title: IgE autoantibodies directed against the major bullous pemphigoid antigen in patients with a severe form of pemphigoid.
abstract: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized in part by circulating and tissue-bound IgG autoantibodies directed against the basement membrane zone. In addition, most of the patients with BP have increased serum IgE levels which seem to be correlated with the disease activity, whereas the presence of circulating anti-basement membrane zone IgE Abs has been reported in some patients. To elucidate whether IgE-dependent mechanisms play a role in the physiopathology of BP, we looked for the presence of IgE Abs specifically directed against the major BP Ag (BPAg1) in sera of BP patients at the onset and after remission of the disease. A radioimmunoassay and a 55-kDa recombinant protein (rBP55) obtained from a cDNA sequence, encoding the C-terminal region of the BPAg1 and containing the BPAg1 immunodominant epitopes, were used. Anti-rBP55 IgE Abs were found in 12 of the 19 sera tested. When the patients were divided into two groups according to the disease severity, anti-rBP55 IgE Abs were found only in patients with a severe form of the disease. Cytophilic IgE was detected on approximately 20% of peripheral blood eosinophils purified from BP patients. Immunohistochemistry studies suggested that some of the IgE-bearing cells in the lesional skin of BP patients are eosinophils. Immunostaining experiments revealed the existence of FcepsilonRI on both peripheral blood and tissue eosinophils. Taken together, these results suggest that IgE-dependent mechanisms could participate in the constitution of the lesions in BP.
authors.affiliation: Dermatology Clinic, Claude-Huriez Hospital, CHRU, Lille, France.
authors.forename: E
authors.initials: E
authors.lastname: Delaporte
authors.forename: A
authors.initials: A
authors.lastname: Dubost-Brama
authors.forename: R
authors.initials: R
authors.lastname: Ghohestani
authors.forename: J F
authors.initials: JF
authors.lastname: Nicolas
authors.forename: J L
authors.initials: JL
authors.lastname: Neyrinck
authors.forename: H
authors.initials: H
authors.lastname: Bergoend
authors.forename: A
authors.initials: A
authors.lastname: Janin
authors.forename: M
authors.initials: M
authors.lastname: Capron
mesh_terms: D000918:Antibody Specificity; D001323:Autoantibodies / Q000097:blood* / Q000378:metabolism; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D004804:Eosinophils / Q000276:immunology; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood* / Q000378:metabolism; D007150:Immunohistochemistry; D008297:Male; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000209:etiology / Q000276:immunology* / Q000503:physiopathology; D017455:Receptors, IgE / Q000378:metabolism; D011994:Recombinant Proteins / Q000276:immunology; D012867:Skin / Q000276:immunology; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D007073:Immunoglobulin E; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D017455:Receptors, IgE; D011994:Recombinant Proteins; D000097805:Collagen Type XVII; C486268:DST protein, human