Familial pseudoxanthoma elasticum and valvular heart disease.

PSEUDOXANTHOMA ELASTICUM has been described as a systemic disease that affects the skin, the eyes, and the blood vessels. That it may directly involve the heart and cause significant valvular disease is not so well known. Histologic changes in the endocardium and heart valves have been described'-" but valvular heart disease has not been a clinical problem in these cases. After studying the following familial cases of pseudoxanthoma elasticum, especially case 1 who died in severe congestive heart failure due to mitral stenosis, it was considered that another differential diagnosis must be added to the uncommon causes of valvular heart disease.