Journal List > J Rheum Dis > v.19(6) > 1064003

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Jung, Yoo, Kim, Seo, Lee, Lee, Park, Park, Baek, Kim, Kim, and Lee: A Case of Pediatric-onset Mixed Connective Tissue Disease Presenting Raynaud's Phenomenon Affecting Tongue, Hands, and Feet
Case Report
Journal of Rheumatic Diseases

Journal of Rheumatic Diseases 2012; 19(6): 344-347.

Published online: 31 December 2012

DOI: https://doi.org/10.4078/jrd.2012.19.6.344

A Case of Pediatric-onset Mixed Connective Tissue Disease Presenting Raynaud's Phenomenon Affecting Tongue, Hands, and Feet

Soon Myung Jung1, Jin Taek Yoo1, Young Hwan Kim1, Yu Na Seo2, Na Young Lee3, Seong Geun Lee4, Seong Hu Park4, Young Eun Park4, Seung Hoon Baek4, Geun Tae Kim5, Sung-Il Kim4, Joung Wook Lee1

1Department of Internal Medicine, Busan St. Mary's Medical Center, Busan, Korea.

2Department of Radiology, Busan St. Mary's Medical Center, Busan, Korea.

3Department of Pediatrics, Busan St. Mary's Medical Center, Busan, Korea.

4Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea.

5Department of Internal Medicine, Kosin University College of Medicine, Busan, Korea.

Corresponding to: Joung Wook Lee, Department of Internal Medicine, Busan St. Mary's Medical Center, 23, Yongho-ro 252 beon-gil, Nam-gu, Busan 608-838, Korea. gesundheit@paran.com

Received 23 October 2012       Revised 12 December 2012       Accepted 30 December 2012

Copyright © 2012 by The Korean College of Rheumatology

(free-access):

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

Mixed connective tissue disease (MCTD) was first described by Sharp and coworkers in 1972, characterized by symptoms of Raynaud's phenomenon or swollen hands, overlapping clinical features of systemic lupus erythematosus, systemic sclerosis, or polymyositis/dermatomyositis, and the presence of anti-U1 RNP antibody. MCTD is rare in children and constitutes 0.3~0.6% of all rheumatologic patients in pediatric rheumatology database of the United States. Here, we report the first Korean case of a 10-year-old female patient with MCTD, presenting Raynaud's phenomenon in the hands, feet, and tongue.

Keywords: Pediatric-onset mixed connective tissue disease, Raynaud's phenomenon, Antibody to ribonucleoprotein

Figures and Tables

Figure 1
Raynaud's phenomenon in the fingertips. Demarcated digital pallor and cyanosis developed after exposure to cold.
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Figure 2
Raynaud's phenomenon in both soles. Demarcated pallor developed after exposure to cold.
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Figure 3
Raynaud's phenomenon in the tongue. The photographs show color changes from red (A) to pallor and (B) after cold application.
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Figure 4
Magnetic Resonance Imaging (MRI) images of the right Foot. MRI shows focal low T1 signal intensity (A) and high T2 signal intensity (B) at the head of the second and fifth metatarsal bone, consistent with bone marrow edema. Scanty effusion is seen at the second and fifth metatarsophalangeal joint.
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References

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