Int J Angiol 1995; 4(3): 169-171
DOI: 10.1007/BF02043014
Original Articles

© Georg Thieme Verlag KG Stuttgart · New York

A case of pseudoxanthoma elasticum associated with sick sinus syndrome

Maki Akasu1 , Keijiro Saku1 , Masanori Okabe1 , Koichi Handa1 , Kunihiro Matsuo1 , Shiro Jimi2 , Kikuo Arakawa1
  • 1Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan
  • 2Department of Pathology, Fukuoka University School of Medicine, Fukuoka, Japan
Part of this work was presented at the 76th Japanese Circulation Society Kyushu Meeting, Japan, June 18, 1994
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Publication History

Publication Date:
22 April 2011 (online)

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Abstract

Typical angioid streaks were found on routine ophthalmologic examination in a 42-year-old female with yellowish, coalescing papules on her neck without any symptoms. Biopsy of the cutaneous lesion showed degeneration and fragmentation of the elastic fibers and many basophilic-stained calcifications in mid-dermis, which are consistent with pseudoxanthoma elasticum. Twenty-four-hour Holter electrocardiography (ECG) showed sinus arrest for 2.5 seconds, and an electrophysiological study revealed sinus nodal dysfunction (sick sinus syndrome), whereas all of the coronary arteries were intact, despite a treadmill stress ECG test showing significant ST depressions. The association of pseudoxanthoma elasticum and sick sinus syndrome is very rare. One possible explanation for this association here is that the degeneration of elastic fiber in endomysium of the sino-atrial node may have affected heart conduction systems, resulting in sick sinus syndrome.