Hypocomplementemic urticarial vasculitis

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Urticarial vasculitis is a rare clinicopathologic entity that is characterized by chronic or recurrent episodes of urticarial lesions. Skin findings of this disease can be difficult to distinguish visually from those of chronic idiopathic urticaria but are unique in that individual lesions persist for ≥24 hours and can leave behind dusky hyperpigmentation. This disease is most often idiopathic but has been linked to certain drugs, infections, autoimmune connective disease, myelodysplastic disorders, and malignancies...

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterised by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. The authors present the case of a 70-year-old man who presented to the ophthalmology clinic with bilateral scleritis and ocular hypertension. He was diagnosed with HUVS after a 6-month period of bilateral scleritis, vestibulitis, significant weight loss, mononeuritis multiplex and recurrent urticarial vasculitis with pronounced persistent hypocomplementemia and the presence of anti-C1q antibodies...

Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments ("leukocytoclasia"). Several medications can cause LCV, as well as infections, or malignancy...

BACKGROUND: Chronic spontaneous urticaria (CSU) may occasionally exhibit long-lasting lesions with bruising, usually considered a hallmark of urticarial vasculitis (UV). Histopathology of these chronic urticarial lesions has not been extensively studied. METHODS: Skin biopsies from patients with anti-H1 resistant CSU were evaluated for several parameters (edema, location, intensity, and cell composition of the inflammatory infiltrate, and abnormalities in the blood vessels)...

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No abstract text is available yet for this article.

Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histologically show changes of leukocytoklastic vasculitis. In association with connective tissue disease it is most commonly seen complicating Systemic lupus erythematous (SLE) and, less often, Sjogren's syndrome. Here, we report a 25-year-old woman who developed SLE in 1998. In May 2013 she presented with urticarial vasculitis; her skin biopsy was consistent with leukocytoclastic vasculitis. She also developed bilateral uveitis...

BACKGROUND: Hypocomplementemic urticarial vasculitis syndrome is an infrequent condition characterized by ocular, renal, gastrointestinal and pulmonary involvement with low serum complement levels and autoantibodies. Renal manifestations vary from microscopic hematuria to nephrotic syndrome and acute kidney injury. Accordingly differing histologic patterns have been reported. CASE PRESENTATION: We present the case of a 65 years old woman with a history of chronic uveitis who presented with arthralgias, urticarial rush, nephrotic syndrome, glomerular hematuria and low serum complement...

Hypocomplementemic urticarial vasculitis syndrome (HUVS), or McDuffie syndrome, is a rare small vessel vasculitis associated with urticaria, hypocomplementemia and positivity of anti-C1q antibodies. In rare cases, HUVS can manifest as an immune-complex mediated glomerulonephritis with a membranoproliferative pattern of injury. Due to the rarity of this disorder, little is known about the clinical manifestation, pathogenesis, treatment response and outcome of such patients. We describe here three cases of HUVS with severe renal involvement...

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No abstract text is available yet for this article.

WHAT IS KNOWN AND OBJECTIVE: Tumour necrosis factor-α-blocking agents potentially cause vasculitis. However, no study has reported on the association between hypocomplementemic urticarial vasculitis (HUV) and certolizumab pegol (CZP) usage. CASE DESCRIPTION: We present the first case of HUV development during CZP treatment for rheumatoid arthritis. Hypocomplementemic urticarial vasculitis improved after CZP was discontinued and the dose of oral prednisolone was increased...

Hypocomplementemic urticarial vasculitis is a rare systemic vasculitis, affecting small vessels, characterised by chronicle urticaria, hypocomplementemia, and systemic manifestations. Renal involvement, whose prevalence varies between 9% and 60%, is mainly glomerular. We here report the case of a 59 years old woman presenting kidney failure, associated with chronicle urticaria and arthralgias. Laboratory investigation showed haematuria, proteinuria, hypocomplementemia and anti-SSa antibody positivity. A percutaneous kidney biopsy revealed focal and segmental glomerulonephritis associated with an acute interstitial nephritis...

Although more than 45 years have passed since hypocomplementemic urticarial vasculitis (HUVS) was first described by McDuffie and colleagues at the Mayo clinic, data on epidemiology, disease outcomes, prognosis and clinical features are scarce. Recently, we published the first epidemiological study of HUVS including data on incidence, prevalence, disease outcomes, prognosis and clinical features using data from two separate Swedish regions during a period of 16 years. The estimation of incidence and prevalence rates indicates that HUVS is rare but not always benign...

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a small vessel vasculitis characterized by hypocomplementemia and urticaria-like exanthema. Some cases also display abdominal pain and membranoproliferative glomerulonephritis (MPGN) with immune complex deposits. We treated a case of HUVS with biopsy-proven gastrointestinal vasculitis and atypical histological findings in a kidney biopsy. The 36-year-old Japanese man, who was previously diagnosed with diffuse panbronchiolitis, visited our hospital due to transient urticaria-like exanthema and rapid deterioration of kidney function...

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare type III hypersensitivity disorder characterized by urticarial vasculitis and prolonged hypocomplementemia. Individuals with HUVS may also have joint involvement, pulmonary manifestations, ocular disease, kidney inflammation, or any other form of organ involvement. Hypocomplementemia, the presence of C1q antibody in the serum, and urticarial vasculitis are the keys to the diagnosis of HUVS. It has been reported to accompany certain infections such as hepatitis B, hepatitis C, infectious mononucleosis, and coxsackie group A...

BACKGROUND: Multiple Sclerosis (MS) has been associated with several immune-mediated diseases but the mechanisms that explain such associations, as well as their implications in clinical practice and treatment are rarely discussed. CASE PRESENTATION: We report the case of a patient with a history of MS since she was 27 years old, followed by a diagnosis of Hypocomplementemic Urticarial Vasculitis (HUV) seven years later. Several disease-modifying treatments for MS and HUV were used but with limited benefit in both diseases and significant MS progression...

Anti-C1q autoantibodies may be found in many conditions, most commonly in systemic lupus erythematosus (SLE) and hypocomplementemic urticarial vasculitis syndrome (HUVS), and are diagnostic markers as well as disease activity markers in lupus nephritis. Sera from patients with SLE and HUVS show partly distinct autoantibody reactivities to separated protein chains B and C of the first component of complement, C1q. These different binding specificities can be detected by Western blot analysis of the autoantibodies under reducing conditions...

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