immune system reaction include: Infections, such as hepatitis B and hepatitis C.
Can vasculitis cause blindness?
Vasculitis can make your eyes look red and itch or burn. Giant cell arteritis can cause double vision and temporary or permanent blindness in one or both eyes. This is sometimes the first sign of the disease. Hands or feet. Some types of vasculitis can cause numbness or weakness in a hand or foot.
Can vasculitis affect only one organ?
There are many types of vasculitis, and most of them are rare. Vasculitis might affect just one organ, or several. The condition can be short term or long lasting. Vasculitis can affect anyone, though some types are more common among certain age groups. Depending on the type you have, you may improve without treatment.
Can cocaine cause vasculitis?
Using cocaine can increase your risk of developing vasculitis. Smoking tobacco, especially if you're a man younger than 45, can increase your risk of Buerger's disease. Medications. Vasculitis can sometimes be triggered by medications such as hydralazine, allopurinol, minocycline and propylthiouracil. Infections.
What is cutaneous vasculitis?
Cutaneous vasculitis is a term which describes inflammation of the blood vessel wall in the dermis and the subcutis, and, as such, is not a diagnosis but a cutaneous reaction pattern that can result from multiple aetiologies. Damage to the blood vessel wall and impaired blood flow result in oxygen and nutrient deprivation in the surrounding tissue, ...
What is vasculitis cutaneous reaction?
Summary. Vasculitis is a cutaneous reaction pattern, resulting from inflammation of the blood vessel wall in the dermis and the subcutis. The causes can be immunological, eosinophilic, non-immunological or idiopathic in nature. The definitive diagnosis of vasculitis relies mainly on history and clinical signs supported by histopathology.
What is eosinophilic vasculitis?
Eosinophilic vasculitis is associated with type I hypersensitivity response and is seen in arthropod bite lesions. Non-immunological vasculitis occurs when the endothelial cells are damaged by burns, trauma or physical blockage of blood vessels affecting the haemodynamics of blood flow.
Why is vasculitis not evident in biopsies?
In many biopsies this is not evident, because of inadequate sample size, inappropriate site selection, timing of the biopsies and secondary infections. Sometimes several sections of the biopsy are required to make the diagnosis.
How often should you monitor azathioprine?
For example, if the owner has limited funds one should refrain from offering azathioprine, because of the cost of the haematology and biochemical parameters that should be monitored every two weeks in the initial stages and, once stable, every two to three months.
What is topical treatment for ulcers?
For localised lesions, topical treatment can be used to successfully manage some of the diseases. For diseases with widespread ulceration and systemic involvement, hospitalisation and supportive treatment and wound management are necessary.
Is there a universal drug for vasculitis?
There is no single universal drug for each condition, but there are a range of drugs with immunomodulating properties that can be employed. For vasculitis, glucocorticoids, ciclosporin, pentoxifylline and azathioprine are used either individually or in combination depending on the individual case (Table 1).