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Dermatomyositis Complicated by Pneumomediastinum and Subcutaneous Emphysema

Dermatomyositis Complicated by Pneumomediastinum and Subcutaneous Emphysema. Andrew Knerl, MD, (Associate) Cuong Doan, MD, (Member) Exempla Saint Joseph Hospital Denver, Colorado. Case Presentation. 29-year-old female 6 month history of dermatomyositis Heliotrope rash and biopsy

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Dermatomyositis Complicated by Pneumomediastinum and Subcutaneous Emphysema

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  1. Dermatomyositis Complicated by Pneumomediastinum and Subcutaneous Emphysema Andrew Knerl, MD, (Associate) Cuong Doan, MD, (Member) Exempla Saint Joseph Hospital Denver, Colorado

  2. Case Presentation • 29-year-old female • 6 month history of dermatomyositis • Heliotrope rash and biopsy • Gottron’s patches • Later developed muscle weakness and vasculitic lesions • 1 month history of interstitial lung disease (ILD) • Based on CT scan from hospitalization for presumed PNA • Bronchoscopy with biopsies negative for PCP • Colonoscopy, EGD, CT abd/pelvis without evidence of cancer

  3. Case Presentation • Treatment with prednisone, azathioprine, hydroxychloroquine, mycophenolate mofetil, and nifedipine • On admission: mycophenolate mofetil, hydroxychloroquine, prednisone, and nifedipine • Admitted to the hospital for neck pain and swelling • No difficulty breathing or swallowing

  4. Exam findings • Crepitance over neck, chest, back and upper arms • Vasculitic lesions on hands and fingers • Ulcerations on fingertips • Labs unremarkable except for an elevated ESR and CPK

  5. Radiology findings • Neck soft tissue x-ray: large subcutaneous air • CXR: subcutaneous emphysema • CT Neck: air tracking into the neck, the base of the skull, and the retropharyngeal space • CT Chest: pneumomediastinum extending into the neck, back, axilla, and spinal column • Etiology inapparent • Thin air around lungs, likely extrapleural • Contrast esophagram: no perforation

  6. Hospital Course • Pulse dose steroids (1 g IV x 3 days) followed by prednisone 60 mg daily • Mycophenolate mofetil and hydroxychloroquine as WBC tolerated • No source of pneumomediastinum or subcutaneous air identified • Subcutaneous emphysema improved and the patient was discharged home to follow up with rheumatology and pulmonology • Meds: mycophenolate mofetil and prednisone

  7. Dermatomyositis • Inflammatory myopathy characterized by a skin eruption that usually precedes muscle weakness

  8. Dermatomyositis • Heliotrope rash on the upper eyelids with edema may be present • Gottron’s sign—an erythematous, raised, scaly eruption involving the knuckles, knees, and elbows

  9. Dermatomyositis • Muscle weakness is a progressive, symmetric process that initially affects the proximal muscles • Pharyngeal muscles may be involved, resulting in dysphagia, and, in advanced cases, respiratory muscles may also be affected

  10. Dermatomyositis and Malignancy • Increased incidence of ovarian ca, breast ca, melanoma, colon cancer, and NHL.

  11. Dermatomyositis • Interstitial lung disease may precede the myopathy or occur early in the disease, and develops in 5-30% of patients • Pneumothorax, pneumomediastinum, and subcutaneous emphysema are rare complications of dermatomyositis-related ILD and carry a poor prognosis • A literature search by Kono et al in 2000 reported 13 cases of pneumomediastinum in the setting of dermatomyositis

  12. Hypotheses • Rupture of subpleural blebs and dissection around perivascular sheaths • Vasculopathy causing subpleural infarctions and interstitial emphysema • Since pneumomediastinum is more common in patients with ulcerative or vasculitic lesions of the skin

  13. Treatment Options • Limited evidence due to small number of reported cases • As an outpatient, was switched from mycophenolate mofetil to cyclophosphamide • Improvement to date with recent CXR showing resolved pneumomediastinum • Back at work and in the gym • Currently tapering prednisone and azathioprine • Vasculitic lesions and ulcerations have resolved

  14. References • FaizFaiz, Saadia A. et al. “Dermatomyositis With Pneumomediastinum and Rapidly Progressing Interstitial Lung Disease.” Chest (2006). • Kono, Hajime et al. “Pneumomediastinum in Dermatomyositis: Association With Cutaneous Vasculopathy.” Annals of the Rheumatic Diseases 2000; 59:372-376. • Powell, Christian et al. “A 34-Year-Old Man With Amyopathic Dermatomyositis and Rapidly Progressive Dyspnea With Facial Swelling.” Chest 2007; 132:1710-1713.

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