Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations. 2018

Khalaf Kridin
Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel. dr_kridin@hotmail.com.

Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world. Observational studies investigating comorbidities and associations among patients with these diseases are inconsistent and sometimes inconclusive. This review provides a brief overview regarding each one of the subepidermal autoimmune bullous diseases. In addition, it summarizes the most recent understanding of the epidemiological features and associations of this group of organ-specific autoimmune diseases.

UI MeSH Term Description Entries
D007557 Israel A country in the Middle East, bordering the Mediterranean Sea, between Egypt and Lebanon. The capital is Jerusalem.
D009928 Organ Specificity Characteristic restricted to a particular organ of the body, such as a cell type, metabolic response or expression of a particular protein or antigen. Tissue Specificity,Organ Specificities,Specificities, Organ,Specificities, Tissue,Specificity, Organ,Specificity, Tissue,Tissue Specificities
D004817 Epidermis The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
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
D015897 Comorbidity The presence of co-existing or additional diseases with reference to an initial diagnosis or with reference to the index condition that is the subject of study. Comorbidity may affect the ability of affected individuals to function and also their survival; it may be used as a prognostic indicator for length of hospital stay, cost factors, and outcome or survival.

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