Urticarial Vasculitis: Types, Causes, Symptoms, Treatment, Prognosis

Rashes, skin lesions, inflammation can be said to be common occurrence for a majority of the population. Though most of them do not require any special kind of treatment and get resolved within a few days, some of the conditions may turn out to be more serious requiring specialized diagnosis and treatment. One such disease is Urticarial Vasculitis which needs special care and attention.

What is Urticarial Vasculitis?

Urticarial Vasculitis can be defined as a type of Cutaneous Vasculitis that is indicated by the inflammation of the walls of the small blood vessels. Though this appearance of erythamatous wheals is somewhat similar to urticaria clinically, it has been found to manifest features of leukocytoclastic vasculitis histologically.

Urticarial Vasculitis

Urticarial Vasculitis may be further divided into three sub categories, viz.-

  • Normocomplementemic Urticarial Vasculitis (NUV)
  • Hypocomplementemic Urticarial Vasculitis (HUV)
  • Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)

All the above sub types of Urticarial Vasculitis are delineated by the levels of “complement” in the blood, which may again be defined as a class of proteins that assists and intensifies the responses of the immune system. These often play a significant role in autoimmune disorders, Urticarial Vasculitis, being among one of the many.

Signs and Symptoms of Urticarial Vasculitis

Urticarial Vasculitis is characterized by a number of signs and symptoms which can be easily linked to the disease. Some of the symptoms of Urticarial Vasculitis are-

  • The very first symptom of the disease is an urticarial appearance which is often accompanied by a painful burning sensation.
  • This rash may be recurrent in nature and may feel like “stinging nettle”, accompanied by pruritus and burning.
  • Patchy lesions with a whitish center lasting for more than a day may also appear at a particular location in the body. Though these heal automatically, they may leave behind ecchymoses or patches resembling bruises or hyper-pigmentation in the way of healing.

Hence, people suffering from such symptoms should get themselves diagnosed clinically in order to find out if they are suffering from Urticarial Vasculitis.

Types of Urticarial Vasculitis

Urticarial Vasculitis can be divided into three subsets, all of which are computed based on the level of “complement” in the blood.

Normocomplementemic Urticarial Vasculitis (NUV)

In this case, the patient manifests the major symptoms of Urticarial Vasculitis accompanied by normal levels of C1q complement level in the blood. Generally considered to be the least serious of all the types of Urticarial Vasculitis, it does not manifest any other symptoms that may lead to complications.

Hypocomplementemic Urticarial Vasculitis (HUV)

In this case, the patient manifests all the major symptoms of Urticarial Vasculitis along with C1q complement level in the blood being lower than what is considered to be normal. This situation co-exists with increased levels of anti-C1q antibodies. HUV is a more serious class of Urticarial Vasculitis and may manifest certain symptoms such as –

These are common symptoms, but there are other symptoms that may occur.

Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)

Along with all the usual symptoms of HUV, patients in this case also manifest certain systemic complications such as –

Along with afore- mentioned issues, the patients also manifest certain complement irregularities, such as lower circulation of third and fourth components of the complements of the blood.

Causes of Urticarial Vasculitis

Causes of Urticarial Vasculitis

The causative factor behind half of the cases of Urticarial Vasculitis remains unknown. Nevertheless, it has been inferred that the occurrence of Urticarial Vasculitis is prompted by certain autoimmune or connective diseases of the tissue such as-

Epidemiology of Urticarial Vasculitis

Though the exact number of people suffering from Urticarial Vasculitis is not known, a previous study conducted in the United Kingdom revealed that approximately 2.1% out of 310 patients were found to be affected with the disease during a consistent period of 3 months. Urticarial Vasculitis affects people in the age range of 15 to 90 years with the median age being 43 years. The male:female ratio of the occurrence of this disease has been found to be 1:2.

Prognosis of Urticarial Vasculitis

Most cases of Urticarial Vasculitis are known to resolve in duration of months to a few years. In this respect, it can be said that the prognosis of Urticarial Vasculitis is generally good in case the histology of the disease which mainly consists of the level of blood complements are normal. Urticarial Vasculitis may take a turn for the serious if the complements are low in level. If Urticarial Vasculitis is linked to other diseases such as, cancer or lupus, the prognosis of those diseases often governs the entire process of this disease.

Diagnosis of Urticarial Vasculitis

Diagnosis of other categories of Vasculitis generally includes the recognition of the sequence of signs and symptoms and the results of various investigations. A blood test is generally conducted in order to examine the increased level of ESR or “Erythrocyte Sedimentation Rate” and CRP or “C- reactive protein”, which are common indicators of inflammation. Often kidney and skin biopsies are also conducted in order to affirm the diagnosis. Various investigations are also conducted for C3, C1q, anti-C1q, C4 components of the complements in the blood in order to determine the category of Urticarial Vasculitis. Additionally, investigations are also conducted in order to assess the levels of ANA or Anti-Nuclear Antibody and Anti ds- DNA.

Urticarial skin lesions can also be a result of a number of systemic disorders, such as urticaria, hematologic diseases, connective tissue diseases and auto-inflammatory diseases. Differential diagnosis of Urticarial Vasculitis, thus, involves the elimination of all of these manifestations including indications erupting out of drug eruptions and Leukocytoclastic Vasculitis.

Treatment of Urticarial Vasculitis

Treatment of Urticarial Vasculitis generally depends upon the magnitude of the signs and symptoms and the involvement of the organs. In most cases, the associated symptoms improve of their own accord or minimum treatment if the level of complement in the blood is normal, absence of underlying disease or non-involvement of organs. In this case, non-steroidal drugs or antihistamines such as, naproxen or ibuprofen proves to be quite helpful. Drugs such as colchicines, hydroxychloroquine, dapsone and corticosteroids and chemotherapies such as cyclophosphamide or azathioprine are prescribed in case of severe form of Urticarial Vasculitis.

If any of the signs and symptoms of urticarial vasculitis is noted, immediate medical advice must be taken. Although it is very easily manageable, negligence towards the disorder can bring in the serious complications.

Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:April 18, 2019

Recent Posts

Related Posts