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Hypocomplementemic urticarial vasculitis syndrome
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Hypocomplementemic urticarial vasculitis syndrome

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Contributors: Annie Yang MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Hypocomplementemic urticarial vasculitis syndrome : ANA positive, Hoarseness, Hypocomplementemia, Microscopic hematuria, Dyspnea, Chronic urticaria
Urticarial vasculitis represents a spectrum of diseases characterized by urticaria and histopathologic evidence of leukocytoclastic vasculitis, the descriptive term for cutaneous vasculitis. There may be localized disease with minimal to severe urticaria and minimal to systemic vasculitis, which when extensive, may be associated with hypocomplementemia. Hypocomplementemic urticarial vasculitis syndrome (HUVS), also known as McDuffie syndrome, is a rare immune complex-mediated small vessel vasculitis characterized by urticaria, hypocomplementemia (low C1q, C3, and C4), and systemic manifestations, and it is also associated with circulating anti-C1q autoantibodies. It can have an initial presentation of an underlying systemic disorder, or systemic manifestations may develop thereafter. Common systemic manifestations involve the pulmonary, visual, musculoskeletal, and renal organs and can present as angioedema, arthralgia, chronic obstructive pulmonary disease (COPD), glomerulonephritis, Sjögren's syndrome, uveitis, or cryoglobulinemia. The most common association has been with systemic lupus erythematosus (SLE), but COPD and uveitis are also associated.

HUVS predominantly affects women more than men, with a ratio of 8:1. It rarely affects children. Its peak incidence is in the fifth decade, with an average age of presentation of 48 years.

Related topic: Urticarial vasculitis (discusses normocomplementemic form)

Codes

ICD10CM:
L95.8 – Other vasculitis limited to the skin

SNOMEDCT:
239945009 – Hypocomplementemic urticarial vasculitis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Systemic lupus erythematosus (SLE) – SOAP BRAIN MD mnemonic (serositis, oral ulcers, arthritis, photosensitivity; blood disorders, renal involvement, ANA, immunologic phenomena [anti-Sm, anti-dsDNA], neurologic disorder; malar rash, discoid rash)
  • Mixed cryoglobulinemia
  • Muckle-Wells syndrome (see cryopyrin-associated periodic syndromes; CAPS) – deafness, recurrent hives, and amyloidosis with fever, chills, and arthralgias; a type of periodic fever syndrome
  • Cogan syndrome – a systemic vasculitis visual loss due to interstitial keratitis with vestibuloauditory symptoms of dizziness and hearing loss; slit lamp examination is needed for diagnosis; commonly diagnosed in patients aged 20-30 years
  • Schnitzler syndrome – splenomegaly and lymphadenopathy, intermittent fever, nonpruritic urticaria, and monoclonal IgG/IgM gammopathy
  • AHA syndrome – arthritis, hives, and angioedema symptoms exacerbated by emotional stress, anxiety, excessive alcohol consumption, and exercise
  • Sharp syndrome – also known as mixed connective tissue disease; ANA and anti-U1 RNP antibodies; overlaps with SLE, Sjögren's rheumatoid arthritis, scleroderma, and myositis
  • Urticarial vasculitis

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References

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Last Updated:11/25/2018
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Hypocomplementemic urticarial vasculitis syndrome
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A medical illustration showing key findings of Hypocomplementemic urticarial vasculitis syndrome : ANA positive, Hoarseness, Hypocomplementemia, Microscopic hematuria, Dyspnea, Chronic urticaria
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