RT Book, Section
A1 Kim, Susan
A2 Zaoutis, Lisa B.
A2 Chiang, Vincent W.
SR Print(0)
ID 1146122423
T1 Juvenile Dermatomyositis
T2 Comprehensive Pediatric Hospital Medicine, 2e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071829281
LK accesspediatrics.mhmedical.com/content.aspx?aid=1146122423
RD 2024/04/19
AB Juvenile  dermatomyositis  (JDM)  is  the  most  common  pediatric  acquired  inflammatory  myopathy.  This  diffuse  vasculopathy  primarily  involves  inflammation  in  skin  and  striated  muscle,  which  often  leads  to  significant  weakness  and  physical  limitations.  However,  despite  its  nomenclature  JDM  is  a  multisystem  disease.  Characteristic  cutaneous  features,  which  classically  include  heliotrope  rash  and  Gottron  papules,  aid  in  the  diagnosis.  The  etiology  of  this  condition  is  unknown,  and  has  an  estimated  incidence  of  1  to  3  in  1  million  children,1,2  with  approximately  a  2:1  female  predominance.  Affected  children  are  typically  between  4  and  10  years  of  age  with  an  average  age  of  onset  of  7  years.  Outcomes  in  JDM  may  vary,  but  historically  there  was  significant  morbidity;  without  adequate  treatment,  one-third  of  affected  persons  will  have  moderate  to  severe  disability.  Mortality  attributable  to  the  disease  occurs  in  one-third  of  children.3,4