Number III Mucous membrane pemphigoid

Abstract

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.

Adolescent Oral Pemphigoid: Report of A Case and Review of Literature

“…2,13,14 The mechanism is further complicated by the inclusion of molecules, such as RANTES, interleukins, tumor necrosis factor alpha (TNF-α), TNF-β, interferon gamma (IFN-γ) and more recently identified molecules, such as eotaxin (Verdolini and Cerio 2003). 1 The collagen produced may lead to cicatrization of the eye or mucous membranes. This process is of particular importance in MMP affecting the eyes, where fibrosis or subsequent cicatrization can cause profound tear insufficiency, symblepharon formation, trichiasis, keratinization of the cornea and several other defects.…”

“…1 They are often collectively referred to as subepithelial bullous dermatoses (SEBDs). They include bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA disease (LAD), chronic bullous dermatosis of childhood (CBDC) and epidermolysis bullosa acquisista (EBA).…”

“…Also obtaining a diagnostic gingival biopsy can be technically challenging and result in a periodontal defect (Siegel and Anhalt 1993). 1 The MMP is histologically characterized by junctional separation at the level of the basement membrane giving rise to a subbasilar split as in other forms of pemphigoid. Classical histopathological features include a subepithelial split with a chronic inflammatory infiltrate containing eosinophils, lymphocytes and neutrophils as well in the lamina propria.…”

“…The desquamation may vary from mild almost insignificant small patches to widespread erythema with a glazed appearance. 1 A differential diagnosis of MMP may include pemphigus vulgaris, and bullous SLE (Scott and Ahmed 1998) as well as pemphigoid subtypes and other immune-mediated subepithelial blistering disease (IMSEBD).…”

Diagnosis and Management of Desquamative Gingivitis

“…MMP is one of a group of autoimmune, subepithelial blistering diseases that predominantly affect the mucous membranes (Bagan et al, 2005;Chan et al, 2002;Fleming & Korman, 2000;Scully & Lo Muzio, 2008). Most patients with this disease are in the fifth or sixth decade of life, and the majority of them are women (Chan et al, 2002;Fleming & Korman, 2000;Scully & Lo Muzio, 2008).…”