Childhood-Onset Anti-Ku Antibody-Positive Generalized Morphea with Polymyositis: A Japanese Case Study

Takayuki Kishi; Takako Miyamae; Ryoko Morimoto; Naoko Ishiguro; Yasuhito Hamaguchi; Manabu Fujimoto; Satoru Nagata; Hisashi Yamanaka

doi:10.1111/pde.12644

Childhood-Onset Anti-Ku Antibody-Positive Generalized Morphea with Polymyositis: A Japanese Case Study

Pediatr Dermatol. 2015 Sep-Oct;32(5):e224-5. doi: 10.1111/pde.12644. Epub 2015 Jul 23.

Authors

Takayuki Kishi^{1

2}, Takako Miyamae^{1

2}, Ryoko Morimoto³, Naoko Ishiguro³, Yasuhito Hamaguchi⁴, Manabu Fujimoto⁵, Satoru Nagata¹, Hisashi Yamanaka²

Affiliations

¹ Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.
² Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan.
³ Department of Dermatology, Tokyo Women's Medical University, Tokyo, Japan.
⁴ Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
⁵ Department of Dermatology, University of Tsukuba, Tsukuba, Japan.

PMID: 26205636
DOI: 10.1111/pde.12644

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.

Publication types

Case Reports

MeSH terms

Adolescent
Adrenal Cortex Hormones / administration & dosage
Antibodies, Antinuclear / immunology*
Antigens, Nuclear / immunology*
Biopsy, Needle
DNA-Binding Proteins / immunology*
Female
Humans
Immunohistochemistry
Japan
Ku Autoantigen
Magnetic Resonance Imaging
Methylprednisolone / administration & dosage
Polymyositis / drug therapy
Polymyositis / immunology*
Polymyositis / pathology*
Pulse Therapy, Drug
Rare Diseases
Risk Assessment
Scleroderma, Localized / drug therapy
Scleroderma, Localized / immunology*
Scleroderma, Localized / pathology*
Severity of Illness Index

Substances

Adrenal Cortex Hormones
Antibodies, Antinuclear
Antigens, Nuclear
DNA-Binding Proteins
Xrcc6 protein, human
Ku Autoantigen
Methylprednisolone