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September 1978

Cardiovascular Pseudoxanthoma Elasticum

Author Affiliations

Buffalo, NY

Arch Dermatol. 1978;114(9):1400. doi:10.1001/archderm.1978.01640210079031

To the Editor.—  Pseudoxanthoma elasticum (PXE) is a systemic heritable disorder aptly named for its xanthoma-like cutaneous lesions and its involvement of elastic tissue. Patients with this disease often have premature and severe vascular disease. It has been suggested that carriers of PXE may not be uncommon and that these people may also be prone to vascular disease and its complications.1 Altman and his associates evaluated the incidence of PXE in Seattle and found that it may be much more frequent than reported, with carriers representing a possible 1% or more of the general population. In the February issue of the Archives (114:279-280, 1978), PXE that was apparently limited to one organ system was reported. The authors of this report speculated that single-organ involvement may represent mosaicism, variable expressivity, or heterozygote expression, and that such involvement might also occur in the cardiovascular system elastic tissue, producing many patients with premature

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