A 49-year-old female with a past medical history of recurrent diverticulitis initially presented with one month of shortness of breath and a minor nonproductive cough for which she was started on doxycycline by her primary care provider. She then developed a rash on her chest, upper back, and face. Antibiotics were switched to amoxicillin and azithromycin. She underwent a brief admission of six days for shortness of breath but did not have an oxygen requirement at that time. She was evaluated by pulmonology (evaluated for cocci, unknown results), and then discharged. She then presented again to the ED with two weeks of worsening shortness of breath, intermittent fevers (Tmax 101°F), nausea/vomiting, fatigue, and arthralgias.

Vitals: BP 100/66; HR 128; Temp 37.2 °C (99 °F); Resp 44; SpO2 84%; BMI 28.25 kg/m2; Wt 79.4 kg (175 lb); Ht 1.676 m (5′ 6″)

General: NAD

Cardiovascular: Tachycardia, no m/r/g

Lungs: Coarse breath sounds at bases bilaterally, tachypneic

Abdomen: Soft, non-distended

Skin: Heliotrope rash to face (violaceous, erythematous rash to eyelids and nasolabial fold), shawl sign (erythematous patches to chest and upper back), shallow ulcers to tongue and lower inner lip, tender papules involving palms and lateral fingers bilaterally, and faint erythema of proximal nail fold

White blood cell (WBC) count: No leukocytosis

ESR: Elevated

LDH: Elevated

CK: Within normal limits

CXR: Bilateral infiltrates

CTPE: Negative for PE, but with scattered areas of ground glass and consolidative opacities throughout both lungs.

If emergency medicine physicians consider MDA5 Dermatomyositis (MDA5 DM) with rapidly progressive interstitial lung disease (RP-ILD) on their differential for patients presenting with skin and pulmonary symptoms, this can result in more rapid diagnosis and aggressive treatment.

This patient was admitted requiring 40 L HFNC, then two days later required intubation for severe ARDS and was placed on VV-ECMO the same day. Her hospital course was complicated by tachyarrhythmias requiring cardioversion, and Takostubo physiology. She was found to be MDA-5 antibody positive and ultimately expired while waiting for a lung transplant.

Take-Home Points

  • Critical actions in approaching ED patients with dermatological physical exam findings (even in the absence of known rheumatological history) with progressive pulmonary symptoms should include early consideration of dermatomyositis, serologic testing, early rheumatology and pulmonology consults, and early consideration of ECMO as a bridge to response to immunotherapy or lung transplant
  • Beginning these critical actions with first patient contact in the ED will only help improve patient outcomes throughout hospitalization.
  • Huang K, Levy RD, Avina-Zubieta JA. Successful lung transplant in rapid progressive interstitial lung disease associated with anti-melanoma differentiation associated gene 5. Rheumatology (Oxford). 2020 Aug 1;59(8):2161-2163. doi: 10.1093/rheumatology/keaa032. PMID: 32068868.
  • Koga T, Fujikawa K, Horai Y, Okada A, Kawashiri SY, Iwamoto N, Suzuki T, Nakashima Y, Tamai M, Arima K, Yamasaki S, Nakamura H, Origuchi T, Hamaguchi Y, Fujimoto M, Ishimatsu Y, Mukae H, Kuwana M, Kohno S, Eguchi K, Aoyagi K, Kawakami A. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology (Oxford). 2012 Jul;51(7):1278-84. doi: 10.1093/rheumatology/ker518. Epub 2012 Feb 29. PMID: 22378718.
  • Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis. Arthritis Care Res (Hoboken). 2016 May;68(5):689-94. doi: 10.1002/acr.22728. PMID: 26414240; PMCID: PMC4864500.

Paige Ruiz, MD

Paige Ruiz, MD

Emergency Medicine Resident
Department of Emergency Medicine
Stanford University

Alfredo Urdaneta

Clinical Assistant Professor
Stanford Medicine