[Pseudoxanthoma elasticum with aortic insufficiency and arterial hypertension in a 12-year-old boy]

Hypertension was found in a 12 year-old Tunisian boy in the weeks following an aortic valvular replacement for aortic regurgitation which was surmised to be rheumatic in origin. In fact, the hypertension and aortic regurgitation were due to the Gröenblad-Strandberg syndrome, or pseudoxanthoma elasticum. The origin of the hypertension was demonstrated by selective renal angiography which showed interruption of the distal cortical renal arteries. Deposition of calcium in the arterial wall was shown by renal ultrasonography. The origin of aortic regurgitation was first imputed to acute rheumatic fever. Thus, the patient presented with complete picture of pseudoxanthoma elasticum with skin, arterial and cardiac involvement. The case reported is the third with a cardiac involvement. The case reported is the third with a cardiac localization of the disease histologically proven in a patient under 20 years of age. The frequency, transmission, histologic findings, etiopathogenesis and clinical aspects of pseudoxanthoma elasticum are reviewed in brief.