Turkish Journal of Nephrology
Case Report

A Rare Cause of Secondary Hypertension; Fabry Disease

1.

Necmettin Erbakan Üniversitesi, Tıp Fakültesi, Nefroloji Bilim Dalı, Konya, Türkiye

Turkish J Nephrol 2018; 27: 324-326
DOI: 10.5262/tndt.2018.2968
Read: 1236 Downloads: 724 Published: 30 January 2019

Fabry disease is an x-linked recessive metabolic storage disorder due to the deficiency of lysosomal α-galactosidase A, and the subsequent accumulation of glycosphingolipids, throughout the body. Patients usually present with decreased sweating (anhidrosis or hypohidrosis), reddish-purple skin rash in the bathing trunk area (angiokeratomas), personal or family history of “burning” or “hot” pain in the hands and feet, particularly during fevers (acroparesthesias), cornea verticillata and proteinuria. In addition, patients can rarely present with severe hypertension. Here, we have reported a Fabry’s patient who presented with severe hypertension as an unusual presentation.

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