2082 val lipodermoid, is reported. The skin biopsy of the cervical nevus at birth found sebaceous nevi, which confirmed the diagnosis of epidermal nevus syndrome. High blood pressure (140/70 mmHg) was incidentally discovered at 3 years of age, at the time of surgery for cervical nevi excision. Clinical examination revealed a systolic cardiac ejection murmur of 3/6 at the right upper sternal edge without radiation, weak bilateral femoral pulses, and asymmetrical arterial blood pressure between upper and lower limbs. The neurological examination did not show any deficits or musculoskeletal anomalies. Echocardiography examination revealed a mild coarcta- tion of the aorta at the isthmus with a nadir pressure gradient of 50 mmHg, so that antihypertensive drug therapy (beta-blocker + ACE inhibitor) was initiated. One year later, the echocardiography was repeated because of persistent elevated blood pressure, but no significant change was noted. Magnetic resonance angiography (MRA) showed dilated ascending aorta (20 mm in diameter) with a hypoplastic transverse arch (7 mm) and descending aorta (10 mm). Surgical repair of the aortic coarctation was carried out at 5 years of age by modified Crafoord technique. Post-surgical echocardiography showed im- proved blood flow velocity (3 m/s) and maximal pressure gradient (36 mmHg) at the aortic isthmus. However, the treatment with beta-blocker and ACE inhibitor could not be discontinued because of ongoing systemic hypertension (140/80 mmHg). Six months later, the patient was referred to our department for further investigation of persistent arterial hypertension (135/82 mmHg). There was no clinical evidence of post- surgical recurrent stenosis of the aorta (symmetrical blood pressure in both upper and lower limbs). There was no sign of impairment of renal function and serum creatinine was within the normal range for age. Renal Doppler ultrasound revealed normal kidney size with normal differentiation, an accelerated blood flow velocity at the right renal artery ostium and proximal left renal artery (2 m/s), at the ostium of the superior mesenteric artery (SMA; 3 m/s) and as well as the coeliac trunk (4–5 m/s). These findings were confirmed by MRA, which showed multiple stenoses at different levels of the aorta without signs of wall thickness or inflammation (Figs. 1, 2, 3), but with stenosis at the ostium of the left subclavian artery (5.7 mm, then 10 mm), narrowing of the descending thoracic aorta at the isthmus (8 mm, then 10 mm), proximal stenosis of the coeliac trunk, SMA, right and left renal arteries. Treatment with beta blocker and ACE inhibitor was continued with a progressive dose increase and close observation of renal function until acceptable control of blood pressure was achieved (120/70 mmHg). At last follow-up, i.e., at 10 years of age, the boy had no overt organ damage with normal growth (height 25th to 50th C B Pediatr Nephrol (2011) 26:2081–2084 A Fig. 1 Left lateral oblique thoracoabdominal aorta–iliac MR angiogram, maximum intensity projection image showing: Arrow A left subclavian artery ostial stenosis, Arrow B the surgical repair site of the coarctation of the thoracic aorta, and Arrow C very mild mid-abdominal aorta narrowing at the level of the coeliac trunk, superior mesenteric artery (SMA), and renal arteries centile, body weight 75th to 97th centile), no remarkable developmental delay, and normal renal function (serum creatinine 38 μmol/l, estimated GFR 130 ml/min/1.73 m2). Since blood pressure control was currently acceptable under treatment with beta-blocker and ACE inhibitor (120/67 mmHg), surgery had been postponed under close surveillance. This strategy was decided according to a surgical team, a radiology team, a nephrology team, and parental input. Discussion Epidermal nevi are circumscribed congenital lesions in the arrangement or quantitative composition of otherwise