Abstract
Urticarial vasculitis is an eruption of erythematous wheals that clinically
resemble urticaria but histologically show changes of leukocytoklastic
vasculitis. In association with connective tissue disease it is most commonly
seen complicating Systemic lupus erythematous (SLE) and, less often, Sjogren’s
syndrome. Here, we report a 25-year-old woman who developed SLE in 1998. In May
2013 she presented with urticarial vasculitis; her skin biopsy was consistent
with leukocytoclastic vasculitis. She also developed bilateral uveitis. She had
most of the clinical and laboratory characteristics of hypocomplementic
urticarial vasculitis syndrome (HUVS) which is difficult to be differentiated
from SLE. She was treated with high-dose prednisone, Mycophenolate Mofetil
(MMF), colchicine, and Dapsone but failed. We decided to give her Rituximab
(RTX), her urticarial vasculitis and uveitis symptoms improved significantly.
Unfortunately, later on she presented with severe discoid lupus. We started her
on thalidomide and responded well. Our case highlights that Rituximab is a good
option for severe refractory urticarial vasculitis and thalidomide is effective
in treatment of discoid lupus erythematosus (DLE), and can be used safely in
specialist rheumatological practice.