Juvenile DermatomyositisKing Hussein Medical Center Experience | ||
| The Egyptian Journal of Hospital Medicine | ||
| Article 12, Volume 55, Issue 1, April 2014, Pages 228-238 PDF (447.55 K) | ||
| Document Type: Original Article | ||
| DOI: 10.12816/0004508 | ||
| Author | ||
| Zeyad Habahbeh* | ||
| Pediatric Allergy, Immunology, and Rheumatology division, Queen Rania Hospital for Children, King Hussein Medical Center, Amman Jordan | ||
| Abstract | ||
| Background: Juvenile dermatomyositis (JDM) is an uncommon, often chronic, and potentially serious childhood systemic autoimmune vasculopathy affecting primarily skin and muscles. It is characterized by pathognomonic rash, and symmetrical proximal muscle weakness. Objective: In this retrospective study, we reviewed the clinical, laboratory profiles, treatment and outcome of Jordanian children diagnosed with JDM in the past 8 years in a tertiary facility in Amman, Jordan. Methods: Sixteen (16) JDM patients, diagnosed based on criteria of Bohan and Peter, and have attended the pediatric 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 and electrophysiological data were studied as well. Treatment and outcome were also reviewed. Results: Our cohort includes 16 patients, 9(56 %) males, and 7 (44%) females (M: F 1.3:1), their age ranges between 2 to 9 years, with average age at diagnosis of 5.4 years. Time to diagnosis varies from 2 months to 12 months, and averages at 4.6 months. Proximal muscle weakness was present at time of diagnosis in 14(87.5 %) cases. Cutaneous signs in form of either poikiloderma in malar distribution, Gottron’s sign and /or heliotrope sign were apparent in all the 16(100%) patients at time of diagnosis, periungual erythema was evident in 10(63%) patients while abnormal nailbed capillaries pattern was only reported in 6(38%) cases. Skin and soft tissue calcification, crusting and ulceration were seen in 3(19 %) patients. Serum Lactate dehydrogenase, (LDH) was elevated in 94% and creatininine phosphokinase (CPK) in 88%. Elevated SGOT (AST) was seen in almost all subjects, while ESR was high in 14 (87.5%) patients. All patients treated with corticosteroids and methotrexate (MTX). Two (12.5%) patients died in our series, complete remission was achieved in 4(25%) of patients, while partial remission was seen in 8 (50%) of patients in our cohort. Conclusion: JDM is a rare disease that has the potential to cause physical disability, poor functional outcome, and death if not recognized early and treated properly. We focused in our study, on importance of early referral, and aggressive therapy in improving outcome, aiming to increase awareness of families and general pediatricians. | ||
| Keywords | ||
| Juvenile dermatomyositis; Myopathy; Calcinosis; Heliotrope sign | ||
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