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.
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References
Christiano AM, Lebwohl MG, Boyd CD, Uitto J (1992) Workshop on pseudoxanthoma elasticum: Molecular biology and pathology of the elastic fibers. J Invest Dermatol 99:660–663.
Gomolin JES (1992) Development of angioid streaks in association with pseudoxanthoma elasticum. Can J Ophthalmol 27:30–31.
Hacker SM, Ramos-Caro FA, Beers BB, Flowers FP (1993) Juvenile pseudoxanthoma elasticum: Recognition and management. Pediatr Dermatol 10:19–25.
Lebwohl M, Schwartz E, Lemlich G, Lovelace O, Shaikh-Bahai F, Fleischmajer R (1993) Abnormalities of connective tissue components in lesional and non-lesional tissue of patients with pseudoxanthoma elasticum. Arch Dermatol Res 285:121–126.
Rosenzweig BP, Guarneri E, Kronzon I (1993) Echocardiographic manifestations in a patient with pseudoxanthoma elasticum. Ann Int Med 119:487–491.
Pope FM (1974a) Two types of autosomal recessive pseudoxanthoma elasticum. Arch Dermatol 110:209–212.
Pope FM (1974b) Autosomal dominant pseudoxanthoma elasticum. J Med Genet 11:152–157.
Pope FM (1975) Historical evidence for the genetic heterogeneity of pseudoxanthoma elasticum. Br J Dermatol 92:493–509.
Saku K, Zhang B, Jimi S, Takebayashi S, Arakawa K (1994) Captopril suppresses atherosclerosis in cholesterol-fed rabbits without affecting lipids and insulin. 15th Scientific Meeting of the International Society of Hypertension (Melbourne) (abstract no. 766).
Griepp E (1983) The heart. In: Histology, Cell and Tissue Biology 5th ed, Weiss (ed). Elsevier Science Publishing Co, Inc: New York, pp 434–446.
Hamada M, Fukuda K, Handa S, Abe J, Hasegawa N, Ogawa S, Nakamura Y (1989) A case of pseudoxanthoma elasticum with coronary invasion manifested with unstable angina. J Jpn Soc Int Med 78:54–58.
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Akasu, M., Saku, K., Okabe, M. et al. A case of pseudoxanthoma elasticum associated with sick sinus syndrome. International Journal of Angiology 4, 169–171 (1995). https://doi.org/10.1007/BF02043014
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DOI: https://doi.org/10.1007/BF02043014