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Article Abstract

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterized by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. Systemic involvement can either be present at the onset of disease or develop later. Here, we present a rare case of a 22-year-old female, who initially presented with generalized rash and was eventually diagnosed with HUVS. She responded well to dapsone. This article emphasizes the importance of a comprehensive review of systemic manifestations accompanying urticaria.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11871547PMC
http://dx.doi.org/10.7759/cureus.78227DOI Listing

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Background: Hypocomplementemic urticarial vasculitis (HUV) syndrome is a rare form of small-vessel vasculitis characterized by a heterogeneous spectrum of clinical and biological findings. It is typically marked by chronic urticarial eruptions, hypocomplementemia and histopathological evidence of leukocytoclastic vasculitis. It may also involve multiple organ systems, with frequent articular, gastrointestinal, renal, and other systemic manifestations.

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Background: Chronic urticarial lesions, a common condition of mostly unknown cause, can occur in immune dysregulation disorders such as hypocomplementemic urticarial vasculitis syndrome (HUVS), neutrophilic urticarial dermatosis (NUD), and systemic autoinflammatory diseases (SAIDs), including Schnitzler syndrome.

Objective: This study aimed to identify the molecular basis of non-pruritic chronic urticarial eruptions in four unrelated sporadic patients initially diagnosed with neonatal NUD, Schnitzler syndrome, HUVS or giant cell arteritis.

Methods: Conventional next-generation sequencing (NGS) of leukocyte DNA was supplemented with high-depth NGS (>1500X) to improve detection of low-level mosaicism in SAID-related genes.

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Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare, severe form of urticarial vasculitis. It is characterized by persistent hypocomplementemia, chronic urticarial vasculitic lesions, and severe multiorgan involvement. Herein, we present long-term follow-up of two siblings diagnosed with HUVS at early ages, who were found to have DNASE1L3 mutations, and their subsequent 20-year follow-up.

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Current therapeutic options for adult patients with urticarial vasculitis: A scoping review.

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Background: Urticarial vasculitis (UV) is a rare form of small vessel vasculitis, and there are limited published data on its management.

Objective: This study aims to review the current therapeutic options for UV.

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Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterized by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. Systemic involvement can either be present at the onset of disease or develop later. Here, we present a rare case of a 22-year-old female, who initially presented with generalized rash and was eventually diagnosed with HUVS.

View Article and Find Full Text PDF