Juvenile dermatomyositis king hussein medical center experience

ABSTRACT

Juvenile dermatomyositis [JDM] is an uncommon, often chronic, and potentially serious childhoodsystemic autoimmune vasculopathy affecting primarily skin and muscles. It is characterized bypathognomonic rash, and symmetrical proximal muscle weakness.In this retrospective study, we reviewed the clinical, laboratory profiles, treatment and outcome ofJordanian children diagnosed with JDM in the past 8 years in a tertiary facility in Amman, Jordan.Sixteen [16] JDM patients, diagnosed based on criteria of Bohan and Peter, and have attended thepediatric rheumatology clinic in King Hussein Medical Center, from January 2006 to September 2013,were recruited. Their medical records were studied for clinical and biochemical profile, radiological andelectrophysiological data were studied as well. Treatment and outcome were also reviewed.Our cohort includes 16 patients, 9 [56%] males, and 7 [44%] females [M F 1.31], their age rangesbetween 2 to 9 years, with average age at diagnosis of 5.4 years. Time to diagnosis varies from 2 monthsto 12 months, and averages at 4.6 months. Proximal muscle weakness was present at time of diagnosis in14 [87.5%] cases. Cutaneous signs in form of either poikiloderma in malar distribution, Gottron's signand /or heliotrope sign were apparent in all the 16 [100%] patients at time of diagnosis, periungualerythema was evident in 10 [63%] patients while abnormal nailbed capillaries pattern was only reported in6 [3 8%] cases.Skin and soft tissue calcification, crusting and ulceration were seen in 3 [19%] patients. Serum Lactatedehydrogenase, [LDH] was elevated in 94% and creatininine phosphokinase [CPK] in 88%. ElevatedSGOT [AST] was seen in almost all subjects, while ESR was high in 14 [87.5%] patients. All patientstreated with corticosteroids and methotrexate [MTX].Two [12.5%] patients died in our series, complete remission was achieved in 4[25%] of patients, whilepartial remission was seen in 8 [50%] of patients in our cohort.JDM is a rare disease that has the potential to cause physical disability, poor functional outcome, anddeath if not recognized early and treated properly. We focused in our study, on importance of earlyreferral, and aggressive therapy in improving outcome, aiming to increase awareness of families andgeneral pediatricians