Cutaneous vasculitis (CV) is a condition in which inflammation of the vessel wall leads to hemorrhage and ischemia [1]. It can be a primary or idiopathic condition (e.g. polyarteritis nodosa (PAN), Wegener's granulomatosis (WG), Churg-Strauss syndrome (CSS), idiopathic cutaneous leukocytoclastic angiitis) or secondary to drugs (e.g. NSAIDs), infections or systemic diseases (e.g. connective tissue disease, rheumatoid arthritis) and malignancy [2].

Another classification of CV is based on the size of the vessel wall affected - small vessel vasculitis (SVV) and medium vessel vasculitis (MVV) [3]. SVV is associated with urticaria, palpable purpura, target lesions as well as vesiculobullous lesions while lesions in MVV include subcutaneous nodules, livedo reticularis, ulcer, infarct, digital pitted scars and gangrene [1] [2] although overlapping clinical features of SVV and MVV have also been reported [4] in the same patient. The cutaneous lesions are usually found on the lower extremity and their appearance on the upper extremity is an indication of severe and/or systemic disease [5]. Constitutional symptoms like fever, malaise, arthralgias are common to CV of the primary as well as secondary types. Although CV can occur in individuals of all ages, it is commonly seen in adults and has a female preponderance. A majority of the children with CV have Henoch-Schönlein purpura (HSP).

Three patterns of CV presentation and progression have been reported [2]:

1. A self-limiting, single episode following an infection or intake of drug which resolves within six months. This is the most common form of the presentation of CV.
2. Waxing and waning symptoms are seen in HSP and connective tissue associated vasculitis
3. Chronic, progressive vasculitis associated with cryoglobulinemia, hypergammaglobulinemia, and malignancy

CV may be associated with extracutaneous (visceral) vascular inflammation in 20% of the patients and this affects mainly the kidneys [1] [5] [6]. Severe forms of life-threatening CV have been reported in < 7% patients [2] [6].

• Infectious Mononucleosis

  Other conditions that may cause or be associated with small-vessel vasculitis include: infections (hepatitis B and C, infectious mononucleosis, Lyme disease), drugs (diltiazem, cimetidine, procarbazine, fluoxetine, etanercept, methotrexate), hematological [clinicaldermatology.eu]

• Vascular Disease

  Cutaneous Vasculitis OVERVIEW The vascular system refers to the collection of all blood vessels in the body. Vasculitis is a term used for a group of diseases characterized by the inflammation of and damage to the blood vessels walls. [dramitdutta.com]

  Infective causes are considered as those where there is direct invasion by pathogens into the vascular wall, resulting in inflammation. Examples include rickettsial vasculitis, syphilitic aortitis and aspergillus arteritis. [patient.info]

  The collagen vascular disease workup revealed positive ANA with speckled pattern and rheumatoid factor in 3 (6%) patients each, while it was observed in 28.5 and 26.4% patients, respectively, by Sais et al. [8] In addition, c-ANCA, HCV and serum cryoglobulins [dx.doi.org]

• Left Ventricular Dysfunction

  A 61-year-old patient who had been treated with lisinopril in the past without any problems was commenced on ramipril for left ventricular dysfunction. He developed a painful symmetrical purpuric eruption over both feet after three days. [ncbi.nlm.nih.gov]

• Cutaneous Manifestation

  Dear Sir, Crohn's disease is associated with a variety of cutaneous manifestations many of which parallel disease activity. [academic.oup.com]

  Cocaine-levamisole-related cutaneous vasculitis with leukopenia is a diagnosis of exclusion, but this diagnosis should be strongly considered in patients with a history of cocaine abuse who present with a tetrad of cutaneous manifestations consisting [ncbi.nlm.nih.gov]

• Eczema

  I have eczema, so I thought maybe he was having an allergic reaction to something. His rashes looked somewhat similar to the breakouts I get when something triggers my eczema. [wisegeek.org]

  ITCHING PURPURATOUS (Eczematide-like purpura ) Itching purpura is an eczema like purpura, which begins usually as an eczematous purpuric reaction around the ankles and spreads peripherally. [drmhijazy.com]

• Malar Rash

  The vasculitis group had a higher frequency of malar rash (97.9% vs. 87.4%, p = 0.004), photosensitivity (91.4% vs. 81.6%, p = 0.030), and Raynaud phenomenon (RP; 27.7% vs. 7.5%, p [ncbi.nlm.nih.gov]

• Myoclonus

  Neurologic features in all five siblings included oral motor deficits, dysarthria, low average IQ (70-80), and essential myoclonus. Four had documented ataxia and/or mild sensory loss with increased patellar but diminished ankle reflexes. [ncbi.nlm.nih.gov]

• Dysarthria

  Neurologic features in all five siblings included oral motor deficits, dysarthria, low average IQ (70-80), and essential myoclonus. Four had documented ataxia and/or mild sensory loss with increased patellar but diminished ankle reflexes. [ncbi.nlm.nih.gov]

The diagnosis of CV can be challenging due to its variable clinical presentation. The physician has to not only diagnose the vasculitis but should also identify the underlying condition leading to the vasculitis, order the appropriate laboratory workup, treat the patient and follow-up carefully. After eliciting a detailed history and performing a multi-system physical examination, routine laboratory tests like complete blood count, erythrocyte sedimentation rate, C-reactive protein, renal function tests, and urinalysis are ordered. Specific tests like antinuclear antibody, anti-neutrophil cytoplasmic antibodies (ANCA), perinuclear ANCA (pANCA), rheumatoid factor, and complement levels should be obtained in CV patients despite the absence of clinical features of connective tissue disorders or rheumatoid arthritis or systemic lupus erythematosus. Adult patients with HSP have been reported to have immunoglobulin (Ig)A-type antiphospholipid antibodies [7] and should be tested for it.

If the etiology of CV has been identified then laboratory tests like serum protein electrophoresis (for paraproteinemia), cryoglobulins, hepatitis C antibody and, HIV should be performed. Bone marrow biopsy is indicated in patients with abnormal peripheral blood smears [8]. Although testing for malignancies is not routinely recommended, it should be performed if the clinical presentation in CV is indicative of it [9]. Blood culture and echocardiography may be indicated in patients with endocarditis.

Skin biopsy of the lesion is the mainstay of CV diagnosis as it differentiates between authentic versus pseudo-vasculitis. The timing of the biopsy, multiple biopsies taken from the most reddish, tender and purpuric cutaneous lesions and extending into the subcutaneously help improve identification of the lesion [2]. Almost all biopsies can detect immunoglobulins in the first 48 hours, although complement can still be detected in a majority of cases after 72 hours [10]. Histology demonstrating pan-dermal small-vessel vasculitis as well as subcutaneous muscular-vessel vasculitis is indicative of a connective tissue disorder, ANCA-associated vasculitis, Behçet disease, or malignancy-associated vasculitis. A concomitant biopsy for direct immunofluorescence evaluation is useful to distinguish between IgA-associated vasculitis (Henoch-Schönlein purpura) and IgG-/IgM-associated vasculitis.

• Chlamydia Trachomatis

  Unlike Chlamydia trachomatis and C. psittaci, the association of C. pneumoniae infection with immunological complications, such as reactive arthritis (ReA) or erythema nodosum (EN) has been rarely reported. [ncbi.nlm.nih.gov]

There is no general treatment strategy and the effects of various treatments have never been confirmed histopathologically. A 43-year old man with DM developed refractory multiple cutaneous ulcers which were revealed as vasculitis by skin biopsy. [ncbi.nlm.nih.gov]

Once confirmed though biopsy, treatment should begin with symptomatic management and treatment of any underlying disorder or causative agent. In more severe cases immunomodulatory medications or steroids. [faoj.org]

For example, a patient with cutaneous leucocytoclastic angiitis and moderate nephritis as component of Henoch-Schonlein purpura has a much better prognosis than a patient with these same findings as a component of Wegener's granulomatosis Reference: [gpnotebook.co.uk]

The diagnosis of gut vasculitis infers a poor prognosis necessitating aggressive therapy. We describe a case with predominant features of a skin-limited vasculitis but atypical debilitating abdominal symptoms. [ncbi.nlm.nih.gov]

The prognosis of systemic vasculitis is dependent upon the severity of involvement of other organs. If vasculitis affects the kidneys, lungs or brain it can be life-threatening. [dermnetnz.org]

OBJECTIVE: To determine the clinical profile, etiologic factors, disease associations, and the laboratory profile of adult patients with cutaneous leukocytoclastic vasculitis (LV) presenting to the Dermatology Department at Farwaniya Hospital, Kuwait. [ncbi.nlm.nih.gov]

Object: We studied the etiological factors and clinico-pathological spectrum of patients with cutaneous vasculitis at a tertiary referral centre of north India. [ijcbr.com]

Although vasculitis can be classified by etiology, many cases have no identifiable cause, and a single etiologic agent can elicit several distinct clinicopathologic expressions of vasculitis. [pdfs.journals.lww.com]

It is important to investigate etiological possibilities. 50% or more of cases appear to be idiopathic. Pathophysiology Pathogenesis. The most commonly accepted mechanism of vasculitis is type III hypersensitivity. [vetstream.com]

Penny K, Fleming M, Kazmierczak D, et al. : An epidemiological study of Henoch-Schönlein purpura. Paediatr Nurs. 2010; 22 (10): 30–35. PubMed Abstract | Publisher Full Text 11. [f1000research.com]

Secondary Raynaud's phenomenon and cutaneous vasculitis associated with the use of interferon Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. [medical-dictionary.thefreedictionary.com]

A number of patients with cutaneous vasculitis may also have an underlying bacterial infection, according to researchers from the Hospital Universitario Marqués de Valdecilla and the rheumatology division of the Epidemiology, Genetics and Atherosclerosis [healio.com]

Herein, we review cutaneous vasculitis focusing on diagnostic criteria, classification, epidemiology, etiology, pathogenesis, and evaluation of the cutaneous vasculitis patient. [pdfs.journals.lww.com]

Pathophysiology Pathogenesis. The most commonly accepted mechanism of vasculitis is type III hypersensitivity. Soluble circulating antigen-antibody immune complexes (formed in antigen excess) become blocked in blood vessel endothelium. [vetstream.com]

Pathophysiology The pathophysiology of cutaneous vasculitis is thought to revolve around the actual deposition of immune complexes in blood vessels at risk in the skin. [clinicaladvisor.com]

This finding on histological examination is termed “leukocytoclastic vasculitis”. [5] Considering the wide range of potential causes leading to cutaneous small vessel vasculitis, there are subtle variations in the underlying pathophysiology for each cause [en.wikipedia.org]

Pathophysiology Immune complex deposition, with resultant neutrophil chemotaxis and release of proteolytic enzymes and free oxygen radicals, is a key component in the pathophysiology of LCV. [10, 11] In addition, other autoantibodies, such as antineutrophil [emedicine.medscape.com]

It occurs more frequently in small blood vessels, and the pathophysiological mechanism involved is the deposition of immune complexes, usually immunoglobulin M and G, that activate the complement pathways, leading to the production of chemotactic factors [scielo.br]

Moreover we give a short review of the recurrence of IgA nephropathy and HSP after renal transplantation followed by possible strategies for prevention and therapy of recurrent disease. [ncbi.nlm.nih.gov]

Diagnosis This article is available in full to registered subscribers Sign up now to purchase a 30 day trial, or Login Treatment This article is available in full to registered subscribers Sign up now to purchase a 30 day trial, or Login Prevention This [vetstream.com]

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