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December 1962

Pseudoxanthoma Elasticum: Internal Manifestations: A Report of Cases and a Statistical Review of the Literature

Author Affiliations


From the Department of Dermatology, Stanford University School of Medicine.

Arch Dermatol. 1962;86(6):729-740. doi:10.1001/archderm.1962.01590120027005

The history of pseudoxanthoma elasticum and angioid streaks is presented, and the question of their pathogenesis is discussed. Complete agreement does not exist as to the pathological process involved.

Four cases of pseudoxanthoma elasticum and angioid streaks (Grönblad-Strandberg syndrome) are presented plus a statistical review of 200 cases from the literature.

Familial studies in the literature have all suggested a recessive inheritance in this disorder wth partial limitation to the female.

The majority of cases have been reported in individuals beyond 30 years of age, but it is suspected that the syndrome often exists in an asymptomatic form from a much earlier age and is detected only when, for example, some circumstance such as trauma to an eye brings the individual to seek ophthalmological aid and the angioid streaks are then detected.

The results of the statistical review herein presented suggest that cramps in the legs, gastrointestinal hemorrhage, and angina-like symptoms, otherwise unexplained, especially in the younger age groups, should at least suggest the possibility of this syndrome and that skin changes and angioid streaks should be looked for closely in these individuals.

Visual difficulties, often severe, and hypertension also seem to be often encountered, but far more commonly in those affected individuals over 30 years of age. The visual difficulties appear to be casually related to retinal degeneration secondary to angioid streaks and tend to become more pronounced as the patient grows older, but the hypertension is far more difficult to evaluate, occurring mainly in an age group where hypertension is frequently seen due to other causes. Even so, it is suggested that more cases of "essential hypertension" may be on the basis of this disorder.

Severe psychic disturbances are sometimes seen, but minor disturbances such as "neurasthenia" and slight memory loss seem to be common occurrences.

The plethysmographic studies of our 4 cases show the usual variation from normal which has been described in these cases. It is felt that the diagnosis of pseudoxanthoma elasticum and/or angioid streaks warrants a complete vascular survey, including careful physical examination, ECG, x-rays of extremities for medial calcifications, and peripheral pulse and blood flow studies.

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