Vasculitis, Leukocytoclastic, Cutaneous: Disorder characterized by a vasculitic syndrome associated with exposure to an antigen such as a drug, infectious agent, or other foreign or endogenous substance. Its pathophysiology includes immune complex deposition and a wide range of skin lesions. Hypersensitivity or allergy is present in some but not all cases.Vasculitis: Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.Purpura, Schoenlein-Henoch: A systemic non-thrombocytopenic purpura caused by HYPERSENSITIVITY VASCULITIS and deposition of IGA-containing IMMUNE COMPLEXES within the blood vessels throughout the body, including those in the kidney (KIDNEY GLOMERULUS). Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement. Most cases are seen in children after acute upper respiratory infections.Skin Diseases, Vascular: Skin diseases affecting or involving the cutaneous blood vessels and generally manifested as inflammation, swelling, erythema, or necrosis in the affected area.Cryoglobulins: Abnormal immunoglobulins, especially IGG or IGM, that precipitate spontaneously when SERUM is cooled below 37 degrees Celsius. It is characteristic of CRYOGLOBULINEMIA.Skin DiseasesSystemic Vasculitis: A heterogeneous group of diseases characterized by inflammation and necrosis of the blood vessel walls.Retinal Vasculitis: Inflammation of the retinal vasculature with various causes including infectious disease; LUPUS ERYTHEMATOSUS, SYSTEMIC; MULTIPLE SCLEROSIS; BEHCET SYNDROME; and CHORIORETINITIS.Biopsy: Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Group of systemic vasculitis with a strong association with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Skin: The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.Vasculitis, Central Nervous System: Inflammation of blood vessels within the central nervous system. Primary vasculitis is usually caused by autoimmune or idiopathic factors, while secondary vasculitis is caused by existing disease process. Clinical manifestations are highly variable but include HEADACHE; SEIZURES; behavioral alterations; INTRACRANIAL HEMORRHAGES; TRANSIENT ISCHEMIC ATTACK; and BRAIN INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, pp856-61)

*  Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases - Full Text View -...

Vasculitis, Leukocytoclastic, Cutaneous. Epidermolysis Bullosa Acquisita. Keratosis. Sweet Syndrome. Disease. Pathologic ... Small Vessel Vasculitis Including Urticarial Vasculitis Erythema Elevatum Diutinum Medium Vessel Vasculitis Procedure: ... Vasculitis. Epidermolysis Bullosa. Erythema. Hidradenitis. Behcet Syndrome. Pemphigus. Pyoderma. Skin Diseases. Pyoderma ... Hypocomplementemic Urticarial Vasculitis Linear IgA Disease Dermatitis Herpetiformis Erythema Elevatum Diutinum Epidermolysis ...

*  Electrophysiological examination in uveitis: a review of the literature.

10697796 - Pulmonary tuberculosis presenting with cutaneous leukocytoclastic vasculitis.. 9134516 - One health maintenance ... Adamantiades-Behçet disease (ABD) is a chronic, recurrent multisystemic vasculitis of unknown etiology, and classic symptoms ... These results indicated that retinal manifestations were present and that retinal vasculitis may coexist. ... Paraneoplastic retinopathy associated with antiretinal bipolar cell antibodies in cutaneous malignant melanomaOphthalmologyYear ...

*  Paroxetine Side Effects in Detail - Drugs.com

A case of cutaneous leukocytoclastic vasculitis has been reported following treatment with paroxetine. The patient originally ... Margolese HC, Chouinard G, Beauclair L, Rubino M "Cutaneous vasculitis induced by paroxetine." Am J Psychiat 158 (2001): 497 ... Flores-Suarez LF, Vega-Memije ME, Chanussot-Deprez C "Cutaneous vasculitis during selective serotonin reuptake inhibitor ... Very rare (less than 0.01%): Severe cutaneous reactions such as Stevens Johnson syndrome and toxic epidermal necrolysis). ...

*  International Journal of Dermatology: Ingenta Connect Table Of Contents

Role of direct immunofluorescence test in cutaneous leukocytoclastic vasculitis pp. 970-971(2) ... Role of direct immunofluorescence test in cutaneous leukocytoclastic vasculitis pp. 971-972(2) ... Disseminated coccidioidomycosis with cutaneous lesions clinically mimicking mycosis fungoides pp. 958-960(3) Author: Crum, ... The use of anti-tubercular therapy (ATT) as a diagnostic tool in pediatric cutaneous tuberculosis pp. 961-963(3) Authors: ...

*  Relapsing Polychondritis | answerstomyownquestions

Cutaneous vasculitis: The prevalence of biopsy-proven cutaneous (small vessel) leukocytoclastic vasculitis is approximately 10 ... Other cutaneous lesions reported in patients with relapsing polychondritis and vasculitis included the following: *Palpable ... Cutaneous polyarteritis nodosa: A patient with relapsing polychondritis presented with relapsing painful red nodules from 1-3 ... For a vasculitis workup, perform a CBC count with differential; metabolic panel; creatinine, liver transaminase, and serum ...

*  Cutaneous small-vessel vasculitis - Wikipedia

Cutaneous small-vessel vasculitis, also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, ... cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis, is inflammation of ... "leukocytoclastic vasculitis". Considering the wide range of potential causes leading to cutaneous small vessel vasculitis, ... Small vessel cutaneous vasculitis is a diagnosis of exclusion and requires ruling out systemic causes of the skin findings. ...

*  Necrotizing vasculitis - Wikipedia

If patients have this in isolation, it is most likely due to cutaneous leukocytoclastic vasculitis. If the purpura is in ... Necrotizing vasculitis also called Systemic necrotizing vasculitus (SNV) is a category of vasculitis, comprising vasculitides ... Biopsy of these lesions reveal inflammation of the small vessels, termed leukocytoclastic vasculitis, which is most prominent ... Hypersensitivity vasculitis (allergic vasculitis). Usually due to a hypersensitivity reaction to a known drug. Drugs most ...

*  Vasculitis 2013

... 模組 Vasculitis 魏正宗 中山醫學大學附設醫院過敏免疫風濕科主任 Tel: 04 ... IgA vasculitis, Henoch-Schönlein purpura Cryoglobulinemic vasculitis Cutaneous leukocytoclastic angiitis ... Microscopic polyangiitis (Leukocytoclastic vasculitis) * 31. IgA Vasculitis (Henoch-Schönlein purpura)    A specific ... Vasculitis 2013 * 1. 醫學系「免疫與感染學 V 」模組 Vasculitis 魏正宗 中

*  Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis.

... is a rare form of cutaneous vasculitis that involves small and medium sized arteries of the dermis and subcutaneous tissue ... Keywords: Cutaneous polyarteritis nodosa, livedo reticularis, subcutaneous nodules, leukocytoclastic vasculitis, methotrexate. ... Cutaneous polyarteritis nodosa (CPAN) is a rare form of cutaneous vasculitis that involves small and medium sized arteries of ... Cutaneous polyarteritis nodosa (CPAN) is an uncommon and rare form of cutaneous vasculitis. It involves small and medium sized ...

*  Pulmonary vasculitis | Thorax

... essential cryoglobulinaemic vasculitis, and cutaneous leukocytoclastic angiitis, the first three of which are strongly ... Isolated cutaneous leukocytoclastic angiitis without evidence of systemic disease or glomerulonephritis is also included in ... LARGE VESSEL VASCULITIS. The large vessel vasculitides include giant cell (temporal) arteritis and Takayasu arteritis where ... MEDIUM VESSEL VASCULITIS. Medium size vessel vasculitis refers to classical polyarteritis nodosa (PAN) and Kawasaki disease. ...

*  Leukocytoclastic - Wikipedia

... also called leukocytoclastic vasculitis) Cutaneous small-vessel vasculitis (also called cutaneous leukocytoclastic angiitis). ... Leukocytoclastic means "tending to destroy white blood cells", which is exemplified by: Hypersensitivity vasculitis ( ...

*  Chapter 24. Inflammatory Rheumatic Diseases | Pathophysiology of Disease, 6e | AccessMedicine | McGraw-Hill Medical

Cutaneous Small-Vessel (Leukocytoclastic) Vasculitis. ++. A common clinical presentation of immune complex-induced vasculitis ... vasculitis. When it manifests itself in the skin, it is also called cutaneous small vessel or leukocytoclastic vasculitis. ... Cutaneous immune complex vasculitis seldom causes severe pain or tissue breakdown and only rarely leads to long-term injury ( ... vasculitis occur?. *10. What are the three most prominent organ systems affected by immune complex vasculitis? Describe the ...

*  Hypersensitivity vasculitis induced by cefoperazone/sulbactam | Annals of Clinical Microbiology and Antimicrobials | Full Text

Zax RH, Hodge SJ, Callen JP: Cutaneous leukocytoclastic vasculitis: serial histopatologic evaluation demonstrates the dynamic ... "leukocytoclastic vasculitis". Hypersensitivity vasculitis can be divided into neutrophilic and lymhocytic subtypes. The former ... Gibson LE, Su WPD: Cutaneous vasculitis. Rheum Dis Clin North Am. 1990, 16: 309-324.PubMedGoogle Scholar. ... Although hypersensitivity vasculitis has multiple causes, coexistence of hypersensitivity vasculitis and cefoperazone treatment ...

*  1 - Free Forex Varennes

Microscopic polyangiitis Not ANCA associated Henoch-Schönlein purpura Cryoglobulinemic vasculitis Cutaneous leukocytoclastic ... as young as 6 months and Large-vessel vasculitis Giant cell Free Forex Varennes Takayasus arteritis Medium-vessel vasculitis ... Kawasakis disease Polyarteritis nodosa Small-vessel vasculitis ANCA associated Wegeners granulomatosis Churg-Strauss syndrome ...

*  BMJ Best Practice

cutaneous calcinosis. *erythroderma. *vesicobullous lesions. *leukocytoclastic vasculitis. *cutaneous necrosis. *non-scarring ... Some patients have cutaneous involvement alone. May be associated with interstitial lung disease, cardiac involvement, ... An idiopathic inflammatory myopathy characterised by the presence of hallmark cutaneous lesions (e.g., heliotrope rash, ... Some patients present with typical cutaneous manifestations but have delayed-onset, subclinical, or absent muscle disease. [2] ...

*  Leukocytoclastic vasculitis

The disease can be confined to the skin (cutaneous) or it can affect many different organs of the body such as the kidneys, ... Leukocytoclastic vasculitis, also called hypersensitivity vasculitis, describes inflammation of small blood vessels. The term ... Leukocytoclastic vasculitis, also called hypersensitivity vasculitis, describes inflammation of small blood vessels. ... and less often malignancy are some of the various conditions associated with the vasculitis. In the skin, damaged blood vessels ...

*  Browse EE Topics - Essential Evidence Plus

Leukocytoclastic vasculitis Essential Evidence Topics, 8-Apr-2017 Overall Bottom Line: Suspect leukocytoclastic vasculitis (LCV ... Overall Bottom Line: Suspect cutaneous (CL) and mucocutaneous (MCL) or mucosal (ML) leishmaniasis in patients with a history of ... This is often self-limited and confined to the cutaneous superficial vascular plexus. Systemic involvement should be ruled out ...

*  Late-onset Warfarin Induced Skin Necrosis: In a Hemodialysis Patient | Open Access Journals

... vasculitides and other cutaneous lesions like purple toe syndrome, pyoderma gangrenosum, microembolization, leukocytoclastic ... This pattern refers to the changes in cutaneous blood flow, which produces bluish-purple retinacular hyperpigmentation on the ... Later, an incisional skin biopsy was performed, and the pathology showed extensive cutaneous necrosis with associated ... vasculitis, necrotizing fasciitis and purpura fulminans which may present similarly during the early stages [3,17]. ...

*  Churg-Strauss Disease: Background, Pathophysiology, Epidemiology

... inflammatory leukocytoclastic systemic small-vessel vasculitides that are associated with antineutrophil cytoplasm antibodies ( ... Eosinophilic vasculitis syndrome: recurrent cutaneous eosinophilic necrotizing vasculitis. Semin Dermatol. 1995 Jun. 14(2):106- ... The pathology most commonly encountered in skin biopsies is small-vessel leukocytoclastic vasculitis with ensuing blood vessel ... lupus vasculitis, serum sickness vasculitis, and infection-induced immune complex vasculitis. Churg-Strauss disease also has ...

*  Drug eruptions CME | DermNet New Zealand

Coumarin necrosis (cutaneous infarction especially in patients deficient in protein C). *Leukocytoclastic vasculitis (purpura ... Mechanisms of cutaneous reactions to drugs. Immunological hypersensitivities. These represent true allergic reactions. *Type I ... Cutaneous atrophy, striae and telangiectasia due to topical or systemic steroids. *Drug-induced alopecia from cytotoxic ... About 3% of patients admitted to hospital have rashes due to adverse drug reactions but in others the cutaneous signs are due ...

*  Tài liệu Skin Infections chọn lọc - TaiLieu.VN

In the group of vasculitic disorders, cutaneous small-vessel vasculitis, also known as leukocytoclastic vasculitis (LCV), is ... Table 54-14 Causes of Urticaria and Angioedema I. Primary cutaneous disorders A. Acute and chronic urticariaa B. Physical ... Systemic diseases A. Urticarial vasculitis B. Hepatitis B or C infection C. Serum sickness D. Angioedema (hereditary and ... Table 54-12 Causes of Vesicles/Bullae I. Primary cutaneous diseases A. Primary blistering diseases (autoimmune) 1. Pemphigusa 2 ...

*  Tài liệu Respiratory Disease chọn lọc - TaiLieu.VN

In the group of vasculitic disorders, cutaneous small-vessel vasculitis, also known as leukocytoclastic vasculitis (LCV), is ...

*  Cutaneous Vasculitis chọn lọc - TaiLieu.VN

In the group of vasculitic disorders, cutaneous small-vessel vasculitis, also known as leukocytoclastic vasculitis (LCV), is ... Xem 1-9 trên 9 kết quả Cutaneous vasculitis. * Báo cáo y học: " Aggressive cutaneous vasculitis in a patient with chronic ... Vasculitides are not rare diseases. In fact, some vasculitides, such as giant cell arteritis, cutaneous vasculitis, and ANCA- ... Palpable purpuric papules on the lower legs are seen in this patient with cutaneous small vessel vasculitis. (Courtesy of ...

*  Hydroserpine 1 Side Effects in Detail - Drugs.com

... morbilliform and leukocytoclastic vasculitis. Thiazides may induce phototoxic dermatitis. In addition, a rare, distinct entity ... Parodi A, Romagnoli M, Rebora A "Subacute cutaneous lupus erythematosus-like eruption caused by hydrochlorothiazide." ... with clinical and laboratory features indistinguishable from those of subacute cutaneous lupus erythematosus is associated with ...

*  List of cutaneous conditions - Wikipedia

... cutaneous leukocytoclastic angiitis, cutaneous leukocytoclastic vasculitis, cutaneous necrotizing venulitis, hypersensitivity ... Cutaneous ciliated cyst Cutaneous columnar cyst Cutaneous horn (Cornu cutaneum) Cystic basal cell carcinoma Dermal eccrine ... Primary cutaneous immunocytoma Primary cutaneous marginal zone lymphoma Retiform parapsoriasis Secondary cutaneous CD30+ large ... Cutaneous group B streptococcal infection Cutaneous Pasteurella hemolytica infection Cutaneous Streptococcus iniae infection ...

(1/116) Alternating antineutrophil cytoplasmic antibody specificity: drug-induced vasculitis in a patient with Wegener's granulomatosis.

We describe a patient who presented with Wegener's granulomatosis associated with antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) with a cytoplasmic immunofluorescence pattern (cANCA), whose ANCA type changed to antimyeloperoxidase antibodies with a perinuclear immunofluorescence pattern (pANCA) when treated with propylthiouracil, and changed back to anti-PR3 antibodies with cANCA after the medication was discontinued. The patient developed flares of vasculitis symptoms associated with rises in either type of ANCA. Tests for antimyeloperoxidase ANCA were repeatedly negative before the drug was started, strongly implicating the drug as the cause of the episode. This case demonstrates that patients with idiopathic ANCA-positive vasculitis may quickly develop a superimposed drug-associated ANCA-positive vasculitis. Iatrogenic vasculitis should be suspected when a patient with idiopathic vasculitis with one type of ANCA develops the other type of ANCA.  (+info)

(2/116) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.

OBJECTIVE: To retrospectively analyze the clinical symptoms, laboratory findings, and outcomes in patients with microscopic polyangiitis (MPA) who were enrolled in various clinical trials conducted by the French Vasculitis Study Group. METHODS: A cohort of 85 patients meeting the Chapel Hill criteria for MPA participated in the study. Seventy-one of them were included in prospective therapeutic trials. Eighty-one diagnoses were biopsy proven. In the other patients, diagnosis was based on clinical findings. RESULTS: Forty-seven men and 38 women, with a mean +/- SD age of 56.8 +/- 14.6 years, met the criteria for MPA. Their main clinical symptoms were renal manifestations (78.8%), weight loss (72.9%), skin involvement (62.4%), fever (55.3%), mononeuritis multiplex (57.6%), arthralgias (50.6%), myalgias (48.2%), hypertension (34.1%), lung involvement (24.7%; alveolar hemorrhage 11.8%), and cardiac failure (17.6%). The mean +/- SD serum creatinine level before treatment was 2.59 +/- 2.96 mg/dl; 47 patients had renal insufficiency (serum creatinine > 1.36 mg/dl). Eight patients underwent dialysis at the time of diagnosis, and long-term dialysis was necessary for 10 patients. Antineutrophil cytoplasmic antibodies (ANCA) were present in 38 of 51 patients (74.5%), of whom 33 had a perinuclear staining pattern (pANCA) and 5 had a cytoplasmic pattern. Antibodies to proteinase 3 were present in 4 patients and antibodies to myeloperoxidase were detected in 31, as determined by enzyme-linked immunosorbent assay. Of the 30 patients who underwent renal and celiac angiography, 4 had microaneurysms. Of the 29 patients (34.1%) who had relapses, 8 died during or after the relapse. During followup, 28 of the 85 patients (32.9%) died. The mean +/- SD duration of followup of the group was 69.9 +/- 60.6 months. Deaths were less frequent when patients had been treated with steroids and immunosuppressive drugs (13 patients [24.1%]) than with steroids alone (15 patients [48.4%]) (P < 0.01). The 5-year survival rate was 74%. CONCLUSION: This study demonstrated that MPA is a multisystemic disease in which renal symptoms are frequent, but the disease is also associated with general symptoms, arthritis, mononeuritis multiplex, and other manifestations that are also seen in various vasculitides. The rarity of abnormal angiogram findings and the high frequency of pANCA are characteristic of MPA. In most cases, the outcome is comparable with those of other systemic vasculitides, but relapses are frequent.  (+info)

(3/116) Immune complexes from vasculitis patients bind to endothelial Fc receptors independent of the allelic polymorphism of FcgammaRIIa.

Cutaneous leukocytoclastic vasculitis is characterized by the deposition of circulating immune complexes, neutrophil extravasation, and vessel destruction, but mechanisms of circulating immune complexes capture within postcapillary venules are unknown. We demonstrate that circulating immune complexes from sera of vasculitis patients bind to cultured endothelium in an Fc gamma receptor IIa-dependent fashion. In lesional skin, endothelial cells bind immunoglobulin G2 > immunoglobulin G3 and immunoglobulin G4, but not immunoglobulin G1, even before obvious neutrophil transmigration and vessel damage. As the human Fc gamma receptor IIa proteins exist in two allotypes (one with a histidine at position 131, which binds immunoglobulin G1, 2, 3 and the other with an arginine at position 131, which binds immunoglobulin G1, and 3, but is unable to bind immunoglobulin G2), we expected an altered prevalence of histidine 131 forms in vasculitis patients. Sequence analysis, however, revealed an equal distribution of allotypes in patients and controls. In conclusion, circulating immune complex binding to endothelial Fc gamma receptor IIa is among the initial steps in the development of vasculitis. Although immunoglobulin G2 is the predominant subtype precipitated at endothelial surfaces, it is not required for fixing circulating immune complexes to endothelium, because patients homozygote for Fc gamma receptor IIa-arginine 131 equally develop leukocytoclastic vasculitis as those bearing the Fc gamma receptor IIa-histidine 131 allele. As immunoglobulin G1 is virtually absent in leukocytoclastic vasculitis lesions and immunoglobulin G4 does not bind to both Fc gamma receptor IIa alleles, these complexes, in addition to immunoglobulin G2, should contain immunoglobulin G3 in order to fix to vascular Fc gamma receptor IIa, at least in persons homozygous for Fc gamma receptor IIa-arginine 131. KEYWORDS: CD32/immunoglobulin G subtypes/leukocytoclastic vasculitis/microvessels.  (+info)

(4/116) Early vasculitis in the mercuric chloride induced Brown Norway rat model is neutrophil independent.

In the Brown Norway rat, mercuric chloride (HgCl2) induces an autoimmune syndrome characterized by necrotizing vasculitis, predominantly affecting the caecum, and a polyclonal B-cell response. The time course of vasculitis is biphasic, with an alphabeta T-cell independent phase occurring within 24 h, and a T-cell and neutrophil dependent phase, maximal at two weeks. The pathogenesis of the early phase of vasculitis is unclear, and this study aims to examine the role of neutrophils. Rat neutrophils were depleted using cyclophosphamide. RP3, an antirat neutrophil monoclonal antibody, inhibited neutrophil leucocytosis but did not deplete neutrophils. Vasculitis was induced by subcutaneous HgCl2 injection. Serial measurements of peripheral blood leucocyte count were made. Rats were killed after 24 or 72 h. The macroscopic appearance of the caecum was scored by an experienced observer, and samples taken for histological examination. Caecums were excised and myeloperoxidase, a marker enzyme for neutrophil infiltration, assayed. Cyclophosphamide induced marked neutropaenia whereas RP3 inhibited the neutrophilia observed after HgCl2 injection. Vasculitis was present in both treated and control animals, with no significant differences in macroscopic or microscopic scores between the groups. Tissue myeloperoxidase activity was low in all animals and did not differ significantly between groups. The data do not support a role for neutrophils in the initial pathogenesis of vasculitis in this model.  (+info)

(5/116) A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature.

OBJECTIVE: To test the usefulness of the Chapel Hill nomenclature, supplemented with surrogate parameters, as diagnostic criteria for primary vasculitides. METHODS: To prospectively evaluate vasculitis patients according to a standardised clinical and para-clinical programme. In accordance with the Chapel Hill publication surrogate parameters were used: proteinuria, haematuria and red blood cell casts (glomerulonephritis), angiographic or ultrasonic demonstration of aneurysms or stenoses (arteritis), radiological lung infiltrates or cavitations of more than one month's duration (granuloma in the lungs), bloody nasal discharge or crusts, chronic sinusitis, otitis and/or mastoiditis, bone and/or cartilage destruction, and acute hearing loss (granuloma in upper airways). RESULTS: The following entities were diagnosed: giant cell arteritis (n=14), Takayasu arteritis (n=1), polyarteritis nodosa (n=2), Wegener's granulomatosis (n=27), Churg-Strauss syndrome (n=2), microscopic polyangiitis (n=12), Henoch-Schonlein purpura (n=2), cutaneous leucocytoclastic angiitis (n=37), and secondary vasculitis (n=21). Giant cell arteritis and cutaneous leucocytoclastic angiitis were in all cases diagnosed by biopsy. Using the Chapel Hill nomenclature supplemented with surrogate parameters, only 8 of 27 patients were diagnosed with Wegener's granulomatosis, and 3 of 12 cases with microscopic polyangiitis. The number of patients in the remaining diagnostic entities were considered to few to evaluate. CONCLUSIONS: The Chapel Hill nomenclature, supplemented with surrogate parameters, failed to act as diagnostic criteria in Wegener's granulomatosis and microscopic polyangiitis. The following diagnostic criteria are proposed for Wegener's granulomatosis: (1) Biopsy or surrogate parameter for granulomatous inflammation in the respiratory system and (2) Biopsy verified necrotising vasculitis in small to medium sized vessels or biopsy/surrogate parameter for glomerulonephritis or positive PR3-ANCA test and (3) Lack of eosinophilia in blood and biopsy samples. The following diagnostic criteria are proposed for microscopic polyangiitis: (1) Biopsy verified necrotising vasculitis in small vessels and/or glomerulonephritis with few or no immune deposits and (2) Involvement of more than one organ system as indicated by biopsy verified vasculitis in small to medium sized vessels or surrogate parameter for glomerulonephritis and (3) Lack of biopsy and surrogate parameter for granulomatous inflammation in the respiratory system. Using these criteria all Wegener's patients and 9 of 12 patients with microscopic polyangiitis could be diagnosed.  (+info)

(6/116) Unusual manifestations of type II cryoglobulinaemia associated with Waldenstrom's macroglobulinaemia.

Cryoglobulinaemia in association with Waldenstrom's macroglobulinaemia is relatively common, ranging from 8% to 18% of cases; however, < 5% have symptoms or complications. We describe a patient with a history of cutaneous, peritoneal, and fallopian tube vasculitis related to type II cryoglobulinaemia associated with Waldenstrom's macroglobulinaemia. Cytotoxic treatment was initiated (cyclophosphamide, vincristine, and prednisone) and had a good initial response. However, after the third course of chemotherapy, the patient presented with septic shock and died. Even though cryoglobulinaemia is a model of systemic vasculitis, peritoneal and fallopian tube vasculitis associated with type II cryoglobulinemia has not been described previously.  (+info)

(7/116) Leukocytoclastic vasculitis as a complication of infected total hip prosthesis.

We present a case of leukocytoclastic vasculitis, associated with an exacerbation of chronic osteomyelitis in a pseudoarthrosis of the hip. Skin lesions of the lower limb were the most prominent features.  (+info)

(8/116) Follicular dendritic cell tumor of the liver: a clinicopathologic and Epstein-Barr virus study of two cases.

Two cases of hepatic follicular dendritic cell (FDC) tumor are described. Both patients were female, aged 57 and 51 years. They presented with epigastralgia or abdominal fullness and weight loss. The first patient refused surgical resection. She developed progressive polyclonal gammopathy and then bilateral purpura over the legs. Skin biopsy revealed leukocytoclastic vasculitis with granular vascular deposits of IgA and C3. The second patient had marked peripheral blood and tissue eosinophilia. The histological diagnosis was confirmed by positive staining for CD21 and CD23. The stromal lymphocytes were predominantly composed of CD3(+)and CD8(+) cells. In situ hybridization for EBER showed a positive nuclear signal in tumor cells but not in inflammatory cells. Polymerase chain reaction amplification for Exon 3 of the latent membrane protein-1 (LMP-1) gene showed a characteristic 30-bp deletion between nucleotides 168282 and 168253, corresponding to the B95-8 sequence. The unique clinicopathological features of our cases have not been reported for FDC tumors before. The clinical significance of the 30-bp deletion in Exon 3 of the LMP-1 gene in FDC tumor of the liver warrants further investigation.  (+info)



Systemic

  • Necrotizing vasculitis also called Systemic necrotizing vasculitus (SNV) is a category of vasculitis, comprising vasculitides that present with necrosis. (wikipedia.org)
  • When using the influential classification known as the "Chapel Hill Consensus Conference", the terms "systemic vasculitis" or "primary systemic vasculitides" are commonly used. (wikipedia.org)
  • Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. (wikipedia.org)
  • Systemic vasculitis of medium and small arteries, including venules and arterioles. (wikipedia.org)
  • 4. Nomenclature of systemic vasculitis 1. (slideshare.net)
  • Cutaneous polyarteritis nodosa (CPAN) is a rare form of cutaneous vasculitis that involves small and medium sized arteries of the dermis and subcutaneous tissue without systemic involvement. (biomedsearch.com)
  • Localised or diffuse pulmonary infiltrates, lobar collapse, atelectasis, and alveolar haemorrhage are other common manifestations of pulmonary involvement in systemic vasculitis. (bmj.com)
  • This paper will review clinical aspects of pulmonary involvement in the systemic vasculitides and explores the impact which the discovery of auto-antibodies in these disorders has had on our understanding of their pathogenesis. (bmj.com)
  • Antineutrophil cytoplasmic antibodies (ANCA) are circulating autoantibodies which identify a specific subpopulation of patients with systemic vasculitis. (bmj.com)
  • Churg-Strauss disease (CSD) is one of three important fibrinoid, necrotizing, inflammatory leukocytoclastic systemic small-vessel vasculitides that are associated with antineutrophil cytoplasm antibodies (ANCAs). (medscape.com)
  • [ 1 ] provided a sensitive, although nonspecific, marker for a particular group of systemic vasculitides including CSD. (medscape.com)
  • Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels. (wikipedia.org)
  • Polyarteritis nodosa (PAN), also known as panarteritis nodosa, periarteritis nodosa, Kussmaul disease, or Kussmaul-Maier disease, is a systemic vasculitis of small- or medium-sized muscular arteries, typically involving renal and visceral vessels but sparing the pulmonary circulation. (wikipedia.org)
  • Recently, an original study, combining the analysis of more than 100 items used to describe patients' characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively discriminating items to be used as a screening tool for diagnosis in patients suspected of systemic vasculitis. (wikipedia.org)

hypersensitivity vasculitis

  • Cutaneous small-vessel vasculitis, also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis, cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis, is inflammation of small blood vessels (usually post-capillary venules in the dermis), characterized by palpable purpura. (wikipedia.org)
  • Hypersensitivity vasculitis (allergic vasculitis). (wikipedia.org)
  • Here, we report a case of hypersensitivity vasculitis associated with cefoperazone/sulbactam. (biomedcentral.com)
  • A 13-year-old girl with appendicitis developed hypersensitivity vasculitis on the fifth day of cefoperazone/sulbactam therapy. (biomedcentral.com)
  • Hypersensitivity vasculitis resolved gradually after removal of the agent on the seventh day and did not recur. (biomedcentral.com)
  • Although hypersensitivity vasculitis has multiple causes, coexistence of hypersensitivity vasculitis and cefoperazone treatment, and the quite resolution of the disease after removal of the drug, strongly favours a causative relationship. (biomedcentral.com)
  • To our knowledge, this is the first report of a hypersensitivity vasculitis associated with cefoperazone. (biomedcentral.com)
  • Hypersensitivity vasculitis presents usually as drug or infection induced purpura and involves dominantly the small vessels. (biomedcentral.com)
  • Leukocytoclastic vasculitis, also called hypersensitivity vasculitis, describes inflammation of small blood vessels. (aarda.org)
  • on microscopy the appearances are of a hypersensitivity vasculitis, and immunofluorescence demonstrates IgA and C3 (a protein of the complement system) in the blood vessel wall. (wikipedia.org)
  • On the basis of symptoms, it is possible to distinguish HSP from hypersensitivity vasculitis (HV). (wikipedia.org)

purpura

  • For example, if the vasculitis is a manifestation of Henoch-Schönlein purpura, individuals may also experience abdominal pain or blood in the urine. (wikipedia.org)
  • In cases where a cause can be determined, medications and infectious pathogens are most common in adults, while IgA vasculitis (Henoch-Schönlein purpura) frequently affects children. (wikipedia.org)
  • Overall Bottom Line: Suspect leukocytoclastic vasculitis (LCV) in patients with palpable purpura. (essentialevidenceplus.com)
  • Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis Skin: nodules on the elbow, purpura, various others (see cutaneous vasculitis) Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex Heart, gastrointestinal tract, brain, other organs: rarely affected. (wikipedia.org)
  • Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, anaphylactoid purpura, purpura rheumatica, and Schönlein-Henoch purpura, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. (wikipedia.org)
  • Henoch-Schönlein purpura is a small-vessel vasculitis in which complexes of immunoglobulin A (IgA) and complement component 3 (C3) are deposited on arterioles, capillaries, and venules. (wikipedia.org)

lesions

  • Biopsy of these lesions reveal inflammation of the small vessels, termed leukocytoclastic vasculitis, which is most prominent in postcapillary venules. (wikipedia.org)
  • The cutaneous lesions faded within a few days and disappeared on seventh day after discontinuation of cefoperazone/sulbactam therapy. (biomedcentral.com)
  • An idiopathic inflammatory myopathy characterised by the presence of hallmark cutaneous lesions (e.g., heliotrope rash, Gottron's papules). (bmj.com)
  • The average duration of cutaneous lesions is 27.9 months. (wikipedia.org)

Polyarteritis

  • Medium-sized vessel vasculitis   Polyarteritis nodosa Kawasaki disease 3. (slideshare.net)
  • Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis. (biomedsearch.com)
  • Cutaneous polyarteritis nodosa (CPAN) is an uncommon and rare form of cutaneous vasculitis. (biomedsearch.com)
  • 2 ] The etiopathogenesis of cutaneous polyarteritis nodosa remains unclear. (biomedsearch.com)
  • In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" an important diagnostic feature of the vasculitis. (wikipedia.org)

ANCA

  • 6. Antineutrophil cytoplasmic antibodies, ANCA    Cause of ANCA-associated vasculitis (AAV) C-antineutrophil cytoplasmic antibodies (CANCA) against proteinase 3 (PR3) - Associated with GPA and MPA - Disease activity is parallel to C-ANCA titer. (slideshare.net)
  • The discovery that antineutrophil cytoplasmic antibodies (ANCA) are strongly associated with vasculitic disorders and their subsequent characterisation has led to improved understanding of the pathogenic mechanisms underlying vasculitis. (bmj.com)
  • 1 8 There has been some recent interest in looking for ANCA in bronchoalveolar fluid of patients suspected of having pulmonary vasculitis in whom searches for serum ANCA antibodies have been fruitless. (bmj.com)

vessel vasculitides

  • At least 3 out of 5 criteria yields sensitivity and specificity of 95 and 91%: Age at onset ≥ 50 years New onset headache with localized tenderness Temporal artery tenderness or decreased pulsation Elevated ESR ≥ 50 mm/hour Westergren Temporal artery biopsy showing vasculitis with mononuclear cell infiltrate or granulomatous inflammation, usually with multinucleated giant cells These conditions are sometimes considered together with the small vessel vasculitides. (wikipedia.org)
  • Although GPA affects small- and medium-size vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system. (wikipedia.org)

fibrinoid necrosis

  • The skin biopsy showed leukocytoclastic vasculitis with fibrinoid necrosis in the dermal vessels. (biomedsearch.com)

blood vessels

  • It is a form of vasculitis (inflammation of blood vessels) that affects small- and medium-size vessels in many organs. (wikipedia.org)

biopsy

  • Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy. (wikipedia.org)
  • The diagnosis is by skin biopsy and characteristic pathologic feature is a leukocytoclastic vasculitis in the small to medium-sized arterioles of the dermis. (biomedsearch.com)
  • A skin biopsy of a new purpuric lesion from the anterior of the leg was done on the second day of the rash and was revealed a cutaneous vasculitis characterized by perivascular mononuclear cell infiltration. (biomedcentral.com)
  • If the person has kidney failure or cutaneous vasculitis, a biopsy is obtained from the kidneys. (wikipedia.org)
  • On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy. (wikipedia.org)

chronic

  • This tends to occur when the vasculitis is associated with chronic conditions such as connective tissue diseases. (wikipedia.org)
  • Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. (wikipedia.org)
  • Blau syndrome Chronic infantile neurologic cutaneous and articular syndrome Familial cold urticaria (familial cold autoinflammatory syndrome) Familial Mediterranean fever Hyper-IgD syndrome Majeed syndrome Muckle-Wells syndrome TNF receptor associated periodic syndrome (familial Hibernian fever, TRAPS, tumor necrosis factor receptor associated periodic syndrome) Chronic blistering cutaneous conditions have a prolonged course and present with vesicles and bullae. (wikipedia.org)
  • there may be causal and clinical distinctions between classic idiopathic PAN, the cutaneous forms of PAN, and the PAN associated with chronic hepatitis. (wikipedia.org)
  • WHIM syndrome, caused by a mutation in CXCR4, is associated with similar chronic cutaneous viral infections. (wikipedia.org)

Granulomatosis

  • The lung is frequently involved in primary vasculitis especially in Wegener's granulomatosis. (bmj.com)

pediatric

  • In the pediatric population, cutaneous PAN is frequently associated to streptococcal infections, and positive streptococcal serology is included in the diagnostic criteria. (wikipedia.org)

involvement

  • Some patients have cutaneous involvement alone. (bmj.com)

diagnostic

  • The diagnostic testing for vasculitis should be guided by the patient's history and physical exam. (wikipedia.org)
  • Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminative abilities on the prevalence of the various vasculitides in the analyzed populations. (wikipedia.org)

clinical

  • It is the most common vasculitis seen in clinical practice. (wikipedia.org)

nodules

  • We report a rare case of benign cutaneous PAN in a 14-year-old girl who presented with history of fever, subcutaneous nodules with cutaneous ulcer and digital gangrene. (biomedsearch.com)

manifestations

  • http://www.ncbi.nlm.nih.gov/pubmed/1089199?tool=bestpractice.com Some patients present with typical cutaneous manifestations but have delayed-onset, subclinical, or absent muscle disease. (bmj.com)

medium-sized

  • CSD also has a reasonably characteristic disease evolution, and histologic demonstration of the distinctive pattern of eosinophilic vasculitis of small and some medium-sized vessels results in an even more secure diagnosis. (medscape.com)

pathophysiology

  • Considering the wide range of potential causes leading to cutaneous small vessel vasculitis, there are subtle variations in the underlying pathophysiology for each cause. (wikipedia.org)

small

  • There are several vasculitides that affect small vessels. (wikipedia.org)

rash

  • About 3% of patients admitted to hospital have rashes due to adverse drug reactions but in others the cutaneous signs are due to the underlying or intercurrent illness (e.g. viral or bacterial exanthemas or internal disease), non-specific reactions to treatment (e.g. sweat- rash due to prolonged bed rest with plastic sheeting) or independent skin disease that has not been recognised by hospital staff. (dermnetnz.org)

refers

  • Leukocytoclastic" refers to the damage caused by nuclear debris from infiltrating neutrophils in and around the vessels. (wikipedia.org)
  • The term leukocytoclastic refers to the debris of neutrophils (immune cells) within the blood vessel walls. (aarda.org)

giant

  • Large vessel vasculitis   Giant cell arteritis Takayasu arteritis 2. (slideshare.net)

cerebral

autoimmune

  • Causes: vasculitis (esp PAN), diabetes or other autoimmune conditions (eg. (slideshare.net)

causes

  • Cutaneous vasculitis can have various causes including but not limited to medications, bacterial and viral infections or allergens. (wikipedia.org)

Patients

  • Overall Bottom Line: Suspect cutaneous (CL) and mucocutaneous (MCL) or mucosal (ML) leishmaniasis in patients with a history of a painless, non-healing skin, nasal, or oral lesion. (essentialevidenceplus.com)

distinguish

  • It is important to distinguish between IgA and non-IgA vasculitis. (wikipedia.org)

symptoms

  • Additional symptoms depend on the cause of the vasculitis and if other organ systems are involved. (wikipedia.org)

present

  • IgA vasculitis is more likely to present with abdominal pain, bloody urine, and joint pain. (wikipedia.org)

skin

  • The disease can be confined to the skin (cutaneous) or it can affect many different organs of the body such as the kidneys, central nervous system, heart, gastrointestinal tract, and lungs. (aarda.org)