title: Childhood Pemphigus Foliaceus with Exclusive Immunoglobulin G Autoantibodies to Desmocollins.
abstract: Pemphigus refers to a group of potentially fatal blistering skin diseases that are often due to the deleterious effects of autoantibodies directed against desmosomal antigens. Although desmogleins have been mainly implicated as autoantigens in pemphigus, a steadily growing body of evidence suggests that other desmosomal proteins may be causally involved as well. Antibodies directed against desmocollin-3 have been shown to play a direct role in the pathogenesis of several types of pemphigus. Here we describe the case of a child with localized pemphigus foliaceus and immunoglobulin G (IgG) reactivity exclusively directed to desmocollins. The present report suggests that autoantibodies against nondesmoglein antigens may play a role in the pathogenesis of superficial pemphigus, in addition to pemphigus vulgaris, paraneoplastic pemphigus, and IgA pemphigus.
authors.affiliation: Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Shamir
authors.initials: S
authors.lastname: Geller
authors.affiliation: Department of Pathology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Andrea
authors.initials: A
authors.lastname: Gat
authors.affiliation: Department of Pediatric Dermatology Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Avikam
authors.initials: A
authors.lastname: Harel
authors.affiliation: Department of Pediatric Dermatology Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Jacob
authors.initials: J
authors.lastname: Mashiah
authors.affiliation: Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Tal
authors.initials: T
authors.lastname: Zeeli
authors.affiliation: Department of Dermatology and Allergology, Philipps University, Marburg, Germany.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Eming
authors.affiliation: Department of Dermatology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.|Institute of Cutaneous Cell Biology, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
authors.affiliation: Department of Dermatology and Allergology, Philipps University, Marburg, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
authors.affiliation: Department of Dermatology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.|Institute of Cutaneous Cell Biology, Kurume University, Kurume, Fukuoka, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.affiliation: Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Eli
authors.initials: E
authors.lastname: Sprecher
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D002648:Child; D051187:Desmocollins / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D010392:Pemphigus / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000473:pathology*
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D051187:Desmocollins; D007074:Immunoglobulin G