Bullous dermatosis associated with dysglobulinemia (two cases). Relationships with epidermolysis bullosa acquisita. 1982

A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau

Two cases of bullous dermatosis with dysglobulinemia are described. The first one was associated with renal and neuromuscular amyloidosis and production of a monoclonal lambda IgG. Optical and electron microscopy showed amyloid deposits beneath basal lamina of the dermis. Results for direct and indirect immunofluorescence (IF) were negative. This bullous dermatosis is not an epidermolysis bullosa acquisita (EBA), in the strict sense, because the amorphous material deposited is amyloid. In the second case, associated with Waldenström's disease, there was no cutaneous or systemic amyloidosis. Direct IF was positive; linear IgM deposits were seen along the basal membrane of the bulla and the healthy skin. Indirect IF showed the presence of circulating antibodies against basal membrane zone. This bullous dermatosis is probably an EBA, despite the absence of IgG deposits. The absence of electron microscopy does not permit the confirmation of this diagnosis.

UI MeSH Term Description Entries
D007074 Immunoglobulin G The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B. Gamma Globulin, 7S,IgG,IgG Antibody,Allerglobuline,IgG(T),IgG1,IgG2,IgG2A,IgG2B,IgG3,IgG4,Immunoglobulin GT,Polyglobin,7S Gamma Globulin,Antibody, IgG,GT, Immunoglobulin
D004406 Dysgammaglobulinemia An immunologic deficiency state characterized by selective deficiencies of one or more, but not all, classes of immunoglobulins. Dysgammaglobulinemias
D004820 Epidermolysis Bullosa Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junctional, and dystrophic. Each of the latter three has several varieties. Acantholysis Bullosa
D005260 Female Females
D005455 Fluorescent Antibody Technique Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy. Antinuclear Antibody Test, Fluorescent,Coon's Technique,Fluorescent Antinuclear Antibody Test,Fluorescent Protein Tracing,Immunofluorescence Technique,Coon's Technic,Fluorescent Antibody Technic,Immunofluorescence,Immunofluorescence Technic,Antibody Technic, Fluorescent,Antibody Technics, Fluorescent,Antibody Technique, Fluorescent,Antibody Techniques, Fluorescent,Coon Technic,Coon Technique,Coons Technic,Coons Technique,Fluorescent Antibody Technics,Fluorescent Antibody Techniques,Fluorescent Protein Tracings,Immunofluorescence Technics,Immunofluorescence Techniques,Protein Tracing, Fluorescent,Protein Tracings, Fluorescent,Technic, Coon's,Technic, Fluorescent Antibody,Technic, Immunofluorescence,Technics, Fluorescent Antibody,Technics, Immunofluorescence,Technique, Coon's,Technique, Fluorescent Antibody,Technique, Immunofluorescence,Techniques, Fluorescent Antibody,Techniques, Immunofluorescence,Tracing, Fluorescent Protein,Tracings, Fluorescent Protein
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
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

Related Publications

A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
January 1981, JAMA,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
January 2005, Dermatology (Basel, Switzerland),
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
September 1997, Clinical and experimental dermatology,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
December 1974, Proceedings of the Royal Society of Medicine,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
March 1994, International journal of dermatology,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
November 1987, Journal of the American Academy of Dermatology,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
May 2010, Acta dermato-venereologica,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
February 1951, Klinische Medizin; osterreichische Zeitschrift fur wissenschaftliche und praktische Medizin,
A Gompel, and O Bletry, and Y de Prost, and J Wechsler, and B Wechsler, and P Lebon, and P Godeau
September 2007, Clinical and experimental dermatology,
Copied contents to your clipboard!