Details

Autor(en) / Beteiligte

• Ortiz Gorraiz, M Angel
• Casares Arias, A
• Tallada Buñuel, M
• Vicente Prados, F J
• Honrubia Vílchez, B
• Fernández Sánchez, A

Titel

Urologic findings in pseudoxanthoma elasticum: report one case

Ist Teil von

• Actas urologicas españolas, 2005-01, Vol.29 (1), p.96-99

Ort / Verlag

Spain

Erscheinungsjahr

2005

Quelle

Free E-Journal (出版社公開部分のみ）

Beschreibungen/Notizen

• A 21-year-old woman presented with retinal angioid streaks and yellowish streak skin abnormalities in neck and axillary folds. Skin biopsy showed bluish-gray tangled masses of calcified elastic fibers in the mid- to lower dermis suggestive of Pseudoxanthoma elasticum (PXE). She consulted in Urology Department for lower urinary tract infection. Renal ultrasonography revealed multiple highly reflective foci in the corticomedullary junction. Pseudoxanthoma elasticum is a rare genetic disorder characterised by fragmentation and calcification of elastic fibers in the skin and media of arteries. Frequent manifestations include hypertension, angina pectoris, transient cerebral ischemic attacks, intermittent claudication, upper gastrointestinal bleeding, retinal angioid streaks and thickened skin. A characteristic appearance of highly reflective foci in the renal parenchyma have been reported in patients with PXE, but it's not specific for this syndrome. However, the presence of this structural pattern in a young patient with dermatological abnormalities should lead to the consideration of PXE in the differential diagnosis list. Besides ultrasonography findings, only a ureteral disruption case in a patient underwent ureteroscopic manipulation has been described to our knowledge. In our case, the true significance of these disease in recurrent urinary tract infection is uncertain.