The skin lesions typically are distributed in the intertriginous areas of the body. The skin changes usually are noted between the second and fourth decades of life. Late in the disease, the skin frequently becomes thickened and hangs in loose redundant folds. Central nervous system Neurologic complications in patients with PXE have been reported in the literature. These include multiple lacunar infarcts, aneurysms, cerebrovascular insufficiency, subarachnoid and intracerebral hemorrhages, progressive intellectual deterioration, and psychic and mental disturbance.
Seizures occur more frequently than in the general population.
Subarachnoid hemorrhage is a potential cause of death. Cardiovascular findings Cardiovascular involvement in patients with PXE occurs at an early age, but it is rarely a presenting manifestation. Angina pectoris is a common finding, but myocardial infarction is rare. Aneurysms may occur in any region of the cardiovascular system. GI hemorrhage has been reported as early as age 6.5 years.