Sunday, January 25, 2009

Urticarial Vasculitis


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Definition of Vasculitis:
A varied group of disorders which all share a common underlying problem of inflammation of a blood vessel or blood vessels. The inflammation may affect any size blood vessel, anywhere in the body. It may affect either arteries and/or veins. The inflammation may be focal, meaning that it affects a single location within a vessel; or it may be widespread, with areas of inflammation scattered throughout a particular organ or tissue, or even affecting more than one organ system in the body.

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  1. Definition of Vasculitis:
    A varied group of disorders which all share a common underlying problem of inflammation of a blood vessel or blood vessels. The inflammation may affect any size blood vessel, anywhere in the body. It may affect either arteries and/or veins. The inflammation may be focal, meaning that it affects a single location within a vessel; or it may be widespread, with areas of inflammation scattered throughout a particular organ or tissue, or even affecting more than one organ system in the body.

    Authors
    Jerry D Brewer, MD
    Mark DP Davis, MD
    Section Editors
    John H Stone, MD, MPH
    Sarbjit Saini, MD
    Deputy Editor
    Anna M Feldweg, MD



    Last literature review version 16.3: October 2008 | This topic last updated: September 19, 2007 (More)


    INTRODUCTION — Urticarial vasculitis (UV) is considered a clinicopathologic entity consisting of two elements:

    Clinical manifestations of urticaria
    Histopathological evidence of cutaneous leukocytoclastic vasculitis (LCV) of the small vessels, largely involving the postcapillary venules [1-4].
    Although UV is most commonly idiopathic, it can occur in association with autoimmune diseases, drug reactions, infections, or malignancy. UV may be systemic or localized to the skin [5]. Hypocomplementemia, when present, may be associated with extensive vasculitis and systemic features that most commonly involve the musculoskeletal, pulmonary, renal, and/or gastrointestinal systems. Discussion of this topic is confounded by the lack of accepted criteria for distinguishing UV from other cutaneous vasculitides and associated conditions.

    A detailed review of the literature has been published by the authors [6]. A practical approach is presented here. The epidemiology, clinical features, laboratory and biopsy findings, differential diagnosis, and prognosis of UV will be reviewed herein. (See "Etiology and diagnosis of urticaria" for a more general discussion of urticaria and related conditions).

    TERMINOLOGY — UV is characterized by a variety of cutaneous, systemic, and serologic features, which have resulted in a confusing array of names for this disorder(s).

    UV likely represents a continuum of disease, ranging from urticaria with minimal vasculitis, to life- or organ-threatening systemic vasculitis with minimal urticaria. Some patients have low complement levels, a feature that is associated with more severe disease and with systemic involvement. By comparison, patients with normal complement levels are more likely to have mild disease (sometimes referred to as normocomplementemic urticarial vasculitis [NUV]).
    Urticarial Vasculitis

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  2. Urticarial Vasculitis
    Darius Mehregan, MD, Associate Professor, Hermann Pinkus Chairman of Dermatology, Department of Dermatology, Wayne State University of Michigan; Clinical Associate Professor of Pathology, University of Toledo; Dermatopathologist, Pinkus Laboratory; Consulting Staff, J Dingell Veterans Affairs Medical Center
    Iltefat Hamzavi, MD, Staff Physician, Department of Dermatology, Wayne State University School of Medicine, Michigan

    Background
    Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histologically show changes of leukocytoclastic vasculitis. Urticarial vasculitis may be divided into normocomplementemic and hypocomplementemic variants. Both subsets can be associated with systemic symptoms (eg, angioedema, arthralgias, abdominal or chest pain, fever, pulmonary disease, renal disease, episcleritis, uveitis). The hypocomplementemic form more often is associated with systemic symptoms and has been linked to connective-tissue disease (ie, systemic lupus erythematosus [SLE]).1

    Pathophysiology
    The pathophysiology of urticarial vasculitis is similar to other forms of cutaneous small vessel leukocytoclastic vasculitis. Urticarial vasculitis is a type III hypersensitivity reaction in which antigen-antibody complexes are deposited in the vascular lumina. This reaction results in complement activation and chemotaxis of neutrophils. These cells release various proteolytic enzymes, such as collagenase and elastase, resulting in damage to the vascular lumina. Some authors have speculated that eosinophils may be involved in the early stages of the vasculitic lesions. Patients with hypocomplementemic urticarial vasculitis are more likely to show autoantibodies to C1q and vascular endothelial cells.2 The presence of antineutrophilic cytoplasmic antibodies is rare

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  3. Immunology Allergy and Rheumatology Section, Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA.

    Urticarial vasculitis is a clinico-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. The cutaneous features may include painful, burning or pruritic skin lesions, the persistence of individual lesions greater than 24 hours, palpable purpura, pronounced central clearing of lesions, and residual hyperpigmentation following resolution. However, because clinical characteristics of urticarial vasculitis may overlap with those of allergic urticaria, confirmation of the diagnosis requires a lesional skin biopsy. This condition is idiopathic in many patients but can also occur in the context of autoimmune disorders, infections, drug reactions, or as a paraneoplastic syndrome. In idiopathic urticarial vasculitis common laboratory findings are an elevation of erythrocyte sedimentation rate and reduction of serum complement. An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized as some clinical manifestations of disease overlap and C1q autoantibodies may be present in both diseases. Normo-complementemic patients usually have minimal or no systemic involvement and often have a better prognosis. On-the-other-hand, hypocomplementemic patients have the propensity to have more severe multi-organ involvement. Response to treatment is variable and a wide variety of therapeutic agents may be efficacious. Initial recommendations for treatment of urticarial vasculitis manifest only as non-necrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required. With necrotizing skin lesions or visceral involvement, corticosteroids are regularly indicated. Cases of severe corticosteroid resistant urticarial vasculitis or where corticosteroid morbidity is evident [table: see text] may require treatment with other immunosuppressive agents such as azathioprine, cyclophosphamide, or cyclosporine.

    PMID: 12221865 [PubMed - indexed for MEDLINE]

    Related ArticlesReviewUrticarial vasculitis. [Int Angiol. 1995] Persistent urticarial eruption in an asthmatic patient. [Ann Allergy Asthma Immunol. 1996] Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis. [J Am Acad Dermatol. 2007] [Urticarial vasculitis syndrome. A case report and review of the literature] [Minerva Med. 1997] ReviewUrticarial vasculitis. [Curr Opin Rheumatol. 2000] » See Reviews... | » See All...

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  4. Urticarial Vasculitis
    Urticarial vasculitis is a subset of vasculitis characterized clinically by urticarial skin lesions and histologically by necrotizing vasculitis. Immune complex deposition (a type II immune complex reaction) in the postcapillary venules is the probable cause. There is a spectrum of clinical and laboratory features.

    Many patients have minimal signs or symptoms of systemic disease. Systemic symptoms include angioedema (42%), arthralgias (49%), pulmonary disease (21%), and abdominal pain (17%). Thirty-two percent have hypocomplementemia, 64% have lesions that last more than 24 hours, 32% have painful or burning lesions, and 35% have lesions.

    Urticarial plaques in most patients with typical chronic urticaria resolve completely in less than 24 hours and disappear while new plaques appear in other areas. Urticarial vasculitis plaques persist for 24 to 72 hours and may have residual changes of purpura, scaling, and hyperpigmentation. The lesions are burning and painful rather than itchy.

    Diagnosis.
    Biopsy shows a histologic picture that is indistinguishable from that seen in cutaneous necrotizing vasculitis (palpable purpura). Fragmentation of leukocytes and fibrinoid deposition occur in the walls of postcapillary venules, a pattern called leukocytoclastic vasculitis. There is an interstitial neutrophilic infiltrate of the dermis. Anti-C1q autoantibody develops in disorders.

    Patients with hypocomplementemia may have an immunofluorescent pattern of immunoglobulins or C3 as determined by routine direct immunofluorescence. As with typical cutaneous vasculitis, most patients have an elevated erythrocyte sedimentation rate. Patients with more severe involvement have hypocomplementemia (hypocomplementemic urticarial vasculitis syndrome) with depressed CH50 , C1q, C4, or C2. Direct immunofluorescence in patients with hypocomplementemia shows deposition of Ig and C3; 87% have fluorescence of the blood vessels, and 70% have fluorescence.

    Treatment.
    Prednisone in dosages exceeding 40 mg/day.

    Other medications reported to be effective are indomethacin (25 mg three times daily to 50 mg four times daily), colchicine (0.6 mg two or three times daily), dapsone (up to 200 mg/day), low-dose oral methotrexate.
    New Treatment for Urticarial Vasculitis

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  5. Urticarial vasculitis with haemorrhagic vesicles successfully treated with reserpine
    T Demitsu*†, K Yoneda‡, E Iida†, M Takada†, R Azuma†, N Umemoto†, Y Hiratsuka†, T Yamada§, M Kakurai†
    †Department of Dermatology, Jichi Medical University Saitama Medical Centre, ‡Department of Dermatology, Jichi Medical University School of Medicine and §Department of Dermatology, Faculty of Medicine, Kagawa University, *Corresponding author, Department of Dermatology, Jichi Medical University Saitama Medical Centre, 1-847 Amanuma-cho, Omiya-ku, Saitama 330-8503, Japan, tel. +81 48 647 2111; fax +81 48 648 5188; E-mail: demitsu@omiya.jichi.ac.jp
    DOI: 10.1111/j.1468-3083.2007.02528.x

    Copyright Journal compilation © 2008 European Academy of Dermatology and Venereology
    Urticarial vasculitis with haemorrhagic vesicles successfully treated with reserpine

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  6. have a patient with urticarial vasculitis diagnosed by biopsy. She is 70 yrs old. She is presently taking ceterizin 20 mg., ranitidine 150 b.i.d. Dox epin25 h.s. for the past one month she is on prednisone, starting at 50 mg daily, tapering to 10 mg daily. She still continues to have urticaria and burning but not too bad. She has been tried on dapson and colchisein past with no improvement.

    I would like to take her off prednisone as she has osteoporosis. What would you suggest to add? Methotrexate or cyclosporine? What dose and for how long? All her blood work is normal. Thank very much.
    My approach to urticarial vasculitis would include:

    1) Determine whether there is prominent involvement of internal organs, particularly in the kidney. If there is such involvement, more aggressive therapy may be indicated.

    2) Determine whether the urticarial vasculitis is secondary to a systemic disease. The most common associations are usually with systemic lupus, mixed essential oryoglobulinemia (including that associated with chronic hepatitis C infection), other paraprotein states, drug reactions. I assume that you have checked the serum C3, C4 levels looking for evidence of hypocomplementemic vasculitis suggesting systemic involvement. Treatment of any associated systemic disease would be indicated.

    3) Assuming that there is no underlying systemic illness, my considerations for steroid- sparing treatment would be:

    a) My first choice because of its very low potential for adverse side effects would be hydroxychloroquine starting with 200 mcg/day. However, it may take several weeks before benefit is seen. Eye checkup (retina) - baseline and every 6 months to rule out hydroxychloroquine toxicity.

    b) If this approach is not successful in allowing tapering of steroid dosage, try methotrexate 15-20 mg once a week. Check liver function test and CBC every month for the first 6 months, then less frequently.

    c) If this is not successful, consider cyclosporine at 3mg/kg/day monitoring serum cyclosporine levels to make sure not in potential toxic range (serum levels > 250). Check renal function q 1-2 months initially. Stop if rising serum creatinine and/or myopathic symptoms.

    d) I would put cyclophosphamide lower on my list of choices because of more potential for adverse effects. If hematuria occurs would have to differentiate between drug adverse effects and renal involvement by the underlying disease

    Meanwhile, gradually increase doxepin dose taken hs if the current dose does not induce sleepy "hang-over" in the following morning. May be able to reach doses of 50-100 mg hs.

    I have enclosed abstracts of some reviews of this subject for your interest.

    Curr Opin Rheumatol. 2000 Jan;12(1):24-31.
    Urticarial vasculitis.
    Wisnieski JJ.
    Louis Stokes Cleveland Department of Veterans Affairs Medical Center, OH 44106, USA.

    Chronic or recurrent urticarial lesions are common in both primary care and referral medicine. Diagnosis and treatment are usually a challenge for both the patient and the medical practitioner. Most patients are eventually diagnosed with chronic idiopathic urticaria. IgG autoantibody to IgE receptor or IgE itself causes urticarial lesions in 30% of these patients. Only a minority (approximately 10%) of patients with chronic urticarial lesions have urticarial vasculitis. Although some cases are benign, urticarial vasculitis by itself can cause significant morbidity, and it is often a manifestation of a serious illness. Successful diagnosis and treatment of urticarial vasculitis requires careful assessment over time for underlying diseases like systemic lupus erythematosus, hypocomplementemic urticarial vasculitis syndrome, Sjogren's syndrome, and mixed cryoglobulinemia.


    Dermatol Clin. 2002 Jul;20(3):449-58.
    Hepatitis C and the skin.
    Jackson JM.
    Division of Dermatology, University of Louisville School of Medicine, 444 South First Street, Louisville, KY 40202, USA.

    Hepatitis C is an important and common cause of chronic hepatitis and cirrhosis. Cutaneous manifestations are often the first signs of infection. Dermatologists must be aware of these manifestations, because early diagnosis is the best treatment. HCV Ab by ELISA should be ordered in patients with LCV-urticarial vasculitis, cryoglobulinemia, lichen planus, Sjogren's syndrome, unexplained pruritus, PCT, PAN, chronic urticaria, patients starting methotrexate, unexplained pruritus, and any patient initiating therapy with a potentially hepatotoxic drug.
    Treatment of urticarial vasculitis

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  7. Urticarial vasculitis is difficult to treat. We report here on a 40-year-old woman with a 16-year history of idiopathic hypocomplementemic urticarial vasculitis syndrome. Her disease had been resistant to treatment with H1- and H2-blockers, indomethacin, dapsone and interferon alpha but responded to > 25 mg/day prednisolone. Monotherapy with pentoxifylline was also of only minor benefit. Using a combination of dapsone (100 mg/day) and pentoxifylline (1,200 mg/day), we observed a gradual improvement resulting in a complete remission within 8 weeks. Complete control of symptoms could be maintained for 18 months without any serious side-effects. This type of treatment may be of benefit in other therapy-resistant cases of hypocomplementemic urticarial vasculitis syndrome, particularly in view of its excellent tolerance.
    Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline.

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  8. This site is dedicated to my husband who has battled chronic urticaria for over 29 years. For more information on this site please click on this link to go back to the main page Urticaria Research Main Page

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