Biomaterial Database

Benign chronic bullous dermatosis of childhood. Linear IgA and C3 deposition on the basement membrane. 1976

S Prystowsky, and J N Gilliam

Benign chronic bullous dermatosis of childhood (BCBDC) is characterized by predominantly monomorphous, large, tense bullae with a predilection for the lower part of the trunk, the pelvic region, and the lower extremities. Most patients have blisters for two to three years and experience spontaneous exacerbations and remissions. We report the first case known to us in which a linear bank of IgA (but no lgG or lgM) and complement was found at the epidermal basal lamina as demonstrated by immunofluorescence studies.

UI	MeSH Term	Description	Entries
D007070	Immunoglobulin A	Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions.	IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
D008297	Male		Males
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D002908	Chronic Disease	Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2).	Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D003165	Complement System Proteins	Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).	Complement Proteins,Complement,Complement Protein,Hemolytic Complement,Complement, Hemolytic,Protein, Complement,Proteins, Complement,Proteins, Complement System
D003176	Complement C3	A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.	C3 Complement,C3 Precursor,Complement 3,Complement C3 Precursor,Complement Component 3,Precursor-Complement 3,Pro-C3,Pro-Complement 3,C3 Precursor, Complement,C3, Complement,Complement, C3,Component 3, Complement,Precursor Complement 3,Precursor, C3,Precursor, Complement C3,Pro C3,Pro Complement 3
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
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