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Cutaneous Vasculitis

Vasculitis of the Skin

Cutaneous vasculitis is a disorder in which the vessel walls become inflamed with consequent hemorrhage and ischemia. It can affect individuals of all ages and can manifest in different ways ranging from a self-limiting disorder involving one organ to a life-threatening condition involving multiple organs.


Presentation

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]
Cat Scratch
Vascular Disease
  • Reduced blood flow :: Stasis , High fat content reduces blood flow in thigh and buttocks :: medication that constricts blood :: vessels :: Varicose veins :: Peripheral vascular disease :: Cold weather Malignancy Auto immune disorders like SLE , Dermatomyositis[dramitdutta.com]
  • Vascular diseases in McKee′s Pathology of the skin with clinical correlations. In: Calonje E, Brenn T, Lazar A, editors. Vol. 1, 4 th ed. China: Elsevier Saunders; 2012. p. 658-710. 2.[ijdpdd.com]
  • disease (eg, rheumatoid arthritis, Sjögren's syndrome, SLE) Inflammatory bowel disease Neoplastic (eg, hairy cell leukaemia) Causes of larger-vessel vasculitis (uncommon) Wegener's vasculitis Churg-Strauss syndrome Polyarteritis nodosa Kawasaki disease[patient.info]
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
  • Cutaneous manifestations are frequent during the course of many systemic vasculitis. Lesions are often not specific, the most frequent being palpable purpura.[ncbi.nlm.nih.gov]
  • 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]
  • Cutaneous manifestations including erythema, papules, subcutaneous nodules, and purpura sometimes occur; however, to date, only six cases have been histologically confirmed to have genuine vasculitis.[ncbi.nlm.nih.gov]
  • 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]
  • Dear Sir, Crohn's disease is associated with a variety of cutaneous manifestations many of which parallel disease activity.[academic.oup.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]
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]
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]

Workup

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
  • Based on the clinical features and the examination data, there is a possibility that a Chlamydia trachomatis infection played a pivotal role in the pathogenesis of those vasculitides.[ncbi.nlm.nih.gov]
  • 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]
Mycobacterium Chelonae
  • Mycobacterium chelonae is a non-tuberculous, rapidly growing mycobacteria and is widely distributed in the natural environment.[ncbi.nlm.nih.gov]
Bartonella Henselae
Multiple Ulcerations
  • Cutaneous examination revealed multiple ulcers 0.5-1 cm in size [Figure:l] with irregular margins with several of them showing a black eschar, situated over both lower legs. Also seen were a few tender erythematous papulonodules over the same sites.[ijdvl.com]
HLA-B27
  • A 45-year-old HLA B27 negative male developed an asymmetric and additive arthritis and a cutaneous leukocytoclastic vasculitis with IgM and complement papillary deposition along hypodermic vessel walls about three weeks after the onset of respiratory[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • Clinicians should be aware of this serious adverse event because any continuation of treatment may be fatal.[ncbi.nlm.nih.gov]
  • Topical treatment with high potency corticosteroids may be useful in the treatment of bullous reactions.[ncbi.nlm.nih.gov]
  • We further propose prophylactic treatment of renal complications in patients with Henoch-Schönlein purpura.[ncbi.nlm.nih.gov]
  • After treatment with cyclophosphamide, the previously resistant abdominal symptoms resolved.[ncbi.nlm.nih.gov]

Prognosis

  • Cutaneous ulcers associated with vasculitis are rarely reported in adult-onset dermatomyositis (DM), and are often resistant to treatment, resulting in a poor prognosis.[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]
  • Appropriate examinations revealed clinical stage III/B with favorable prognosis (IPS 2). After eight cycles of ABVD therapy complete remission was achieved as confirmed by FDG-PET.[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]
  • In most cases it has a favorable prognosis, although a small percentage of patients may develop residual renal damage.[ncbi.nlm.nih.gov]

Etiology

  • 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]
  • Secondary objectives of the project will be: 1) definition of the etiological agents that are most frequently associated with CLV; 2) search for possible correlations between causative agent and peculiar clinical and/or histopathological aspects; 3) evaluation[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • 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]

Prevention

  • 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]
  • Physicians should keep in mind this fact to make an early diagnosis of infection and, consequently, prevent life-threatening complications.[ncbi.nlm.nih.gov]
  • Therefore, efforts to prevent further bites and monitor for evidence of systemic vasculitis should be made in patients with bullous reactions to bedbug bites.[ncbi.nlm.nih.gov]
  • Abstract The purpose of this case report and review of the literature is to provide an exploration of the clinical symptoms, diagnosis, prevention, and management of propylthiouracil (PTU)-associated vasculitis in the intensive care setting.[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]

References

Article

  1. Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol. 2006;24:414–29.
  2. Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: Diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol. 2005;27:504–28.
  3. Jeanette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92.
  4. Khetan P, Sethuraman G, Khaitan BK, et al. An aetiological and clinicopathological study on cutaneous vasculitis. Indian J Med Res. 2012 Jan; 135 (1): 107 -113
  5. Ioannidou DJ, Krasagakis K, Daphnis EK, et al. Cutaneous small-vessel vasculitis: an entity with frequent renal involvement. Arch Dermatol 2002 Mar; 138 (3): 412-4
  6. Tai YJ, Chong AH, Williams RA, et al. Retrospective analysis of adult patients with cutaneous leukocytoclastic vasculitis. Australas J Dermatol 2006 May; 47 (2): 92-6
  7. Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. High titer of serum antiphospholipid antibody levels in adult Henoch-Schönlein purpura and cutaneous leukocytoclastic angiitis. Arthritis Rheum. 2008 Apr 15; 59(4):561-
  8. de Hollanda A, Beucher A, Henrion D, et al. Systemic and immune manifestations in myelodysplasia: a multicenter retrospective study. Arthritis Care Res (Hoboken). 2011 Aug; 63(8):1188-94.
  9. Solans-Laqué R, Bosch-Gil JA, Pérez-Bocanegra C, et al. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. J Rheumatol. 2008 Feb; 35(2):294-304.
  10. Sais G, Vidaller A, Jucgla A, et al. Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Arch Dermatol 1998; 134 (3): 309-15

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Last updated: 2019-07-11 21:11