Department of Dermatology, University of Münster, Von-Es-march-Street 58, D-48149 Münster, Germany.
Intravenous immunoglobulins (IVIgs) are derived from a purified human plasma pool of healthy blood donors and contain IgG as well as traces of other immunoglobulins or aggregates. They exert a variety of immunomodulating activities and are currently used for the treatment of primary and secondary immunodeficiency diseases, autoimmune disorders, and certain infectious diseases (Rutter and Luger, 2002; Shoenfeld and Krause, 2004; Wetter et al., 2005). The complex mechanisms of action of IVIgs are still not fully understood. There is evidence of a suppression in autoantibody production, neutralization of complement-mediated effects, blocking of Fc receptors on macrophages and downregulation of costimula-tory molecules. Moreover, IVIg preparations contain amounts of soluble CD4, CD8, and MHC-I and -II molecules, which may have the ability to inhibit autoreactive T lymphocytes. IVIgs also contain anti-Fas-receptor antibodies, which are able to block molecular Fas ligand/Fas receptor interactions and consequently cause keratinocyte apoptosis. This seems to play a vital role in the therapy of toxic epidermal necrolysis with IVIgs. IVIgs were also found to increase glucocorticoid receptor sensitivity and thereby in combination with glucocorticoids, they synergistically suppress lymphocyte activation (Viard et al., 1998; Spahn et al., 1999; Rütter and Luger, 2002; Ibanez and Montoro-Ronsano, 2003).
There is evidence from many case reports and clinical trials for the high efficacy of IVIg as an adjunctive treatment of otherwise refractory autoimmune diseases with skin involvement. Accordingly, IVIgs have been used successfully for the treatment of dermatomyositis, scleroderma, systemic lupus erythematosus (SLE), pemphigus vulgaris and pemphigus foliaceus, bullous pemphigoid, cicatrical pemphigoid, epidermoly-sis bullosa aquisita, pemphigoid gestationis, linear IgA bullous dermatoses, and toxic epidermal necrolysis. Other skin diseases with some evidence for a favourable response to IVIg are chronic autoimmune urticaria, atopic dermatitis, graft-versus-host disease, psoriasis, pyoderma gangrenosum, lichen ruber, livedoid vasculitis, scleromyxedema, pretibial myxedema, and acquired von Willebrand disease. Further potential advances in the treatment with IVIg require careful patient selection and appropriately designed placebo-controlled, randomized studies (Rütter and
Luger, 2002; Emmi and Chiarini, 2002; Wetter et al., 2005).
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