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  • Title: Dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency.
    Author: Holtman JH, Neustadt DH, Klein J, Callen JP.
    Journal: J Rheumatol; 1990 Sep; 17(9):1222-5. PubMed ID: 2290166.
    Abstract:
    The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. Patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of Sjögren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.
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