Urticarial Vasculitis
What is Urticarial Vasculitis ?
Urticarial vasculitis (UV) is a rare inflammatory disorder characterized by recurrent episodes of urticaria (hives) that persist longer than 24 hours and vasculitis (inflammation of small blood vessels) that may leave residual pigmentation or bruising in the skin. It commonly affects the skin around the hands. UV is classified into two subtypes based on complement (a type of inflammatory protein) levels in the blood:
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Normocomplementemic UV: Normal complement levels, typically limited to skin involvement; less likely to affect organs and therefore less severe.
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Hypocomplementemic UV syndrome (HUVS): Low complement levels (C1q, C3, C4), associated with systemic complications; affects joints, lungs, kidneys, GI, and eyes.
Synonyms
- Persistent urticaria with vasculitis
- Hypocomplementemic urticarial vasculitis syndrome
- Normocomplementemic urticarial vasculitis
- Hypocomplementemic urticarial vasculitis
- McDuffie syndrome
Urticarial vasculitis (UV) is a rare inflammatory disorder characterized by recurrent episodes of urticaria (hives) that persist longer than 24 hours and vasculitis (inflammation of small blood vessels) that may leave residual pigmentation or bruising in the skin. It commonly affects the skin around the hands. UV is classified into two subtypes based on complement (a type of inflammatory protein) levels in the blood:
-
Normocomplementemic UV: Normal complement levels, typically limited to skin involvement; less likely to affect organs and therefore less severe.
-
Hypocomplementemic UV syndrome (HUVS): Low complement levels (C1q, C3, C4), associated with systemic complications; affects joints, lungs, kidneys, GI, and eyes.
The exact prevalence of UV is unknown, but it accounts for approximately 5-10% of chronic urticaria cases, and predominantly affects women (2:1 female-to-male ratio) between 30-60 years of age. The hypocomplementemic form is rarer than the normocomplementemic form.
| Name | Abbreviation |
|---|---|
| Persistent urticaria with vasculitis | |
| Hypocomplementemic urticarial vasculitis syndrome | HUVS |
| Normocomplementemic urticarial vasculitis | NUV |
| Hypocomplementemic urticarial vasculitis | HUV |
| McDuffie syndrome |
The cause of UV is idiopathic (unknown) in most cases. UV is considered an autoimmune disorder where the body’s immune system mistakenly attacks its own blood vessels. It can be associated with certain autoimmune diseases (e.g., systemic lupus erythematosus, Sjögren’s disease, rheumatoid arthritis), off-target drug reactions (NSAIDS, antibiotics, ACE inhibitors, fluoxetine), malignancies and genetic factors (familial HUVS linked to DNASE1L3 gene mutations).
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Skin Lesions:
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Urticaria (hives) lasting >24 hours
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Painful or burning sensation (not just itchy)
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Lesions may heal with hyperpigmentation or purpura (red or purple spots)
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Systemic symptoms (especially in HUV/HUVS):
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Fever
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Arthralgia or arthritis
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Abdominal pain
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Kidney involvement (e.g., blood or protein in urine)
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Lung symptoms (e.g., cough, shortness of breath)
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Eye inflammation - bloodshot or inflamed appearance
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Fatigue is common.
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Physical examination: painful itchy hives persisting for >24 hours with residual skin coloration changes.
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Skin biopsy showing blood vessel inflammation.
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Laboratory tests: Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) tests for inflammation; complement levels (C3 and C4 low in HUV); presence of auto-reactive antibodies (anti-nuclear antibody, anti-C1q) in the blood; urinalysis, liver function tests.
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Imaging for pulmonary complications.
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Mild cases:
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Antihistamines
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NSAIDs (with caution)
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Moderate to severe cases:
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Corticosteroids – mainstay for acute flares
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Immunosuppressants:
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Hydroxychloroquine
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Dapsone
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Colchicine
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Methotrexate or azathioprine
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Biologics (e.g., rituximab, omalizumab)
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Treat underlying cause, if identified.
The prognosis varies depending on severity, whether systemic involvement is present, and the response to treatment. Many individuals manage the condition with treatment, but it can become chronic.
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Normocomplementemic UV: Generally good prognosis with mainly skin involvement, may resolve within months to years.
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Hypocomplementemic UV: Can be chronic and systemic, with potential for organ damage especially in the kidneys or lungs.
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Jachiet M, et al. 2015. “The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients.” Arthritis Rheumatol. 67(2):527-34. doi: 10.1002/art.38956. PMID: 25385679.
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Rothermel ND, et al. 2024. “Managing Urticarial Vasculitis: A Clinical Decision-Making Algorithm Based on Expert Consensus.” Am J Clin Dermatol. 26(1):61-75. doi: 10.1007/s40257-024-00902-y. Epub 2024 Nov 13. PMID: 39535577; PMCID: PMC11748462.
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Orphanet: Hypocomplementemic urticarial vasculitis.
Last updated 9 Jul 2009, 09:02 PM
9 Jul 2009, 09:02 PM
hello. i do not have this illness, but http://www.inspire.com/groups/rare-disease/journal/huvs-hypocomplementemic-urticarial-vasculitis/ they do. also this had a lot of great information http://emedicine.medscape.com/article/1085087-overview let me know if i can help
6 Jul 2009, 05:13 PM
I have been suffering from this illness for nearly 8 months now and i am desprate to fing othr people that have ths ilness.
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Trying to find other people with this illness
Created by bettyboop31 | Last updated 9 Jul 2009, 09:02 PM
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