Acute inflammatory myopathy with severe subcutaneous edema, a new variant?Report of two cases and review of the literature

Acute inflammatory myopathy with severe subcutaneous edema is extremely rar e and has been reported in only a handful of cases. We describe two similar patients presenting with this disorder and generalized rash. Unlike the fi ve previously reported cases, the clinical and histologic features of our t wo patients are ore suggestive of dermatomyositis than polymyositis. Nevert heless, scrutinizing all seven reported patients, a umber of specific chara cteristics could be defined. All patients were adult males. Dysphagia was p resent in four. In six patients, acute inflammatory myopathy was idiopathic while malignancy was present in one. Two patients died despite intensive t herapy, three improved on corticosteroid treatment, and two recovered spont aneously. In all patients, limb involvement with marked subcutaneous edema was present, clinically mimicking deep vein thrombosis in both our patients . The presence of severe subcutaneous edema may be a hallmark of a distinct ive Variant of acute inflammatory myopathy. More cases are needed to discer n subtypes of this general entity and to establish guidelines for treatment and prognosis.