The clinical expression of bullous pemphigoid is not determined by the specificity of the target antigen

Summary The major bullous pemphigoid (BP) antigen is a 220–240‐kDa polypeptide, although some BP sera recognize hands of 180–200 kDa or lower molecular weight. We have investigated to what extent this heterogeneity of the target antigen accounts for the clinical diversity of BP. Immunoblotting studies against extracts of salt‐separated epidermis were performed on sera from 39 patients with BP. The blotting patterns obtained were correlated with the clinical findings, with particular reference to prodromal itching, lesion morphology and severity, mucosal involvement, presence of milia, dapsone responsiveness and disease duration. The results confirm that the major BP antigen is a 220‐kDa polypeptide, and that the 180‐kDa polypeptide is a second and sometimes the sole BP antigen identified in immunoblots. Rarely, multiple bands of lower molecular weight were found. There was no correlation between the pattern of BP antigens detected in immunoblots and the clinical presentation and course of BP. There was considerable clinical diversity even among the nine patients showing specificity for a single 220‐kDa target antigen. Although two patients with a single 180‐kDa antigen specificity had a disease of unusually long duration, factors other than antigen specificity must determine the clinical expression of BP.

[1]  A. Parodi,et al.  Pemphigoid nodularis , 1993, The British journal of dermatology.

[2]  U. Haustein,et al.  HLA in Bullous Pemphigoid , 1991, International journal of dermatology.

[3]  J. Bystryn,et al.  Identification of a 160-kD molecule as a component of the basement membrane zone and as a minor bullous pemphigoid antigen. , 1990, The Journal of investigative dermatology.

[4]  S. C. Kim,et al.  Studies of the relationship of the 230-kD and 180-kD bullous pemphigoid antigens. , 1990, The Journal of investigative dermatology.

[5]  J. Zone,et al.  Two groups of bullous pemphigoid antigens are identified by affinity-purified antibodies. , 1990, The Journal of investigative dermatology.

[6]  R. Pye,et al.  Recognition of two distinct major antigens by bullous pemphigoid sera , 1990, The British journal of dermatology.

[7]  V. Venning,et al.  HLA type in bullous pemphigoid, cicatricial pemphigoid and linear IgA disease , 1989, Clinical and experimental dermatology.

[8]  I. Leigh,et al.  Identification of the epidermolysis bullosa acquisita antigen by LH 7.2 monoclonal antibody: use in diagnosis * , 1989, The British journal of dermatology.

[9]  J. Saurat,et al.  Heterogeneous bullous pemphigoid antibodies: detection and characterization by immunoblotting when absent by indirect immunofluorescence. , 1989, The Journal of investigative dermatology.

[10]  P. Millard,et al.  Dapsone as first line therapy for bullous pemphigoid , 1989, The British journal of dermatology.

[11]  H. Su,et al.  Bullous pemphigoid and cicatricial pemphigoid: immunoblotting detection of involved autoantigens. , 1989, Autoimmunity.

[12]  J. Stanley,et al.  A 230-kD basic protein is the major bullous pemphigoid antigen. , 1989, The Journal of investigative dermatology.

[13]  D. Gawkrodger,et al.  Clinical, histological and immunological studies in 50 patients with bullous pemphigoid. , 1988, Dermatologica.

[14]  R. Graham‐Brown Bullous pemphigoid with figurate erythema associated with carcinoma of the bronchus , 1987, The British journal of dermatology.

[15]  L. Diaz,et al.  Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting. , 1985, Journal of immunology.

[16]  D. Woodley,et al.  Identification and partial characterization of pemphigoid antigen extracted from normal human skin. , 1984, The Journal of investigative dermatology.

[17]  E. Shevach,et al.  Characterization of bullous pemphigoid antigen: A unique basement membrane protein of stratified squamous epithelia , 1981, Cell.

[18]  W. M. Stewart [Pemphigus and pemphigoid]. , 1980, Dermatologica.

[19]  W. F. Lever Pemphigus and pemphigoid: A review of the advances made since 1964 , 1979 .

[20]  A. Ahmed,et al.  Bullous Pemphigoid: Clinical and Immunologic Follow-up After Successful Therapy , 1977 .

[21]  L. Blumenson,et al.  HLA in bullous pemphigoid. , 1977, Archives of dermatology.

[22]  A. Ahmed,et al.  Bullous pemphigoid: clinical and serologic evaluation for associated malignant neoplasms. , 1977 .

[23]  E. Beutner,et al.  Basement zone antibodies in bullous pemphigoid. , 1967, JAMA.

[24]  Arthur Rook,et al.  PEMPHIGUS AND PEMPHIGOID. , 1953, The British journal of dermatology.