Biomaterial Database

Vesiculo-bullous mucocutaneous disease: benign mucous membrane and bullous pemphigoid. 1990

D M Williams

Department of Oral Pathology, London Hospital Medical College, England.

Bullous pemphigoid (BP) and benign mucous membrane pemphigoid (BMMP) are autoimmune diseases characterised by subepithelial bulla formation and showing substantial overlap in clinical signs and symptoms. BP principally involves skin and BMMP the oral mucosa and eyes. The gingiva are affected in 90% of cases of BMMP and buccal mucosa and palate in up to 30%. Lesions may heal with scarring. Extension into the pharynx and esophagus causes sore throat and dysphagia. Severe ocular involvement may cause blindness. Bulla formation is attributed to complement activation, following IgG binding to the basement membrane zone, with subsequent polymorphonuclear leukocyte accumulation. The target antigen in BP is a 180-230 kD protein associated with the basilar membrane of basal keratinocytes. The gene encoding the BP antigen has been partially cloned. It is likely that the same antigen is involved in BMMP, but the mechanism of scarring is not understood. Treatment of BP and BMMP includes systemic steroid and azathioprine therapy and topical steroids.

UI	MeSH Term	Description	Entries
D009061	Mouth Mucosa	Lining of the ORAL CAVITY, including mucosa on the GUMS; the PALATE; the LIP; the CHEEK; floor of the mouth; and other structures. The mucosa is generally a nonkeratinized stratified squamous EPITHELIUM covering muscle, bone, or glands but can show varying degree of keratinization at specific locations.	Buccal Mucosa,Oral Mucosa,Mucosa, Mouth,Mucosa, Oral
D010390	Pemphigoid, Benign Mucous Membrane	A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring. It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement.	Pemphigoid, Cicatricial,Benign Mucosal Pemphigoid,Benign Mucous Membrane Pemphigoid,Cicatricial Pemphigoid, Ocular,Mucous Membrane Pemphigoid, Benign,Ocular Cicatricial Pemphigoid,Pemphigoid, Ocular Cicatricial,Benign Mucosal Pemphigoids,Cicatricial Pemphigoid,Cicatricial Pemphigoids, Ocular,Mucosal Pemphigoid, Benign,Mucosal Pemphigoids, Benign,Ocular Cicatricial Pemphigoids,Pemphigoid, Benign Mucosal,Pemphigoids, Benign Mucosal,Pemphigoids, Ocular Cicatricial
D010391	Pemphigoid, Bullous	A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis.	Pemphigoid,Bullous Pemphigoid,Pemphigoids
D003320	Corneal Ulcer	Loss of epithelial tissue from the surface of the cornea due to progressive erosion and necrosis of the tissue; usually caused by bacterial, fungal, or viral infection.	Keratitis, Ulcerative,Keratitides, Ulcerative,Ulcer, Corneal,Ulcerative Keratitides,Ulcerative Keratitis
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005891	Gingivitis	Inflammation of gum tissue (GINGIVA) without loss of connective tissue.	Gingivitides
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001327	Autoimmune Diseases	Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.	Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune
D001485	Basement Membrane	A darkly stained mat-like EXTRACELLULAR MATRIX (ECM) that separates cell layers, such as EPITHELIUM from ENDOTHELIUM or a layer of CONNECTIVE TISSUE. The ECM layer that supports an overlying EPITHELIUM or ENDOTHELIUM is called basal lamina. Basement membrane (BM) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. BM, composed mainly of TYPE IV COLLAGEN; glycoprotein LAMININ; and PROTEOGLYCAN, provides barriers as well as channels between interacting cell layers.	Basal Lamina,Basement Lamina,Lamina Densa,Lamina Lucida,Lamina Reticularis,Basement Membranes,Densas, Lamina,Lamina, Basal,Lamina, Basement,Lucida, Lamina,Membrane, Basement,Membranes, Basement,Reticularis, Lamina
D012872	Skin Diseases, Vesiculobullous	Skin diseases characterized by local or general distributions of blisters. They are classified according to the site and mode of blister formation. Lesions can appear spontaneously or be precipitated by infection, trauma, or sunlight. Etiologies include immunologic and genetic factors. (From Scientific American Medicine, 1990)	Bullous Skin Diseases,Pustular Dermatosis, Subcorneal,Skin Diseases, Bullous,Skin Diseases, Vesicular,Sneddon-Wilkinson Disease,Subcorneal Pustular Dermatosis,Vesicular Skin Diseases,Vesiculobullous Skin Diseases,Bullous Dermatoses,Vesiculobullous Dermatoses,Bullous Skin Disease,Dermatoses, Bullous,Dermatoses, Subcorneal Pustular,Dermatoses, Vesiculobullous,Dermatosis, Subcorneal Pustular,Pustular Dermatoses, Subcorneal,Skin Disease, Bullous,Skin Disease, Vesicular,Skin Disease, Vesiculobullous,Sneddon Wilkinson Disease,Subcorneal Pustular Dermatoses,Vesicular Skin Disease,Vesiculobullous Skin Disease