Abstract Details

Title A Mixed Picture: Dermatomyositis with features of Necrotizing Myopathy

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-010

Objective NA

Background Dermatomyositis is an autoimmune inflammatory myopathy that affects women more than men. It can occur in childhood or adulthood acutely or insidiously. It is characterized by a rash that precedes or accompanies proximal muscle weakness. Patients have an increased risk of cancer. Serum creatine kinase is elevated in 70% of cases. Muscle biopsy typically shows perimysial and perivascular inflammation. Patients respond well to immunosuppressive therapy.

Design/Methods We present a 60 year old woman with past medical history of hypothyroidism, with a chief complaint of 2 week history of bilateral upper and lower proximal extremity myalgias and muscle weakness. Her symptoms were preceded by a 1 week history of intermittent facial flushing and rash involving the cheeks and chest area. On exam, her shoulder and pelvic girdles were tender to palpation. She could not lift her arms above her head and had difficulty standing from a sitting position.

Results Labs were significant for high erythrocyte sedimentation rate, elevated creatine kinase, elevated aldolase and abnormal liver function tests. Her presentation was suspicious for dermatomyositis and she was empirically started on high dose steroids. By third day of steroid therapy her symptoms significantly improved and her rash resolved. SLE, PMR, RA, sarcoidosis, HIV and autoimmune hepatitis were excluded by lab work. Serologic testing revealed autoantibodies against Mi-2 which are highly specific for dermatomyositis. Muscle biopsy however revealed findings that were more consistent with necrotizing autoimmune myopathy.

Conclusions It is of particular interest in this case that the preponderance of evidence supports a diagnosis of dermatomyositis, however the histopathology suggests otherwise. This may represent an exception or particularly necrotizing subset of dermatomyositis.

Authors/Disclosures
Aqsa Ullah, DO (.) PRESENTER	No disclosure on file
Michelle S. Nunes, Sr., MD (Baptist Health Jacksonville)	Dr. Nunes has nothing to disclose.
Abdul R. Mamsa, MD	No disclosure on file
Enrique A. Serrano, MD (University of Miami Miller School of Medicine)	No disclosure on file

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