Because of the COVID-19 outbreak, you are now doing telemedicine only. Your first patient is a 55-year-old man whose main complaint is " I'm having trouble swallowing my food". He denies odonophagia, gastroesophageal reflux symptoms, emesis, or nausea, but states that he has lost 5 pounds of weight and has resorted to eating liquid foods. He also notes the recent onset of a non-pruritic rash and some "puffiness" of his eyes. He is on no medications, and his past medical history is notable only for a 20-pack year of tobacco use. Below is your view of the patient.
What is your diagnosis, what test(s) would you order to confirm your suspicions, and what treatment, if any, do you recommend?
DIAGNOSIS: Dermatomyositis (DM).
The telemedicine view of the patient shows periorbital edema and a typical heliotrope-colored rash on the face and trunk; his sad appearance is the result of facial muscle weakness. Other physical findings in DM may include Gottron's nodules (on the knuckles), proximal muscle weakness, periungual telangiectasias, a scaly alopecia, and interstitial fibrosis (diffuse mid-to-end inspiratory crackles). Dysphagia for both solids and liquids may be the presenting complaint and is the result of weakness of the muscles of deglutition; it may be accompanied by dysphonia. Patients with DM may also have arthralgias or frank arthritis.
The diagnosis is confirmed by obtaining myositis-specific antibodies, an EMG, muscle enzymes (CPK) and an esophagoscopy. Magnetic resonance tomography may show muscle edema. Muscle biopsies show capillary deposition of 5b-9 complement components (membrane attack complex), perifascicular inflammatory infiltrates (predominantly CD4+ T cells, B cells, and plasmacytoid denditic cells), endothelial edema, and perifascicular atrophy.
This patient's heavy history of tobacco use emphasizes the need to assess adult patients with DM for possible underlying malignancies. The frequency of malignancy in DM varies greatly (3-40%) with the more accurate figure being ∼12% with longterm followup; CT scans are reported as being the most effective means of making the diagnosis.
DM may be amyopathic, can occur in juveniles, and is one of five inflammatory myopathies. Prenisone ± a corticoid-sparing agent is the mainstay treatment. Cutaneous disease is difficult to manage, and may require the use of antimalarials, methotrexate, and/or intravenous immunoglobulin.