Background The mainstay of therapy of autoimmune mucocutaneous blistering diseases has been prolonged high-dose systemic corticosteroids and immunosuppressive agents. Recently, high-dose intravenous ...immunoglobulin (IVIg) has been employed in selected cases, with excellent results in most of them. Objective We sought to evaluate the outcome of the use of IVIg in patients with autoimmune mucocutaneous blistering diseases refractory to conventional therapy or with contraindications for it. Methods We performed a retrospective analysis of clinical response to monthly cycles of IVIg in 19 patients affected with autoimmune mucocutaneous blistering diseases: 10 patients with pemphigus vulgaris (PV), 2 with pemphigus foliaceus (PF), 4 with mucous membrane pemphigoid (MMP), 2 with epidermolysis bullosa acquisita, and one with linear IgA bullous dermatosis. Results Four (21%) of 19 cases presented a complete response (2 PV, 1 MMP and 1 epidermolysis bullosa acquisita). Five (26%) patients did not respond to the treatment (3 PV, 1 PF, 1 MMP). Ten patients (53%) had a partial response. Limitations This was a retrospective noncontrolled study with a heterogeneous group of patients. Conclusion The effectiveness of IVIg was inferior to that previously reported. This difference could be attributed to the preparations employed, the different severity of the disease, or individual responses in each patient dependent on Fc receptor gamma polymorphisms.
Autoimmune blistering diseases (AIBD) are relatively uncommon all over the world. Therefore most physicians and even dermatologists are generally unfamiliar with their diagnosis and treatment. In ...Spain there are very few dermatologists and dermatology clinics specialized in AIBD. This article provides an overview of the management of AIBD in Spain at the present time.
Mucous membrane pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of mucous membranes, skin, or ...both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for mucous membrane pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index.
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