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Urticarial Vasculitis with Circulating Immune Complexes and Mixed Cryoglobulins: Studies of Pathogenesis
Author(s) -
Yasue Takashi,
Yasue Atsuko
Publication year - 1987
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1987.tb03634.x
Subject(s) - cryoglobulin , cryoglobulins , cryoglobulinemia , medicine , purpura (gastropod) , immunology , vasculitis , rheumatoid factor , immune complex , serum sickness , pathology , immune system , mixed connective tissue disease , immunoglobulin m , antibody , immunoglobulin g , biology , disease , ecology , hepatitis c virus , virus
A 64‐year‐old woman with urticarial vasculitis associated with mixed IgM‐IgG‐C cryoglobulinemia and circulating immune complexes developed skin lesions, which were urticaria‐like during the stages when cryoglobulinemia and hypocomplementemia were found but mimicked erythema multiforme and anaphylactoid purpura when there were no such findings. Typical leukocytoclastic vasculitis was observed in lesions of the latter type. Granular deposits of immunoglobulins (IgG, IgM) and complement components (Clq, C3) were found in the dermal‐epidermal junction, in the dermal blood vessel walls, and in the dermal connective tissue of the involved and uninvolved skin. IgM deposits predominated during the cryoglobulin‐positive and IgG deposits during the cryoglobulin‐negative stage of the disease. Perivascular neutrophilic infiltration and edema were observable in the uninvolved skin adjacent to the lesion. Electron microscopy revealed microtubular structures in the vascular endothelium of the involved skin. Systemic low or moderate doses of corticosteroids (prednisolone, <20 mg/day) were not very effective but dapsone or colchicine elicited an excellent response. Apparently, Clq precipitin, mixed IgM‐IgG cryoglobulins and IgG‐rheumatoid factor complexes are all capable of inducing urticarial vasculitis; these substances have biological activities similar to those of immune complexes.