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

Vol. 71 No. 1 (2019)
Submitted: 29 March 2017
Accepted: 28 October 2018
Published: 1 April 2019
Fibromuscular Dysplasia, Hemiatrophy, Childhood, Hypertension.
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Authors

B. Gharib
Children’s Medical Center, Pediatrics Center of Excellence, Tehran; Growth and Development research Center, Tehran University of Medical Sciences, Iran, Islamic Republic of.
A. Ghareh Zadeh Shirazi
Children’s Medical Center, Pediatrics Center of Excellence, Tehran, Iran, Islamic Republic of.
E. Moradi
Shiraz University of Medical Sciences, Shiraz, Iran, Islamic Republic of.
B. Yaghmaei
Children’s Medical Center, Pediatrics Center of Excellence, Tehran; Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran, Islamic Republic of.
V. Ziaee
ziaee@tums.ac.ir
http://orcid.org/0000-0003-4648-3573
Children’s Medical Center, Pediatrics Center of Excellence, Tehran; Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran; Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran, Islamic Republic of.

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.

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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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