Abstract
A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute amyopathic dermatomyositis. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).
Highlights
A patient presenting with isolated periorbital and facial edema may pose diagnostic challenges
We present a case where skin and muscle biopsy established the previously unsuspected diagnosis of DM in a patient presenting acutely with periorbital and facial edema without myopathy
There have been no previous reports of isolated facial edema in the setting of acute amyopathic dermatomyositis
Summary
A patient presenting with isolated periorbital and facial edema may pose diagnostic challenges. The differential diagnosis includes allergic reactions, soft tissue infections, intracranial venous thrombosis, auto-immune (dermatomyositis (DM), lupus erythematosus) and auto-inflammatory (periodic syndrome) disorders, and trichinosis In this setting, it may be difficult to make an accurate clinical diagnosis, and the patient is often treated empirically, pending the results of specific laboratory investigations. Case report A 45-year-old Asian man, unskilled worker, who had lived in Greece for the last three years, presented to the Emergency Department with a three day history of painful facial swelling, pyrexia and vomiting. His medical history was notable only for type 2 diabetes mellitus treated with metformin and gliclazide.
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