Biomaterial Database

Atopic dermatitis and impaired neutrophil chemotaxis in Job's syndrome. 1977

D Paslin, and M E Norman

A 22-year-old white woman with Job's syndrome was found to have atopic dermatitis and impaired neutrophil chemotaxis in vitro. Major clinical features of Job's syndrome included large, "cold" and recurrent staphylococcal abscesses, and intermittent bacterial and yeast infections. Evidence for atopic disease included infantile eczema progressing to flexural dermatitis, a family history of atopy, positive immediate hypersensitivity skin tests, and hyperimmunoglobulinemia E. Defective erythema responses to histamine, methyl niacinate, and methacholine (Mecholyl) chloride may explain the lack of redness, heat, or pain signalling the development of abscesses (hence the term "cold"). Impaired chemotaxis was probably due to an intrinsic neutrophil defect since patient's serum generated normal amounts of chemotactic factors and did not contain an inhibitor of neutrophil chemotaxis. A delay in neutrophil exudation in vivo may explain the abscess formations and the atopic diathesis may explain the absence of clinical signs of inflammation that have been described in this and other patients with Job's syndrome.

UI	MeSH Term	Description	Entries
D007109	Immunity	Nonsusceptibility to the invasive or pathogenic effects of foreign microorganisms or to the toxic effect of antigenic substances.	Immune Process,Immune Response,Immune Processes,Immune Responses,Process, Immune,Response, Immune
D007589	Job Syndrome	Primary immunodeficiency syndrome characterized by recurrent infections and hyperimmunoglobulinemia E. Most cases are sporadic. Of the rare familial forms, the dominantly inherited subtype has additional connective tissue, dental and skeletal involvement that the recessive type does not share.	HIES, Autosomal Recessive,Hyper-IgE Syndrome, Autosomal Recessive,Hyper-Immunoglobulin E Syndrome, Autosomal Recessive,Hyperimmunoglobulin E-Recurrent Infection Syndrome,Job's Syndrome,Buckley Syndrome,HIE Syndrome,HIES, Autosomal Dominant,Hyper-IgE Recurrent Infection Syndrome, Autosomal Recessive,Hyper-IgE Syndrome,Hyper-IgE Syndrome, Autosomal Dominant,Hyper-Immunoglobulin E Syndrome, Autosomal Dominant,Hyperimmunoglobulin E, Recurrent Infection Syndrome,Hyperimmunoglobulinemia E Syndrome,Job-Buckley Syndrome,Autosomal Dominant HIES,Autosomal Dominant HIESs,Autosomal Recessive HIES,Autosomal Recessive HIESs,Buckley Syndromes,HIE Syndromes,HIESs, Autosomal Dominant,HIESs, Autosomal Recessive,Hyper IgE Recurrent Infection Syndrome, Autosomal Recessive,Hyper IgE Syndrome,Hyper IgE Syndrome, Autosomal Dominant,Hyper IgE Syndrome, Autosomal Recessive,Hyper Immunoglobulin E Syndrome, Autosomal Dominant,Hyper Immunoglobulin E Syndrome, Autosomal Recessive,Hyper-IgE Syndromes,Hyperimmunoglobulin E Recurrent Infection Syndrome,Hyperimmunoglobulinemia E Syndromes,Job Buckley Syndrome,Job Syndromes,Job-Buckley Syndromes,Jobs Syndrome,Syndrome, Job,Syndromes, Job
D009504	Neutrophils	Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.	LE Cells,Leukocytes, Polymorphonuclear,Polymorphonuclear Leukocytes,Polymorphonuclear Neutrophils,Neutrophil Band Cells,Band Cell, Neutrophil,Cell, LE,LE Cell,Leukocyte, Polymorphonuclear,Neutrophil,Neutrophil Band Cell,Neutrophil, Polymorphonuclear,Polymorphonuclear Leukocyte,Polymorphonuclear Neutrophil
D010585	Phagocyte Bactericidal Dysfunction	Disorders in which phagocytic cells cannot kill ingested bacteria; characterized by frequent recurring infection with formulation of granulomas.	Bactericidal Dysfunction, Phagocyte,Bactericidal Dysfunctions, Phagocyte,Dysfunction, Phagocyte Bactericidal,Dysfunctions, Phagocyte Bactericidal,Phagocyte Bactericidal Dysfunctions
D002634	Chemotaxis, Leukocyte	The movement of leukocytes in response to a chemical concentration gradient or to products formed in an immunologic reaction.	Leukotaxis,Leukocyte Chemotaxis
D003876	Dermatitis, Atopic	A chronic inflammatory genetically determined disease of the skin marked by increased ability to form reagin (IgE), with increased susceptibility to allergic rhinitis and asthma, and hereditary disposition to a lowered threshold for pruritus. It is manifested by lichenification, excoriation, and crusting, mainly on the flexural surfaces of the elbow and knee. In infants it is known as infantile eczema.	Eczema, Atopic,Eczema, Infantile,Neurodermatitis, Atopic,Neurodermatitis, Disseminated,Atopic Dermatitis,Atopic Eczema,Atopic Neurodermatitis,Disseminated Neurodermatitis,Infantile Eczema
D004890	Erythema	Redness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes.	Erythemas
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults