Diagnostic Advances
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Dermatol. May 2, 2016; 5(2): 65-71
Published online May 2, 2016. doi: 10.5314/wjd.v5.i2.65
CD34+ dermal dendritic cells and mucin deposition in dermatomyositis
Emi Yokoyama, Yoshitaka Nakamura, Tomoko Okita, Nobuyuki Nagai, Masahiko Muto
Emi Yokoyama, Yoshitaka Nakamura, Tomoko Okita, Nobuyuki Nagai, Masahiko Muto, Department of Dermatology, Yamaguchi University Graduate School of Medicine, Yamaguchi 755-0855, Japan
Author contributions: All the authors fully contributed to the preparation of this manuscript.
Supported by A research grant from the Japan Society for the Promotion of Science (25461695 to Masahiko Muto); and by a grant for Research on Measures for Intractable Diseases (to Masahiko Muto) from the Ministry of Health, Labour and Welfare, Japan.
Conflict-of-interest statement: We have no conflicts of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Masahiko Muto, MD, Professor, Chief, Department of Dermatology, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami- Kogushi, Ube, Yamaguchi 755-0855, Japan. mmuto@yamaguchi-u.ac.jp
Telephone: +81-836-222269 Fax: +81-836-222269
Received: October 27, 2015
Peer-review started: November 1, 2015
First decision: November 30, 2015
Revised: December 19, 2015
Accepted: January 27, 2016
Article in press: January 29, 2016
Published online: May 2, 2016
Processing time: 180 Days and 17.5 Hours
Abstract

Dermal mucinosis is often associated with collagen diseases such as rheumatoid arthritis, lupus erythematosus, and dermatomyositis, in addition to autoimmune thyroiditis. We report eight cases of dermal mucin deposition secondary to typical dermatomyositis with cutaneous lesions known as heliotrope rash and Gottron’s papules. Striking mucin deposition was observed in both the papillary dermis and reticular dermis of all biopsy specimens. Immunohistochemical analysis showed that CD34+ dermal dendritic cells (DDCs) in the perilesional area in combination with vimentin+ cells within the mucinous lesion might be important in giving rise to abnormal deposition of dermal mucin. On the other hand, numbers of factor XIIIa+ DDCs and tryptase+ mast cells were reduced within and surrounding the mucin deposition, as compared with those in the dermis of normal controls. A pathogenic mechanism of dermal mucin deposition is proposed.

Keywords: Mucin deposition; Dermatomyositis; CD34+ dermal dendritic cell

Core tip: Immunohistochemical analysis of skin biopsy specimens with dermatomyositis showed the involvement of CD34+ dermal dendritic cells, α-smooth muscle actin+ myofibroblasts and possibly mast cells, as well as vimentin+ fibroblasts for abnormally dermal mucin production. Further pathophysiological studies are required to more precisely clarify secondary cutaneous mucin deposition by CD34+ dermal dendritic cells. CD34+ dermal dendritic cells and mast cells might be important in giving rise to deposition of dermal mucin in dermatomyositis.