Anasarca: not a nephrotic syndrome but dermatomyositis

Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract, and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of JDM, generalized edema has...

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Bibliographic Details
Published in:European journal of pediatrics 2008-07, Vol.167 (7), p.831-834
Main Authors: Zedan, Magdy, El-Ayouty, Moustafa, Abdel-Hady, Hesham, Shouman, Basma, El-Assmy, Mohamed, Fouda, Ashraf
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child, Preschool
Dermatomyositis - diagnosis
Dermatomyositis - physiopathology
Diagnosis, Differential
Electromyography
General aspects
Glomerulonephritis
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Medicine
Medicine & Public Health
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
Nephrotic Syndrome - diagnosis
Pediatrics
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Short Report
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Summary:Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract, and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of JDM, generalized edema has rarely been reported. Here, we report a 3.5-year-old boy with JDM presenting with generalized edema. The diagnostic criteria of JDM rely on typical clinical manifestations that include: severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum skeletal muscle enzymes, and myopathic electromyographic pattern. Our patient initially received methylprednisolone and intravenous immunoglobulin (IVIG) without significant improvement, so he was given azathioprine and a prolonged course of oral prednisolone. We conclude that JDM should be suspected in patients presenting with anasarca in the absence of laboratory parameters of other causes of generalized edema and an appearance of heliotrope rash with muscle weakness. Also, we suggest that muscle magnetic resonance imaging (MRI) should be considered among the diagnostic tools of JDM.
ISSN:0340-6199
1432-1076
DOI:10.1007/s00431-008-0716-z