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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. She responded well to dapsone. This article emphasizes the importance of a comprehensive review of systemic manifestations accompanying urticaria.
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http://dx.doi.org/10.7759/cureus.78227 | DOI Listing |
Front Immunol
August 2025
Clinical Immunology Department, Pasteur Institute of Tunis, Tunis, Tunisia.
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.
View Article and Find Full Text PDFBr J Dermatol
June 2025
Sorbonne Université, INSERM UMR_S933, Maladies génétiques d'expression pédiatrique, Hôpital Trousseau, Paris, France.
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.
Clin Rheumatol
July 2025
Division of Pediatric Rheumatology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey.
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.
View Article and Find Full Text PDFJ Am Acad Dermatol
August 2025
Vasculitis Clinic, Department of Medicine, Sacre-Coeur Hospital, Université de Montréal, Montréal, Quebec, Canada.
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.
Methods: A PubMed search was conducted, selecting articles published from 2000 to January 2024.
Cureus
January 2025
Internal Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND.
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