Emergency Rheumatology: Where not all multifocal opacities are COVID
CASE
In her first visit, a 43-year-old female with no significant past medical history presents to the emergency department and is found to have lymphadenopathy. Three weeks prior to presentation, she began to notice swelling to her eyelids, worse when she woke up in the morning. One week prior to presentation, she noticed swelling to her right posterior neck and left anterior neck, as well as pain in her jaw when she chewed. The patient denied fevers, chills, congestion, sore throat, trouble breathing or swallowing, or cough. Her exam revealed very faint upper eyelid swelling and right suboccipital and left anterior cervical lymphadenopathy. Laboratory testing, including a CBC and BMP were normal, hCG was not consistent with pregnancy and HIV was nonreactive. The patient was discharged from the ED with PCP follow-up.
On her second visit, one month later, the patient represented to the emergency department with new symptoms, including worsening neck and shoulder pain, worsening facial swelling and erythematous patches to the skin overlying the MCP, PIP and DIP joints. Labs were repeated, revealing a mild transaminitis, a CK and inflammatory markers that were within normal reference ranges. The chest X-ray was normal as well.
The ED team was concerned for dermatomyositis. The differential for cervical lymphadenopathy and rash includes a wide variety of infections, malignancies and rheumatologic disorders, but the pattern of the rash was, to the team, highly suggestive of the Gottron papules seen in dermatomyositis. The patient was begun on a short steroid burst, and referred to rheumatology and dermatology. At her follow-up telehealth visit with dermatology, two weeks later, the patient was prescribed hydroxychloroquine and continued on high-dose prednisone. The diagnosis remained uncertain, particularly given her normal inflammatory markers and CK.
The patient returned to the ED one month later with worsening myalgias, as well as dyspnea, cough and diarrhea. The patient was found to be hypoxic, requiring 6 liters nasal cannula, as well as tachycardic and febrile to 101 degrees Fahrenheit. Her chest X-ray showed multifocal opacities and prominent interstitial markings; her COVID-19 PCR test was negative. Her other labs were notable for a mild elevation of CRP, to twice the upper limit of normal. CK remained within normal limits.
DIAGNOSIS
Amyopathic dermatomyositis
DISCUSSION
The multiple hyperkeratotic papules with central atrophy present over the patient’s MCP and IP joints were what are known as Gottron papules, (still!) named after German dermatologist and vocal Nazi supporter Heinrich Adolf Gottron. These are highly specific for dermatomyositis.
Dermatomyositis is a relatively rare disorder. It more often affects women than men, and the peak age of incidence is in the sixth decade of life. Dermatomyositis can be divided into two entities. “Classic” dermatomyositis involves both cutaneous findings (Gottron papules, heliotrope eruption, photo-distributed erythema, poikiloderma) as well as myopathy (proximal muscle weakness and elevated CK). Ten to 30 percent of dermatomyositis is clinically amyopathic dermatomyositis (CADM), which involves cutaneous findings without clinical evidence of muscle weakness, and patients with this form often have normal CK levels. The important thing to remember is that both of these entities can progress to interstitial lung disease. This is what happened in this patient’s case.
CASE RESOLUTION
After a biopsy and send-out testing, the patient was eventually diagnosed with anti-MDA-5 dermatomyositis, a subtype of the disease with both mucocutaneous and systemic features, the most ominous of which is rapidly progressive interstitial lung disease. The clinical manifestations of this disease are cutaneous ulcers, alopecia, digit hyperkeratosis, palmar papules, necrotic fingertips, oral ulcer and panniculitis. Treatment options for this genre of dermatomyositis include mycophenolate mofetil, IVIG, rituximab and cyclophosphamide. Unfortunately, in this patient’s case, despite aggressive treatment, the ILD progressed to respiratory failure and ARDS. The patient was placed onto an ECMO circuit, but after progressive multiorgan failure, she was transitioned to comfort measures, and passed away.
KEY TAKE-AWAYS
Gottron papules are a poorly named but highly specific sign of dermatomyositis, even in the absence of myopathy
Maintain a broad differential for imaging findings “consistent with COVID-19”
Referrals for outpatient specialty follow-up can be key in ensuring timely diagnoses
Categories:
dermatomyositis; rheumatology; dermatology; interstitial lung disease
AUTHOR: Luke Messac, MD, PhD is a PGY-4 resident in emergency medicine at Brown University.
FACULTY REVIEWER: Aluisio, Adam MD, MSc
REFERENCES
Kurtzman, Drew, Ruth Ann Vleugels. Anti melanoma differentiation-associated genome (MDA5) dermatomyositis: a concise review with an emphasis on distinctive clinical features. Journal of the American Academy of Dermatology 78; 4(2018): 776-785
Ricceri, Federica, Francesca Prignano. Gottron papules: a pathognomonic sign of dermatomyositis. CMAJ 185;2 (2013): 148.
Weyers, Wolfgang. Death of medicine in Nazi Germany: dermatology and dermatopathology under the Swastika. Madison Books, 1998.
Wikimedia Commons. “Dermatomyositis.”