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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Ann+Indian+Acad+Neurol
2018 ; 21
(1
): 70-74
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A Viral Polymyositis Masquerade: Life-Threatening Case of Juvenile
Dermatomyositis Complicated by Systemic Capillary Leak Syndrome
#MMPMID29720802
Shelley BP
; Chakraborti S
Ann Indian Acad Neurol
2018[Jan]; 21
(1
): 70-74
PMID29720802
show ga
This is a case report of an 8-year-old boy who developed an atypical, rare
subphenotype of autoimmune inflammatory acute juvenile dermatomyositis (JDM),
initially masquerading as viral polymyositis (PM)-like presentation, that was
complicated by a hitherto unreported fulminant, life-threatening pediatric
systemic capillary leak syndrome (SCLS). We highlight the close differential
between viral PM and JDM, the baffling clinical syndromic constellation of
hypotension with hemoconcentration - a "shock"-like syndrome, hypoalbuminemia
without albuminuria, and generalized edema with the atypical JDM presentation,
and stress crucial need to implement early aggressive, multipronged
immunomodulatory treatment along with intensive fluid resuscitation which saved
the life, this patient from a stormy, and turbulent 4-week clinical illness. This
is the first published case description in the current literature of the
association of an aggressive subphenotype of JDM and life-threatening pediatric
SCLS. This report opens the Pandora's Box to explore the genetic and
pathomechanisms of both disorders.