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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. Hypocomplementemic urticarial vasculitis diagnosis was established after identifying anti-C1q antibodies. The lack of a dry syndrome, the negativity of a Schirmer test and the lack of sialadenitis on a salivary gland biopsy excluded an associated Gougerot-Sjögren Syndrome. The patient was treated with hydroxychloroquine and low-dose steroids, enabling a clinical and biological recovery. Of the 82 cases in the literature describing hypocomplementemic urticarial vasculitis associated nephropathies, 72 (88%) were a glomerular impairment, most frequently secondary to membranoproliferative glomerulonephritis. Only 6 (7%) tubulo-interstitial nephritis have been reported, 4 of them being associated with a glomerulonephritis. Patients were more likely to be women, aged in their third decade. The most frequent renal manifestations were haematuria (60%), and proteinuria (52%). Kidney failure was rarely observed (22%), with a fairly good renal prognosis. Hypocomplementemic urticarial vasculitis was associated with a systemic disease in 11 (13%) patients. In the absence of recommendations, the treatment strategy remains to be defined.
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http://dx.doi.org/10.1016/j.nephro.2019.09.004 | 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.
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