title: Childhood-Onset Anti-Ku Antibody-Positive Generalized Morphea with Polymyositis: A Japanese Case Study.
abstract: We report a 16-year-old Japanese girl with anti-Ku antibody-positive generalized morphea and polymyositis who, at the age of 7 years, developed multiple brownish plaques on her left forearm that gradually extended to her upper arm, back, and left thigh, which a skin biopsy revealed as morphea. Laboratory testing was positive for antinuclear antibody and a high serum creatine kinase level. Although there were no clinical signs of muscular involvement, magnetic resonance imaging revealed findings consistent with myositis. The patient is one of the youngest reported cases positive for anti-Ku antibody. Anti-Ku positivity concomitant with generalized morphea is rare.
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.|Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kishi
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.|Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Takako
authors.initials: T
authors.lastname: Miyamae
authors.affiliation: Department of Dermatology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Ryoko
authors.initials: R
authors.lastname: Morimoto
authors.affiliation: Department of Dermatology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Ishiguro
authors.affiliation: Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Hamaguchi
authors.affiliation: Department of Dermatology, University of Tsukuba, Tsukuba, Japan.
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Nagata
authors.affiliation: Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Yamanaka
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000974:Antibodies, Antinuclear / Q000276:immunology*; D034961:Antigens, Nuclear / Q000276:immunology*; D001707:Biopsy, Needle; D004268:DNA-Binding Proteins / Q000276:immunology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007564:Japan; D000072200:Ku Autoantigen; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage; D017285:Polymyositis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D020551:Pulse Therapy, Drug; D035583:Rare Diseases; D018570:Risk Assessment; D012594:Scleroderma, Localized / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D012720:Severity of Illness Index
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D034961:Antigens, Nuclear; D004268:DNA-Binding Proteins; C000606509:Xrcc6 protein, human; D000072200:Ku Autoantigen; D008775:Methylprednisolone