Clinical Characteristics and Treatment Outcomes in Patients with Urticarial Vasculitis

Abstract

Clinical characteristics and treatment outcomes in patients with urticarial vasculitis Orıgınal Article DERMATOLOGY Cite this article as: Serarslan G, Okyay E. Clinical characteristics and treatment outcomes in patients with urticarial vasculitis. North Clin Istanb UNCORRECTED PROOF U rticarial vasculitis (UV) is a form of cutaneous leukocytoclastic vasculitis with urticarial plaques last longer than 24 hours and with inflammatory damage to dermal capillaries and postcapillary venules in the histopathology [1, 2]. UV is reported to be rare and there are no population-based epidemiological studies describing its incidence and prevalence. The disease may be mild or manifest with severe systemic and specific organ findings. UV is often idiopathic however infection, drug reaction, autoimmune reactions and malignancy may be among the underlying etiologies [2]. UV is classified as normocomplementemic UV (NUV) and hypocomplementemic UV (HUV). HUV is thought to be driven by deposition of immune complexes in the skin. This activates the complement (C) cascade, followed by a reduction in complement levels. Low C levels indicate the more severe form of the disease. Therefore, when hypocomplementemia is detected, more organ involvement is likely to occur [2].

Clinical profile of pathological urticarial vasculitis: A retrospective study

“…Additionally, 17% of them had episodes of dyspnoea, as described classically in UV but also in some patients with CU 24,30 . Pruritus, classically found in CU and known to be less present or absent in UV, was also found in all patients from the HUV group and in two‐third of patients from the NUV group, consistent with some studies that have described pruritus in UV with a range of 31%–100% 3,14,25,27,28,31,32 …”

“…Omalizumab, used classically in AH‐resistant CU, 33 was prescribed for one NUV patient and one patient with “undetermined diagnosis” with a complete remission. As in our observations, omalizumab has been reported as effective in NUV 32,40–45 …”

“…24,30 Pruritus, classically found in CU and known to be less present or absent in UV, was also found in all patients from the HUV group and in two-third of patients from the NUV group, consistent with some studies that have described pruritus in UV with a range of 31%-100%. 3,14,25,27,28,31,32 Skin biopsy is not recommended in the case of classical urticarial lesion. However, an atypical dermatological presentation of urticaria may lead the clinician to the realisation of a skin biopsy, such as: fixed urticarial lesions, vasoconstriction halo, livedo, purpura or residual pigmentation.…”

“…As in our observations, omalizumab has been reported as effective in NUV. 32,[40][41][42][43][44][45]…”

Breaking Out of the Hive: A Case Report on Hypocomplementemic Urticarial Vasculitis Syndrome

“…For patients who exhibit partial response or require high doses of steroids, steroid-sparing agents such as mycophenolate mofetil (CellCept) or azathioprine may be employed. Additionally, rituximab, a monoclonal antibody against CD20 on B-cells, has been shown to be effective in cases resistant to traditional therapies [ 14 ]. Rituximab's role in B-cell depletion helps in reducing autoantibody production, which is crucial in autoimmune diseases such as HUVS [ 2 ].…”