TY - JOUR AU - Gharib, B. AU - Ghareh Zadeh Shirazi, A. AU - Moradi, E. AU - Yaghmaei, B. AU - Ziaee, V. PY - 2019/04/01 Y2 - 2024/03/29 TI - A rare disease with a rare presentation: hemi-atrophy caused by fibromuscular dysplasia in a 27 month old girl JF - Reumatismo JA - Reumatismo VL - 71 IS - 1 SE - Case Reports DO - 10.4081/reumatismo.2019.990 UR - https://www.reumatismo.org/reuma/article/view/990 SP - 31-36 AB - <p>We discuss the case of a 27-month-old girl afflicted with fibromuscular dysplasia. She presented with hemiatrophy of left upper and lower limbs, nail dystrophy, ulcers on the tips of her toes, cold and painful limbs, foot drop, and hypertension. The initial appearance started at 2 months of age and other diagnoses such as <em>complex regional pain syndrome, reflex sympathetic syndrome, vasculitis </em>and<em> coagulation disorders</em> had been considered. Angiography revealed that all the arterial branches of the left lower and upper limbs, from brachial to ulnar and radial, and from iliac and femoral to tibialis arteries were affected. Sural nerve biopsy confirmed the diagnosis. In the follow-up visits until 2 years after the patient’s discharge she did not develop any new problem and her blood pressure was controlled by enalapril and amlodipine.</p> ER -