Vasculitis: types (cutaneous, purpura, leukocytoclastic, urticarial)

Vasculitis (or angiitis) is an inflammation of the blood vessels (arteries, veins, and capillaries) that can be affected by necrosis (cell death).

It is a rare pathology that can affect people of any age.

This systemic disease affects the vascular system and changes the primary function:

  • The arteries that carry oxygen-rich blood to the body tissues,
  • The veins that return deoxygenated blood to the heart.

Vasculitis is a multisystem disease that can affect the following organs:

  • Skin
  • Nephritic
  • Heart
  • Annoy
  • Lungs
  • Joints
  • Intestine
  • Eyes
  • Ears
  • Nose.


Classification of vasculitis

Vasculitides are divided into:

  • Primary – idiopathic, that is, one does not know the cause,
  • Secondary – they are caused by: infectious diseases, diseases of connective tissue, tumors or cryoglobulinemia, taking medications, radiation, chemical agents, organ rejection, etc.

Classification according to the size of the affected blood vessels

2011-2012 adopted designations by International Chapel Hill Consensus Conference Nomenclature of the Vasculitides

By blood vessels large 1. Takayasu’s arteritis

  1. Giant cell arteritis medium 1. Polyarteritis nodosa
  2. Kawasaki disease small 1. ANCA-associated vasculitides

– Microscopic polyangiitis

– Wegener granulomatosis

– Eosinophilic granulomatosis with eosinophilic polyangiitis 2. Immune complex vasculitis

– Goodpasture syndrome

– Cryoglobulenic vasculitis

– Systemic IgA vasculitis

– Hypocomplementary urticarial vasculitis variable 1. Behcet’s disease

  1. Cogan syndrome

Other types Individual


  1. Cutaneous leukocytoclastic vasculitis
  2. Cutaneous arteritis
  3. Primary vasculitis of the central nervous system
  4. Isolated aortitis
  5. Other systemic


  1. Vasculitis in lupus
  2. Vasculitis in rheumatoid arthritis
  3. Sarcoidosis vasculitis
  4. Other

Possible cause

  1. Cryoglobulenemic vasculitis in viral C hepatitis
  2. Vasculitis in viral B hepatitis
  3. Aortitis in syphilis
  4. Immune complex vasculitis associated with medications
  5. Vasculitis caused by ANCA drugs
  6. Vasculitis associated with cancer
  7. Other

Description of types of vasculitis

Vasculitis of the large vessels

  • giant cell arteritis (or temporalis);
  • Takayasu’s arteritis;

Takayasu’s arteritis and temporal arteritis (in adults) occur on the large elastic arteries and show similar histological features (of the tissue).

This results in the following steps:

  • Dendritic cells in the tunica adventitia (outer layer) of the artery favor and stimulate CD4+ cells.
  • These cells activate monocytes and macrophages, which cause violations of the vascular wall.

Vascular lesions are characterized by inflammation with penetration into lymphocytes and monocytes in all artery wall layers.

As a rule, activated T cells and macrophages form granulomas in which multinucleated giant cells are also present.

Often it comes to narrowing of the blood vessel, because in the inner tunica there is an important cell proliferation and thus an increase in volume.

Takayasu’s arteritis mainly affects the thoracic aorta at the level of the aortic arch and its large secondary branches, especially the subclavian artery.

In 30% of cases, the following are affected:

  1. Other sections of the aorta,
  2. renal arteries,
  3. Coronary arteries (coronary artery involvement can lead to relevant cardiological problems, especially heart attack and death).

In 50% of cases, the pulmonary arteries can also be affected.

The reason for this is not known, but scientists believe that the cause could be:

  • infections with Treponema pallidum, streptococci and the mycobacterium of tuberculosis,
  • Weakened immune system (due to autoimmune diseases, Crohn’s disease, ulcerative colitis, sarcoidosis, etc.)

In the aorta, there is a narrowing of the vascular lumen, which leads to the disappearance of the arterial pulse (therefore the disease is called pulsless disease or aortic arch syndrome).

The development of the disease can occur quickly, it can have a duration of one to two years.

After the symptoms have passed and the survival rate is good, some neurological and visual disorders may persist.

Vasculitis of medium-sized vessels

This type of vasculitis mainly affects the middle arteries, which are mainly the visceral arteries and their branches.

Inflammation of the muscular arteries occurs.

Fibrinoid necrosis is typical of the medium-sized vessels and can lead to the formation of:

  • nodules (vasoconstriction),
  • Aneurysm.

Possible causes in the case of Kawasaki disease are:

  • Infections
  • Activation of lymphocytes.

The triggering factors of polyarteritis nodosa are unclear, but may be caused by hepatitis B or C.

Kawasaki disease (can provoke rhythm disturbances, pericarditis, rashes and inflammation in the mouth);

The cause is unknown, but the most reliable hypothesis is parvovirus B19 infection, which affects the cells of the lymphatic system and blood (red blood cells, erythroblasts, etc.).

Consider other hypotheses:

  • toxic substances,
  • Allergens.

Often the coronary arteries are affected (as in polyarteritis nodosa), so differential diagnosis can be difficult.

The development of coronary artery lesions can lead to the formation of aneurysms.

In most cases, the prognosis is good, but in 1-3% of cases, sudden death from vasculitis can occur due to:

  1. rupture of coronary artery aneurysms,
  2. thrombosis of the coronary arteries,
  3. Infarct
  4. Myocarditis.

This disease can affect any organ (kidney, heart, liver, intestines, testicles, etc.).

Many symptoms may occur and:

  • be non-specific,
  • Be systemic.

First of all, the patient has a fever and an unexplained weight loss.

Often the patient also suffers from:

  • Hypertension
  • Muscle
  • Diarrhoea
  • nausea and vomiting,
  • Neuritis
  • Melena (digested blood in the stool) due to intestinal ulcers,
  • Stomach ache.

The course is different, it can be chronic or intermittent, as well as accompanied by periods of long periods of remission.

A necrotizing inflammation is observed, which affects the arterial wall focal (localized) and / or affects the entire vascular circumference.

Over time, fibrosis forms.

The most significant complication is renal hypertension.

In typical cases, angiography shows the presence of aneurysms in the visceral arteries.

How long does the disease last? The prognosis remains severe, despite therapy with immunosuppressants.

Vasculitis of small vessels

Antibody-mediated vasculitis

ANCA antibodies (anti-neutrophils cytoplasmic antibodies) are directed at proteins located in the cytoplasm of neutrophils and monocytes (for example, PR3, MPO) and also on the cell surfaces.

IgG ANCA can directly activate:

  1. neutrophils,
  2. The monocytes.

These activated cells react with the cells of the endothelium through adhesion molecules, which are receptors (proteins) on the cell membrane.

They also release inflammatory mediators (substances that cause inflammation) such as granzymes (enzymes that break down chemical compounds into proteins) and reactive oxygen metabolites.

The consequences are:

  • apoptosis,
  • Necrosis.

In addition, the anti-MPO IgG can activate the MPO, which causes severe endothelial damage.

The activated ANCA neutrophils can release factors that activate the alternative pathway of complement (body defense system).

In this way, a cycle of amplification (cellular process) begins, which causes the necrotizing inflammation. (Jennette JC, Falk RJ. New insights into the pathogenesis of vasculitis associated with antineutrophil cytoplasmic autoantibodies. Curr Opin Rheumatol. 2008 Jan. 20(1): 55-60)

Antibody deposition

Schoenlein-Henoch purpura is generally characterized by the deposition of IgA antibodies in the affected tissues.

Skin biopsy indicates leukocytoclastic vasculitis with accumulation, namely:

  • Neutrophils
  • Lymphocytes
  • Monocytes.

In addition, IgA, C3 and fibrin are also found:

  • In the walls of the affected blood vessels and in the postcapillary venules in the dermis,
  • In the endothelial and mesangial cells of the kidney.

Some patients may have an elevated IgA in the blood.

The purpura Schoenlein-Henoch mainly affects children.

Vasculitis of the small arteries is divided into:

  1. ANCA positive: when antibodies against cytoplasmic antigens of neutrophils are involved
  2. ANCA negative: in other cases.

In Goodpasture syndrome, the circulating antibodies bind to the type IV collagen inside the basement membrane of the renal corpuscle.

Pulmonary hemorrhages occur when these antibodies penetrate the basement membrane of the alveoli.

This often occurs with necrotizing glomerulonephritis, which can cause significant renal dysfunction. (Ozen S, Anton J, Arisoy N, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004 Oct. 145(4): 517-22).

Microscopic polyangiitis (mPA) is necrotizing vasculitis, characterized by:

  1. Glomerulonephritis
  2. Inflammation of the pulmonary capillaries.

Symptoms include:

  • livious rash,
  • protein in the urine,
  • blood in the urine,
  • Bloody tinged cough,
  • CNS dysfunction,
  • Joint pain.

In Churg-Strauss syndrome and granulomatosis with polyangiitis, granulomas form around and outside the vessels.

In the other vasculitis, inflammation occurs only in the small vessels.

In the tissue area, diagnosis is based on three parameters:

  1. penetration of eosinophils,
  2. necrotizing vasculitis,
  3. Perivascular and extravascular granulomas with the formation of fibrosis and calcifications.

However, this never occurs at the same time.

Granulomatosis with polyangiitis (for example, Wegener’s granulomatosis) mainly affects the respiratory system, but can also affect any other organ.

Characteristic can be:

  1. livious rash,
  2. recurrent sinusitis,
  3. Shortness
  4. Nosebleed
  5. Coughing up blood due to alveolar hemorrhage.

Churg-Strauss syndrome mainly concerns:

  1. Lung
  2. Heart
  3. Peripheral nervous system.

The onset occurs in asthmatic crises and peripheral hypereosinophilia.

After a variable period of months or years, the vasculitic phase begins with symptoms of:

  1. Pericarditis
  2. Myocarditis
  3. Pneumonia
  4. mononeuritis,
  5. Disorders of other organs or organ systems.

A positive ANCA is observed in approximately 40% of patients.

Churg-Strauss syndrome, Wegener’s granulomatosis and microscopic polyarteritis are characterized by the presence of ANCA in the blood serum, while deposits of immunoglobulins on the vascular walls are more or less absent.

Schönlein-Henoch purpura and mixed essential cryoglobulinemia are characterized by immune complexes in the vessel walls, while the search for ANCA is negative.

Cutaneous vasculitis

Cutaneous vasculitis can be an important component of systemic vasculitis, such as:

  • those of rheumatoids,
  • Those associated with ANCA (Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis).

The characteristic sign is a palpable purpura, which has superficial vasculitis in the small vessels.

Less often develop:

  1. A nodular erythema,
  2. Livedo racemosa (red reticulated patches on the skin),
  3. Deep ulcers,
  4. Digital gangrene.

In these cases, vasculitis is deep-seated, at the level of the dermis or subcutaneous tissue.

To make the diagnosis, a biopsy is required, which extends into the subcutaneous tissue, taken from the most painful, most reddened or bluish discolored area.

Among cutaneous vasculitis, the most common form is leukocytoclastic (or hypersensitive) vasculitis, which mainly causes:

  1. Purpura
  2. Local necrosis of the feet and ankles,
  3. Blow
  4. Papules
  5. Node
  6. Net-like, blue-violet drawings.

It can also cause ulceration.

Urticaria vasculitis is a form of vasculitis that affects the skin by causing:

  1. wheals or hives,
  2. Red spots.

There are two forms:

  1. One with normal concentration of complement proteins in the blood (normocomplementemic).
  2. The other with small amounts of complement proteins, called vasculitis with hypocomplementemia.

Urticaria vasculitis is a type III hypersensitivity reaction in which the antigen-antibody complexes are deposited in the vascular layer.

This reaction causes:

  1. activation of complement,
  2. Attraction of neutrophils.

These cells release various proteolytic enzymes, such as collagenase and elastase. These cause damage in the vessel wall.

The most common symptoms are:

  1. Itch
  2. Ache
  3. Burning sensation.

The spots on the skin often have a red border with a white center.

Under the skin there may be:

  1. Petechiae
  2. Bleeding.

In most cases, this condition is idiopathic or has no specific causes. However, doctors associate them with:

  • Some disorders of the immune system,
  • Leukaemia
  • Sjögren’s syndrome,
  • Systemic lupus.

In addition, some viral diseases can cause urticaria vasculitis, such as:

  1. HBV (Hepatitis B),
  2. HCV (Hepatitis C),
  3. herpes zoster virus,
  4. Infectious mononucleosis.

Urticaria vasculitis could be the cause of lupus.

The disease could also be a side effect to medications, such as:

  1. Penicillin
  2. ACE inhibitors,
  3. Antidepressants.

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