DESCRIPTION
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Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)
is a rare type of chronic autoimmune inflammation of small blood vessels and abnormally
low levels of complement. HUVS is often associated with systemic diseases such as
COPD, systemic lupus, and Sjögren syndrome.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Laboratory tests of serum documenting complement deficiency and positive C1q antibody,
and skin or organ biopsy documenting leukocytoclastic vasculitis (LCV), also known
as hypersensitivity vasculitis. The clinical criteria for diagnosis of HUVS include
characteristic chronic urticaria with residual hyperpigmentation, and inflammatory
vasculitis with angioedema, glomerulonephritis, chronic obstructive pulmonary disease,
arthritis, or uveitis.
Physical findings: HUVS usually involves the skin with painful urticaria (hives), and may cause inflammatory
changes in joints, kidneys, gastrointestinal tract, lungs, heart, and eyes.
ICD-9:
279.4
ICD-10: M35.9
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Suggested MER for Evaluation:
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Claimant’s medical source(s) records of clinical history and physical findings;
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Skin and/or tissue biopsy report;
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Laboratory reports showing abnormal complement levels and C1q antibody; or
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Evidence of organ dysfunction, especially eye, renal, cardiac, and respiratory systems
(for example, pulmonary function tests).
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