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].

• Weight Loss

  Additionally, he reported of asthenia, anorexia and weight loss during the prior month. Chest radiography showed an enlarged mediastinum and thoracoabdominal-pelvic CT scan revealed a bulky left hilar mass. [ncbi.nlm.nih.gov]

  […] or more of the following ten criteria are required: More than 4 kg (8.8 lb) weight loss Lace-like purplish discoloration of the skin (livedo reticularis) Testicular pain Pain in a muscle or group of muscles ( myalgias ) Damage to peripheral nerves Elevation [en.wikipedia.org]

  The patient sought emergency treatment presenting with headache, arthralgia, cutaneous rash, and weight loss (4 kg) in the last 20 days. [scielo.br]

  General symptoms such as fevers, weight loss and muscle aches may occur. Five to 25% of people with CSVV can have joint, genitourinary or gastrointestinal symptoms. This raises the possibility of internal involvement. [dermcoll.edu.au]

• 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]

• Burning Pain

  Itching or a burning sensation may occur There is a female preponderance Investigations The hallmark of this condition is polyclonal hypergammaglobulinaemia primarily composed of IgG The ESR is often raised and there can be associated anaemia and leucopenia [pcds.org.uk]

• Blood in Stool

  […] in stool in the case of intestinal involvement Related Stories Overview of the roles of neutrophils in systemic autoimmune and autoinflammatory diseases Intensity and longevity of SARS-CoV-2 vaccination response in patients with immune-mediated inflammatory [news-medical.net]

  Routine urine examination showed albuminuria in five patients, while urine microscopy demonstrated blood cells in two patients and pus cells and bacilli in one patient each. The stool for occult blood was positive in one patient. [dx.doi.org]

• Melena

  There was history of pain abdomen in 11 patients, diarrhea in 3 patients and melena and hemoptysis in 1 patient each. Twenty five patients gave history of drug intake prior to the appearance of lesions. [dx.doi.org]

  Gastrointestinal manifestations: haematemesis, melena, peptic ulceration, esophageal ulceration. These usually manifest with nausea, vomiting, diarrhea and anorexia. Congestive heart failure manifestations. Lung involvement leads to pneumonitis. [drmhijazy.com]

• 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]

• Arthralgia

  We describe 4 patients who presented with palpable purpura, arthralgia or arthritis, leukopenia, and antineutrophil cytoplasmic antigen (ANCA) positivity most likely as a result of a hypersensitivity reaction to cocaine-levamisole induced vasculopathy [ncbi.nlm.nih.gov]

  A few days after the initiation of treatment, the patient developed persistent arthralgia in the upper and lower limbs. [scielo.br]

• Cutaneous Manifestation

  Most of the cutaneous manifestations of APS have been found to be thrombotic on histopathology without evidence of perivascular inflammation. Vasculitis is usually seen in APS patients with coexistent Systemic Lupus Erythematosus (SLE). [ncbi.nlm.nih.gov]

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

• Skin Ulcer

  With a broad spectrum of differential diagnosis, the new appearance of a skin rash in transplanted patients can be challenging. We present the case of a 69-year-old man with palpable purpura, skin ulcerations, and diffuse arthralgias. [ncbi.nlm.nih.gov]

  ulcers healing in 5 of 5 patients, and complete resolution of widespread urticarial vasculitis in 1 patient. 6 During 6 months of follow-up, purpura recurred in 2 patients and ulcers recurred in 1 patient; however, all 3 patients responded to a second [jamanetwork.com]

• 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]

• Withdrawn

  Once vasculitis is under control, medications may be cautiously withdrawn, with the hope that the patient will sustain a long remission or cure, without the need for further treatment. [webmd.com]

  New lesions may appear almost daily until treatment is initiated or a trigger withdrawn. Successive flares can occur at various intervals, interspaced from days or weeks to sometimes years. [rheumatologyadvisor.com]

• Hematuria

  Figure 3: Dermal-renal vasculitis in a chronic hepatitis patient presenting as recurrent purpura of lower legs with cryoglobulinemia, hypocomplementemia and hematuria/ proteinuria. [omicsonline.org]

  Patients with hematuria or abnormal renal function as measured by creatinine or with acute onset of hypertension should be seen by a nephrologist. [clinicaladvisor.com]

  Extracutaneous involvement was noted in 29% of patients' i.e., joint pain, abdominal pain and hematuria. Leukocytoclastic vasculitis was the commonest histologic diagnosis. [e-ijd.org]

  Over the next several years, her rash became persistent, progressed to involve her forearms and abdomen, and was accompanied by hematuria and increases in serum creatinine levels. She had no pulmonary or neurologic symptoms. [jamanetwork.com]

• Burning Sensation

  Rituximab therapy was well tolerated, with only 1 incident of a burning sensation across the nose, cheeks, and ears during infusion that resolved when the pump was stopped for 30 minutes and then slowly restarted. [jamanetwork.com]

  Burning sensation and pain may be mild or sometimes severe depending on the site and extent of the lesions. Arthralgia and swollen joints. Kidney involvement : leads to manifestations of glomerulonephritis. [drmhijazy.com]

  Itching or a burning sensation may occur There is a female preponderance Investigations The hallmark of this condition is polyclonal hypergammaglobulinaemia primarily composed of IgG The ESR is often raised and there can be associated anaemia and leucopenia [pcds.org.uk]

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.

• Neutrophilia

  Presence of neutrophilia was significantly increased in biopsies from group a) when compared with the other two groups. Also, a trend to higher frequency of pustular dermatosis was found in patients from group a). [ncbi.nlm.nih.gov]

  Hematology revealed a mild normocytic normochromic anemia and a mild mature neutrophilia. On serum chemistry there was a slightly decreased ALT, slightly elevated amylase, slightly elevated creatine kinase, and low iron. [ecommons.cornell.edu]

  Neutrophilia or eosinophilia may occur. Hypocomplementaemia is usual. Circulating immune complexes are often demonstrated. [drmhijazy.com]

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]

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]

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 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]

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]

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]

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]

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]

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]

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]

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]

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]

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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