A Case of Pachydermoperiostosis Combined with Undifferentiated Arthritis

Jung Hoon Lee; Seung Pyo Hong; Jun Kwon Ko; Eun Young Lee; Kang Lok Lee; Sung Jun Chung; Gun Woo Koo; So-Young Bang; Hye-Soon Lee

doi:10.4078/jrd.2015.22.4.256

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Lee, Hong, Ko, Lee, Lee, Chung, Koo, Bang, and Lee: A Case of Pachydermoperiostosis Combined with Undifferentiated Arthritis

Case Report

J Rheum Dis 2015;22(4):256-259.

Published online: 31 October 2015

DOI: https://doi.org/10.4078/jrd.2015.22.4.256

A Case of Pachydermoperiostosis Combined with Undifferentiated Arthritis

Jung Hoon Lee¹, Seung Pyo Hong¹, Jun Kwon Ko¹, Eun Young Lee¹, Kang Lok Lee¹, Sung Jun Chung¹, Gun Woo Koo¹, So-Young Bang^1,², Hye-Soon Lee^1,²

¹Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea

²Division of Rheumatology, Department of Internal Medicine, Hanyang University Guri Hospital, Guri, Korea

Corresponding to: Hye-Soon Lee, Division of Rheumatology, Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, 153 Gyeongchun-ro, Guri 11923, Korea. E-mail: lhsberon@hanyang.ac.kr

Received 6 July 2014 Revised 3 September 2014 Accepted 15 September 2014

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Pachydermoperiostosis (PDP) is a primary hypertrophic osteoarthropathy characterized by digital clubbing, pachydermia, and periostosis, which is inherited as an autosomal dominant or recessive trait. We report on a patient suffering from bilateral knee arthritis for 6 years who was newly diagnosed as PDP. PDP was confirmed by bilateral digital clubbing, hyperhidrosis, and cutis verticis gyrata, findings of pachydermatosis on the forehead and scalp, X-ray findings of proliferative periostitis. This case indicates that PDP is one of several possible rare diseases that should be considered in patients with undifferentiated arthritis.

Keywords: Pachydermoperiostosis, Primary hypertrophic osteoarthropathy, Undifferentiated arthritis

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Figure 1.

Sacroiliac joint radiography showing suspicious sclerosis with some erosions suggestive of bilateral grade II sacroiliitis; (A) right, (B) left.

Figure 2.

Enlargement of the hands with digital clubbing.

Figure 3.

Cutis verticis gyrate, forehead.

Figure 4.

Proliferative periostitis in the diaphysis of the middle phalanges and proximal phalanges of bilateral digits (arrows).

Figure 5.

Hypertrophic arthropathy involving bilateral knee joints.

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