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Pseudoxanthoma Elasticum and Sickle Cell Anemia

Arch Intern Med. 1964;113(1):135-141. doi:10.1001/archinte.1964.00280070137022.
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Introduction  In recent years there has been considerable interest focused on hereditary disorders. This paper is concerned with the unusual combination of two of these disorders, pseudoxanthoma elasticum (PXE) and sickle cell anemia. There is now some evidence that there may be an overlap in the histological findings of the two diseases.The basic defect of PXE is probably a generalized defect of elastic tissue, and therefore symptoms are multiple and varied. Organ systems may be involved wherever elastic tissue exists. The disease is most often recognized because of cutaneous lesions; however, ocular disturbances are also common. Involvement of the cardiovascular system causes a great variety of problems such as cardiac enlargement, vascular damage, aneu- rysm, peripheral vascular disease, arterial calcification, and gastrointestinal hemorrhage.Sickle cell anemia, the more familiar of the two diseases, has been better understood since the discovery by Pauling and his associates.1 Their studies indicate


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