title: A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180.
abstract: Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9-year-old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120-kDa soluble ectodomain and against the C-terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt-split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non-scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow-up of patients with LVPC is required.
authors.affiliation: University of Münster, D-48149 Münster, Germany. schuman@uni-muenster.de
authors.forename: H
authors.initials: H
authors.lastname: Schumann
authors.forename: U
authors.initials: U
authors.lastname: Amann
authors.forename: K
authors.initials: K
authors.lastname: Tasanen
authors.forename: S
authors.initials: S
authors.lastname: Müller
authors.forename: D
authors.initials: D
authors.lastname: Zillikens
authors.forename: D
authors.initials: D
authors.lastname: Metze
authors.forename: T
authors.initials: T
authors.lastname: Luger
authors.forename: L
authors.initials: L
authors.lastname: Bruckner-Tuderman
authors.forename: G
authors.initials: G
authors.lastname: Bonsmann
mesh_terms: D001323:Autoantibodies / Q000032:analysis* / Q000097:blood; D015415:Biomarkers / Q000032:analysis / Q000097:blood; D002648:Child; D003094:Collagen / Q000276:immunology*; D003176:Complement C3 / Q000032:analysis; D003937:Diagnosis, Differential; D004591:Electrophoresis, Polyacrylamide Gel; D018604:Epitope Mapping; D000939:Epitopes / Q000276:immunology; D005260:Female; D006683:HLA-DQ Antigens / Q000032:analysis; D059866:HLA-DQ beta-Chains; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis*; D008856:Microscopy, Fluorescence; D010392:Pemphigus / Q000175:diagnosis*; D012867:Skin / Q000276:immunology*; D014845:Vulvar Diseases / Q000175:diagnosis*
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D003176:Complement C3; D000939:Epitopes; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D007074:Immunoglobulin G; D003094:Collagen