Dermatomyositis associated with bronchiolitis obliterans organizing pneumonia (BOOP).

Bronchiolitis obliterans organizing pneumonia (BOOP) is rarely associated with dermatomyositis and may be resistant to conventional corticosteroid therapy under this circumstance. We present a case of BOOP associated with dermatomyositis that responded to a combination of cyclophosphamide and corticosteroid therapy after corticosteroid treatments, alone, had failed. We believe this case shows it is important to recognize that facial rash in the presence of respiratory distress may represent dermatomyositis with BOOP and aggressive treatment may be necessary for resolution of pulmonary symptoms.