https://doi.org/10.4081/reumatismo.2019.990
A rare disease with a rare presentation: hemi-atrophy caused by fibromuscular dysplasia in a 27 month old girl
Submitted: 29 March 2017
Accepted: 28 October 2018
Published: 1 April 2019
Accepted: 28 October 2018
Abstract Views: 1192
PDF: 912
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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 complex regional pain syndrome, reflex sympathetic syndrome, vasculitis and coagulation disorders 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.
How to Cite
Gharib, B., Ghareh Zadeh Shirazi, A., Moradi, E., Yaghmaei, B., & Ziaee, V. (2019). A rare disease with a rare presentation: hemi-atrophy caused by fibromuscular dysplasia in a 27 month old girl. Reumatismo, 71(1), 31–36. https://doi.org/10.4081/reumatismo.2019.990
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