Urticarialvasculitis. Urticarialvasculitis is a rare condition manifesting with a variety of clinical presentations ranging from skin limited lesions to life-threatening systemic illnesses. This review aims to highlight the recent findings on the etiology, diagnostic modalities, and therapeutic strategies and course of urticarialvasculitis. In addition to well established triggers, urticarialvasculitis (UV) cases associated with severe acute respiratory syndrome coronavirus 2 (SARS-Cov2) disease and COVID-19 vaccines, vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome, and adenosine deaminase (ADA) deficiency have been reported. A clinical-dermoscopic model for differentiating urticarialvasculitis has been developed with purpuric patches and globules favoring UV
Managing UrticarialVasculitis: A Clinical Decision-Making Algorithm Based on Expert Consensus. Urticarialvasculitis (UV) is a rare and difficult-to-treat, small-vessel leukocytoclastic vasculitis presenting with recurrent long-lasting wheals. So far, no guidelines and treatment algorithms exist that could help clinicians with the management of UV. In this review, we describe evidence on systemic treatments used for UV and propose a clinical decision-making algorithm for UV management based on the UrticarialVasculitis Activity Score assessed for 7 days (UVAS7). Patients with occasional UV-like urticarial lesions and patients with UV with skin-limited manifestations and/or mild arthralgia/malaise (total UVAS7 ≤7 of 70) can be initially treated using the step-wise algorithm for chronic
Successful treatment of urticarialvasculitis with omalizumab in children: a case series. Urticarialvasculitis (UV) is a small vessel leukocytoclastic vasculitis, which often needs to be distinguished from urticaria and other dermatoses. Treatment of UV in children is challenging because of the unsatisfying efficacy of antihistamines and the safety concern of long-term systemic corticosteroids
Urticarialvasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosis. Urticarialvasculitis (UV) is a rare cutaneous vasculitis of small vessels characterized by recurrent episodes of wheal-like lesions that tend to last more than 24 hours, healing with a residual ecchymotic postinflammatory hyperpigmentation. The histopathologic pattern of UV
A case report of hypocomplementemic urticarialvasculitis presenting with membranoproliferative glomerulonephritis. Hypocomplementemic urticarialvasculitis 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 with steroids, cyclophosphamide and hydroxychloroquine followed by rapid clinical improvement and remission of proteinuria. Maintenance treatment consisted of rituximab pulses. The majority of hypocomplementemic urticarialvasculitis syndrome cases is idiopathic, although an association to drugs, infections or other autoimmune disorders has been recorded. Given the rarity and heterogeneity of the disease
Eosinophilic infiltration: an under-reported histological finding in urticarialvasculitis. Urticarialvasculitis (UV) is a clinicopathologic entity in which skin lesions resemble urticaria clinically but last more than 24 hours and resolve with residual purpura. In this study, we evaluated distinguishing histological features of UV from common urticaria to differentiate between these two
Patient-reported outcomes in urticarialvasculitis treated with omalizumab: case report. Despite the current knowledge of UV, there is a lack of consensus among diagnostic criteria and management. In general, antihistamine therapy is regularly used for the symptomatic management of pruritus but does not control inflammation or alter the course of the disease. Monoclonal antibodies such as omalizumab (anti-IgE) have been proposed as a potential treatment for urticarialvasculitis. A few studies have reported the benefits of omalizumab in patient-reported outcome measures (PROMs). Herein we describe a female patient with urticarialvasculitis who was treated with omalizumab. We discuss the response to treatment and possible implications of PROMs in guiding the management of the disease. We
Normocomplementaemic urticarialvasculitis: effective treatment with omalizumab We report two patients with normocomplementaemic urticarialvasculitis with impressive response to omalizumab. This contrasts recent reports on hypocomplementaemic urticarialvasculitis syndrome, highlighting the need for clinical trials of omalizumab in normocomplementaemic urticarialvasculitis.
Effectiveness of omalizumab in a case of urticarialvasculitis. Urticarialvasculitis (UV) is a mainly leucocytoclastic vasculitis with urticarial plaques. Treating these patients is challenging as the available treatments have poor efficacy. Oral corticosteroids are considered the first-line treatment, but H1 antihistamines, dapsone, colchicine, antimalarials, ciclosporin and antileucotrienes have all been tried also. However, because of their adverse effects and/or lack of efficacy, new agents are still needed. Omalizumab, an anti-IgE antibody, shows efficacy in chronic spontaneous urticaria, and might also be a good treatment for angio-oedema and urticarialvasculitis. To our knowledge, there have been only seven relevant case reports published in the English literature. We add a new
A Case of Crescentic Glomerulonephritis Complicated with Hypocomplementemic UrticarialVasculitis Syndrome and ANCA-Associated Vasculitis Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction and subendothelial deposits with circumferential mesangial interposition. She fulfilled the diagnostic criteria for hypocomplementemic urticarialvasculitis syndrome (HUV), and ANCA-associated vasculitis (AAV) was also indicated by small vessel vasculitis and positive MPO-ANCA. Steroid pulse therapy with methylprednisolone followed by oral prednisolone improved her general condition and hypocomplementemia, and MPO
Hypocomplementemic UrticarialVasculitis Syndrome with Membranous Nephropathy: Case Report Urticarialvasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes . Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarialvasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarialvasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards
Normocomplementaemic UrticarialVasculitis in a 19-Month-Old Girl Urticaria is common in children. Urticarialvasculitis (UV) is a potentially more serious, rare variant. The youngest reported case was 12 months of age. A systemically well, 19-month-old girl presented with her mother who was concerned about the development of a rash. On presentation, the child had normal vital signs, was alert
UrticarialVasculitis-Associated Intestinal Ischemia Urticarialvasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarialvasculitis-associated intestinal ischemia.
Urticarialvasculitis induced by OTC diet pills: a case report UrticarialVasculitis (UV) is in most of the cases idiopathic; however it has been associated with several conditions and drugs. Over the counter (OTC) diet pills are widely available, even on-line, but they are rarely regulated by pharmaceutical control. We present the case of a 35-year-old female patient suffering of pruriginous
Hypocomplementemic urticarialvasculitis arising in a patient with immunoglobulin G4-related disease. Hypocomplementemic urticarialvasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease virus infection was detected, although the structure had been destroyed by the infiltration of lymphocytes and plasma cells. Among total IgG-producing cells, approximately 40% were positive for IgG4. All symptoms and abnormal laboratory findings improved in response to oral prednisolone. Hypocomplementemic urticarialvasculitis may represent a clinical symptom of IgG4-related disease.
A 14-year paraneoplastic rash: urticarialvasculitis and dermal binding bullous pemphigoid secondary to chronic lymphocytic leukaemia. A 72-year-old woman with a 14-year history of urticarialvasculitis (UV) and a 13-year history of bullous pemphigoid (BP) presented with associated progressive chronic lymphocytic leukaemia (CLL). Both skin conditions responded poorly to treatment, until