WordNet

inflammation of a blood vessel

PrepTutorEJDIC

じんましん(蕁麻疹)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2019/08/22 10:05:46」(JST)

wiki en

Urticarial vasculitis (also known as "chronic urticaria as a manifestation of venulitis", "hypocomplementemic urticarial vasculitis syndrome", "hypocomplementemic vasculitis" and "unusual lupus-like syndrome")^[1] is a skin condition characterized by fixed urticarial lesions that appear histologically as a vasculitis.^[2]^:834

Mechanism

Antibodies are usually raised against foreign proteins, such as those made by a replicating virus or invading bacterium. Virus or bacteria with antibodies opsonized or "stuck" to them highlight them to other cells of the immune system for clearance.

Antibodies against self proteins are known as autoantibodies, and are not found in healthy individuals. These autoantibodies can be used to detect certain diseases.

C1q

C1q is an integral component within the complement pathway – a complicated cascade of protein interactions, culminating in an immune response against a broad variety of pathogens. The anti-C1q antibodies found in patients with hypocomplementemic urticarial vasculitis activate C1q, which instigates activation of the entire complement pathway. Consequently, levels of all complement proteins become low.

Impaired classical complement pathway

C1 complex comprising C1q, C1r and C1s

The complement pathway is composed of several subset pathways: the lectin/mannose pathway, alternative pathway and the classical pathway. All pathways culminate in the production of a C3 convertase, which catalyses C3 into its constitutive parts (better detailed here – classical complement pathway).^[3]

In brief, the crucial role of C1q in the pathway is its importance as the first protein to start the complement cascade (which ends in the destruction of the invading bacteria or virus), and its ability to link the two important arms of the immune system – the innate immune system: a broad defence system; and the adaptive immune system: the strong immune response capable of remembering previous infections, allowing fast response against recurrent infections, meaning that people with a normal immune system don't continually catch the same cold or same strain of flu repeatedly.^[3]

Case studies of individuals with HUV have also highlighted other potential complicating factors which it seems the anti-C1q antibodies play a role in. This can mean in some cases the deposition of large immune complexes in the kidney which cannot be cleared by the usual cells of the immune system (e.g. macrophages which are unable to bind the Fc portion of the C1q antibody), leading to further complications.^[4] This seems to be rare, but can occur when a pre-existing renal condition is apparent. Also, there has been some speculation as to an additional autoantibody against an inhibitor protein (in the complement pathway) named C1-inhibitor. The inhibition of C1-inhibitor leads to over-activation of the complement pathway and one protein that builds up controls angioedema (vessel – swelling),^[4] resulting in excess water building up under the skin (the weal appearance).

Diagnosis

A rare autoimmune disease characterized by recurrent urticaria (nettle rash), first described in the 1970s. There is no defined paradigm for the syndrome aetiology and severity in progression. Diagnosis is confirmed with the identification of at least two conditions from: venulitis on skin biopsy, arthritis, ocular inflammation, abdominal pain or positive C1q antibodies to immune complexes.^[5] It is this last category, anti-C1q antibodies, that all HUV patients test positive for.^[4] In vitro experiments and mouse models of the disease have not thoroughly determined the link between these antibodies and the disease, even though the link is so pronounced.

Treatment

Unfortunately there are no known specific therapies for HUV. The regime of prescription steroids and other immunosuppressive drugs aims to dampen the body's production of anti-C1q antibodies.^[5] However, this again renders the individual immunocompromised.

Popular culture

Urticarial vasculitis is featured prominently in the 2010 documentary film Fat, Sick and Nearly Dead.^[6] The main character and narrator suffers from the disease.

References

^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ James, William D.; Berger, Timothy G.; Arthur Hawley Sanford; et al. (2006). Andrews' Diseases of the Skin clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.CS1 maint: Multiple names: authors list (link)
^ ^a ^b Janeway, Travers, Walport and Shlomchik. Immunobiology, 6th Edition, Chapter 2; pp 55–75
^ ^a ^b ^c [Kallenberg Cees G.M. Autoimmunity Reviews 7 (2008) pp612-615]
^ ^a ^b [Ozen S. Education Review. Journal of Pediatric Nephrology (2010) 25:pp1633-1639]
^ "Fat, Sick & Nearly Dead". 1 September 2014 – via www.imdb.com.

External links

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 蕁麻疹性血管炎 urticarial vasculitis
2. 新規発症の蕁麻疹 new onset urticaria
3. 慢性蕁麻疹：臨床症状、診断、病因および自然経過 chronic urticaria clinical manifestations diagnosis pathogenesis and natural history
4. 血管炎の皮膚病変を有する成人の評価 evaluation of adults with cutaneous lesions of vasculitis
5. 血清病および血清病様反応 serum sickness and serum sickness like reactions

English Journal

Clinical Immunology Review Series: An approach to the patient with a periodic fever syndrome.

Lachmann HJ.SourceNational Amyloidosis Centre and Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine, University College London Medical School, Royal Free Campus, London, UK.
Clinical and experimental immunology.Clin Exp Immunol.2011 Sep;165(3):301-9. doi: 10.1111/j.1365-2249.2011.04438.x. Epub 2011 Jul 7.
ARTICLES PUBLISHED IN THIS CLINICAL IMMUNOLOGY REVIEW SERIES allergy in childhood, allergy diagnosis by use of the clinical immunology laboratory, anaphylaxis, angioedema, management of pulmonary diseases in primary antibody deficiency, periodic fever syndrome, recurrent infections in childhood, rec
PMID 21736563

Refractory urticarial vasculitis as a complication of ulcerative colitis successfully treated with rituximab.

Swaminath A, Magro CM, Dwyer E.SourceFrom the *Division of Digestive and Liver Disease, Department of Medicine, Columbia University Medical Center; †Dermatopathology, Weill Medical College of Cornell University; and ‡Division of Rheumatology, Department of Medicine, Columbia University Medical Center, New York, NY.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases.J Clin Rheumatol.2011 Aug;17(5):281-3.
Ulcerative colitis can be complicated by the development of leukocytoclastic vasculitis, a cutaneous vasculitis with the potential for systemic involvement. We present a man with a history of ulcerative colitis complicated by end-stage liver disease secondary to sclerosing cholangitis requiring a li
PMID 21778903

Japanese Journal

Hypocomplementemic Urticarial Vasculitis Syndrome is Associated with High Levels of Serum IgG4: A Clinical Manifestation that Mimics IgG4-related Disease

Wakamatsu Ryo,Watanabe Hitoshi,Suzuki Keisuke,Suga Norihiro,Kitagawa Wataru,Miura Naoto,Nishikawa Kazuhiro,Yokoi Toyoharu,Banno Syogo,Imai Hirokazu
Internal Medicine 50(10), 1109-1112, 2011
… A 58-year-old Japanese woman presented with recurrent abdominal pain, chronic urticaria, and petechiae on her extremities, and hypocomplementemia, findings that were consistent with hypocomplementemic urticarial vasculitis syndrome (HUVS). … A skin biopsy demonstrated leukocytoclastic vasculitis with IgG4 deposition in the vascular lumen and vascular walls. …
NAID 130000649583

Clinical efficacy of reserpine as "add-on therapy" to antihistamines in patients with recalcitrant chronic idiopathic urticaria and urticarial vasculitis

DEMITSU Toshio,YONEDA Kozo,KAKURAI Maki,SASAKI Kaoru,HIRATSUKA Yu-ichiro,AZUMA Ryu-ichi,YAMADA Tomoko,UMEMOTO Naoka
Journal of dermatology 37(9), 827-829, 2010-09-01
NAID 10027067334

Related Pictures

★リンクテーブル★

リンク元	「蕁麻疹群」「蕁麻疹様血管炎」
拡張検索	「hypocomplementemic urticarial vasculitis」
関連記事	「urticarial」

「蕁麻疹群」

Classification	D ICD-10: L95.8 (ILDS L95.810);
External resources	eMedicine: article/1085087;

Urticarial vasculitis
Specialty	Dermatology, immunology

　　[★]

1. 蕁麻疹 urticaria

「蕁麻疹様血管炎」

　　[★]

英: urticarial vasculitis
関: 血管炎、

24時間以上、蕁麻疹様皮疹、多形紅斑様皮疹が持続。消退後に紫斑や色素沈着を残す。(NDE.110)

「hypocomplementemic urticarial vasculitis」

　　[★] 低補体血症性蕁麻疹様血管炎

「urticarial」

　　[★]

adj.

蕁麻疹の、じん麻疹の、じんま疹の、蕁麻疹様の

関: hives、urticaria

[Bolognia-1] Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.

[Andrews-2] James, William D.; Berger, Timothy G.; Arthur Hawley Sanford; et al. (2006). Andrews' Diseases of the Skin clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.CS1 maint: Multiple names: authors list (link)

[Janeway-3] Janeway, Travers, Walport and Shlomchik. Immunobiology, 6th Edition, Chapter 2; pp 55–75

[Kallenberg-4] [Kallenberg Cees G.M. Autoimmunity Reviews 7 (2008) pp612-615]

[Ozen-5] [Ozen S. Education Review. Journal of Pediatric Nephrology (2010) 25:pp1633-1639]

[6] "Fat, Sick & Nearly Dead". 1 September 2014 – via www.imdb.com.

v t e Cutaneous vasculitis and other vascular-related cutaneous conditions (L95, 709.1)
Cutaneous vasculitis	Erythema elevatum diutinum Capillaritis Urticarial vasculitis Nodular vasculitis
Microvascular occlusion	Calciphylaxis Cryoglobulinemic purpura/Cryoglobulinemic vasculitis vascular coagulopathy: Livedoid vasculitis Livedoid dermatitis Perinatal gangrene of the buttock Malignant atrophic papulosis Sneddon's syndrome
Purpura	Nonthrombocytopenic purpura: Cryofibrinogenemic purpura Drug-induced purpura Food-induced purpura IgA vasculitis Obstructive purpura Orthostatic purpura Purpura fulminans Purpura secondary to clotting disorders Purpuric agave dermatitis Pigmentary purpuric eruptions Solar purpura Traumatic purpura Waldenström hyperglobulinemic purpura Painful bruising syndrome ungrouped: Paroxysmal hand hematoma Postcardiotomy syndrome Deep vein thrombosis Superficial thrombophlebitis Mondor's disease Blueberry muffin baby Fibrinolysis syndrome
Systemic vasculitis	see Template:Systemic vasculitis
Vascular malformations	Arteriovenous malformation Bonnet–Dechaume–Blanc syndrome Cobb syndrome Parkes Weber syndrome Sinusoidal hemangioma lymphatic malformation Hennekam syndrome Aagenaes syndrome telangiectasia: Generalized essential telangiectasia Hereditary hemorrhagic telangiectasia Unilateral nevoid telangiectasia
Ulcer	Venous ulcer Arterial insufficiency ulcer Hematopoietic ulcer Neuropathic ulcer Acroangiodermatitis
Lymphedema	see Template:Lymphatic vessel disease
Ungrouped vascular-related cutaneous conditions	Raynaud's phenomenon Thromboangiitis obliterans Erythromelalgia Septic thrombophlebitis Arteriosclerosis obliterans Bier spots/Marshall–White syndrome Cholesterol embolus Reactive angioendotheliomatosis Trousseau's syndrome

匿名

検索

案内

案内

urticarial vasculitis