Tijdschrift voor Geneeskunde vol:66 issue:13 pages:658-661
A 50-year-old woman with chronic urticaria, joint pain, episcleritis and dyspnea
The hypocomplementemic urticarial vasculitis syndrome (HUVS) was first described in 1973 and is also known as McDuffie’s syndrome.
HUVS is a rare disorder characterized by the presence of an urticarial vasculitis associated with arthralgias or arthritis and serum complement activation with marked decrease in C1q and other complement factors. A venulitis of the dermis (established via biopsy) with a deposition of C1q in the vessel wall is mandatory for the diagnosis.
HUVS is frequently associated with a modest glomerulonephritis, ocular inflammation, abdominal pain and chronic obstructive lung disease.
It may be treated with hydroxychloroquine and corticosteroids.