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Document Details :
Title: Een 50-jarige patiënte met chronische urticaria, gewrichtspijn, oogontstekingen en dyspneu
Author(s): LAMBELIN M, HAUBEN E, BLOCKMANS D
Journal: Tijdschrift voor Geneeskunde
Volume: 66 Issue: 13 Date: 2010
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.