TY  - JOUR
TI  - Complete and sustained resolution of calcinosis universalis in a juvenile dermatomyositis case with mycophenolate mofetil
AB  - Juvenile dermatomyositis (JDM) is a rare, multisystemic, idiopathicvasculopathy mainly affecting the muscles and the skin. Gastrointestinalsystem, lungs, joints and heart may also be involved. Characteristic skin findingsare heliotrope rash and Gottron papules but extensive skin involvement aslarge necrotic lesions are rarely reported. Calcinosis is one of the major issuesin the long term. Delay in diagnosis, inadequate therapy at the initial phase,prolonged persistent disease activity are considered as major risk factors forthe development of calcinosis. Treatment of calcinosis is also a major issuebecause no single treatment modality has been found to reproducibly stop orreverse calcification.A 5-year-old girl was admitted to our clinic with typical signs and symptomsof JDM. She was initially treated with high-dose corticosteroids, methotrexateand intravenous immunoglobulin (IVIG). Soon after, she developed necroticulcerative skin lesions and cyclosporine was added to her treatment regimen.By this treatment all muscle and skin manifestations were controlled but onthe first year of follow-up she developed superficial calcification plaques onthe upper extremities and calcinosis universalis like calcifications on the lowerextremities. Calcifications did not respond to bisphosphonate (pamidronate)and IVIG treatment but mycophenolate mofetil resulted in rapid and sustainedresolution of all calcification plaques.
AU  - KARADAĞ, Şerife Gül
AU  - AYAZ, Nuray Aktay
AU  - Çakan, Mustafa
DO  - 10.24953/turkjped.2019.05.018
PY  - 2019
JO  - Turkish Journal of Pediatrics
VL  - 61
IS  - 5
SN  - 0041-4301
SP  - 771
EP  - 775
DB  - TRDizin
UR  - http://search/yayin/detay/353375
ER  -