Mucosal disease series. Number III. Mucous membrane pemphigoid.
Mucous membrane pemphigoid (MMP) is a sub-epithelial vesiculobullous disorder. It is now quite evident that a number of sub-epithelial vesiculobullous disorders may produce similar clinical pictures, and also that a range of variants of MMP exist, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated sub-epithelial blistering diseases (IMSEBD) has therefore been used. Immunological differences may account for the significant differences in their clinical presentation and responses to therapy, but unfortunately data on this are few. The diagnosis and management of IMSEBD on clinical grounds alone is impossible and a full history, general, and oral examination, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. No single treatment regimen reliably controls all these disorders, and it is not known if the specific subsets of MMP will respond to different drugs. Currently, apart from improving oral hygiene, immunomodulatory-especially immunosuppressive-therapy is typically used to control oral lesions. The present paper reviews pemphigoid, describing the present understanding of this fascinating clinical phenotype, summarising the increasing number of subsets with sometimes-different natural histories and immunological features, and outlining current clinical practice.
University of Valencia, Spain.
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J
Bagan
L
L
Lo Muzio
L L
LL
Muzio
C
C
Scully
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000145:classification / Q000188:drug therapy / Q000276:immunology; D001768:Blister; D004847:Epithelial Cells / Q000276:immunology; D005882:Gingival Diseases / Q000188:drug therapy / Q000276:immunology*; D022002:Hemidesmosomes / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010390:Pemphigoid, Benign Mucous Membrane / Q000145:classification / Q000188:drug therapy / Q000276:immunology
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D007166:Immunosuppressive Agents