Cutaneous Leukocytoclastic Vasculitis With Hyperglobulinemia and Splenomegaly

Mycophenolate mofetil treatment of a patient with hyperglobulinemic purpura

“…[1][2][3][4][5]10 Associated symptoms on the affected areas such as burning, stinging, pruritus, and edema have been commonly reported. [2][3][4][5]8,11 Less commonly, other symptoms such as fever, night sweats, generalized lymphadenopathy, malaise, weight loss, myalgias, and arthralgias have been seen.…”

“…It is thought that HGPWrepresents an autoimmune process because of its overrepresentation in women, the presence of autoantibodies, and its association with other autoimmune diseases, such as Sjögren's syndrome or systemic lupus erythematosus. [2][3][4][5][6][7][8]10 In support of an autoimmune process, several individual cases and studies have demonstrated the presence of immunoglobulin within blood vessel walls and circulating immune complexes as the most likely cause of HGPW. 8,[10][11][12]18,19 MMF is an immunosuppressive agent with a mechanism of action that has been well described.…”

“…[2][3][4][5][6][7][8]10 In support of an autoimmune process, several individual cases and studies have demonstrated the presence of immunoglobulin within blood vessel walls and circulating immune complexes as the most likely cause of HGPW. 8,[10][11][12]18,19 MMF is an immunosuppressive agent with a mechanism of action that has been well described. [13][14][15][16] It has been used to treat a variety of cutaneous inflammatory/autoimmune conditions (Table I) including autoimmune blistering disorders and cutaneous collagen vascular diseases.…”