Hypocomplementemic urticarial vasculitis

Common Name(s)

Hypocomplementemic urticarial vasculitis

Hypocomplementemic urticarial vasculitis (HUV) is a rare form of cutaneous small-vessel vasculitis characterized by recurrent episodes of urticaria and painful, tender, burning or itchy skin lesions, often associated with extracutaneous involvement but usually with no significant peripheral nerve damage. Patients with this condition are likely to have systemic involvement, including angioedema, arthralgias, pulmonary disease, abdominal or chest pain, fever, renal disease, and episcleritis.  Hypocomplementemic urticarial vasculitis is thought be an autoimmune response involving a specific region of complement 1 (C1).  It can present as or precede a syndrome that includes obstructive pulmonary disease , uveitis, systemic lupus erythematous (SLE), Sjšgren's syndrome, or cryoglobulinemia (which is closely linked with hepatitis B or hepatitis C virus infection). Some cases of hypocomplementemic urticarial vasculitis respond to therapies commonly used for the treatment of SLE, including low-dose prednisone, hydroxychloroquine, dapsone, or other immunomodulatory agents.  
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hypocomplementemic urticarial vasculitis" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hypocomplementemic urticarial vasculitis" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Hypocomplementemic urticarial vasculitis" returned 14 free, full-text research articles on human participants. First 3 results:

[Severe pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV)].
 

Author(s): Mohammed Raoufi, Mustapha Laine, Hicham Naji Amrani, Hicham Souhi, Hicham Janah, Hanane Elouazzani, Ismail Abderrahmane Rhorfi, Ahmed Abid

Journal:

 

Pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV) or Mac Duffie syndrome is extremely rare with a poor prognosis. We report the case of a 55-year-old female patient treated for HUV over a period of 20 years. The diagnosis was confirmed on the basis of urticarial ...

Last Updated: 31 Dec 1969

Go To URL
Systemic Manifestations of Hypocomplementemic Urticarial Vasculitis: Comment on the Article by Jachiet et al.
 

Author(s): Barbara Hauser

Journal: Arthritis Rheumatol. 2015 Jul;67(7):1984-5.

 

Last Updated: 31 Dec 1969

Go To URL
The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients.
 

Author(s): Marie Jachiet, Béatrice Flageul, Alban Deroux, Alain Le Quellec, François Maurier, Florence Cordoliani, Pascal Godmer, Claire Abasq, Leonardo Astudillo, Pauline Belenotti, Didier Bessis, Adrien Bigot, Marie-Sylvie Doutre, Mikaël Ebbo, Isabelle Guichard, Eric Hachulla, Emmanuel Héron, Géraldine Jeudy, Noémie Jourde-Chiche, Denis Jullien, Christian Lavigne, Laurent Machet, Marie-Alice Macher, Clotilde Martel, Sara Melboucy-Belkhir, Cécile Morice, Antoine Petit, Bernard Simorre, Thierry Zenone, Laurence Bouillet, Martine Bagot, Véronique Frémeaux-Bacchi, Loïc Guillevin, Luc Mouthon, Nicolas Dupin, Selim Aractingi, Benjamin Terrier,

Journal: Arthritis Rheumatol. 2015 Feb;67(2):527-34.

 

Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV.

Last Updated: 31 Dec 1969

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Hypocomplementemic urticarial vasculitis" returned 1 free, full-text review articles on human participants. First 3 results:

Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge.
 

Author(s): Wolfgang Grotz, Hideo A Baba, Jan U Becker, Martin W Baumgärtel

Journal: Dtsch Arztebl Int. 2009 Nov;106(46):756-63.

 

Chronic urticaria often points the way to the diagnosis of a systemic disease, particularly when urticarial vasculitis can be demonstrated. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is considered to be an independent immunological disease.

Last Updated: 31 Dec 1969

Go To URL
 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

Journey of Patients With Vasculitis From First Symptom to Diagnosis
 

Status: Recruiting

Condition Summary: Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatous Vasculitis; Temporal Arteritis; Giant Cell Arteritis; Granulomatosis With Polyangiitis; Wegener Granulomatosis; Henoch Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Urticarial Vasculitis

 

Last Updated: 18 Jan 2018

Go to URL
Clinical Transcriptomics in Systemic Vasculitis (CUTIS)
 

Status: Recruiting

Condition Summary: Cryoglobulinemic Vasculitis (CV); Drug-induced Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); IgA Vasculitis; Isolated Cutaneous Vasculitis; Granulomatosis With Polyangiitis (GPA); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Urticarial Vasculitis; Vasculitis

 

Last Updated: 10 Apr 2018

Go to URL
Vasculitis Pregnancy Registry
 

Status: Recruiting

Condition Summary: Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

 

Last Updated: 27 Apr 2017

Go to URL