Bingham A et al. - Certain Dermatomyositis (DM) pts with a severe, prolonged clinical course and a high frequency of calcinosis appear to be at greater risk for the development Lipodystrophy (LD). Further study is warranted to investigate the pathogenesis of acquired LD in pts with DM. Methods
This study describes the clinical features of 28 pts with juvenile dermatomyositis (JDM) and 1 patient with adult-onset DM
Results
All pts developed LD that could be categorized into generalized, partial, or focal, based on the pattern of fat loss distribution
LD onset was often delayed, beginning at 4.6 y after diagnosis of DM
Calcinosis, muscle atrophy, joint contractures, and facial rash were DM disease features found to be associated with LD
Panniculitis was associated with focal lipoatrophy while the anti-p155 autoantibody, was more associated with generalized LD
Specific LD features such as acanthosis nigricans, hirsutism, fat redistribution, and steatosis/nonalcoholic steatohepatitis were frequent in pts with LD
Insulin resistance and hypertriglyceridemia was recognized in pts with generalized and partial LD
Regional fat loss was more frequent in generalized than in partial LD and absent from DM pts w/o LD
Cytokine polymorphisms, the C3 nephritic factor, insulin receptor antibodies, and lamin mutations did not appear to play a pathogenic role in the development of LD in these pts
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