title: Case with Brunsting-Perry-like localized subepidermal blister formations and immunoglobulin G antibodies against unidentified basement membrane zone antigen.
abstract: Brunsting-Perry type bullous pemphigoid is defined by the blister formation limited to the head and neck, and autoantibodies to type VII collagen are detected in several cases. However, the pathomechanisms and autoantigens in this condition remain unknown. We report a 20-year-old female patient with a more than 2-year history of recurrent tense blisters localized on the face with no distinct atrophic scar formation. The patient had neither extensive sun exposure nor a history suggestive of contact dermatitis. Oral betamethasone was effective on the skin lesions. Histopathology revealed subepidermal blister formation with dermal infiltrates of neutrophils. Although direct and indirect immunofluorescence tests detected immunoglobulin G antibodies to the basement membrane zone (BMZ), no known dermal or epidermal autoantigens were detected in immunoblot analyses. Therefore, this case may be a rare variant of Brunsting-Perry type localized bullous pemphigoid with autoantibodies to an undetermined BMZ antigen.
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Mami
authors.initials: M
authors.lastname: Sato-Shibuya
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Teruki
authors.initials: T
authors.lastname: Dainichi
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Gyohei
authors.initials: G
authors.lastname: Egawa
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Honda
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Otsuka
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan.
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
authors.affiliation: Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.|Shiga Medical Center for Adults, Shiga, Japan.
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Miyachi
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Kabashima
mesh_terms: D000284:Administration, Oral; D000328:Adult; D001323:Autoantibodies / Q000032:analysis* / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology*; D001623:Betamethasone / Q000008:administration & dosage / Q000627:therapeutic use*; D004817:Epidermis / Q000473:pathology; D005145:Face; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis* / Q000276:immunology; D008856:Microscopy, Fluorescence; D010391:Pemphigoid, Bullous / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D005938:Glucocorticoids; D007074:Immunoglobulin G; D001623:Betamethasone
keywords: Brunsting-Perry bullous pemphigoid; blister; epidermolysis bullosa acquisita; face; localized