A 61-year-old man presented with a characteristic periorbital purplish discoloration (Heliotrope rash). It was associated with malar erythema and edema, less striking erythema of the neck and presternal area (Shawl sign) and atrophic papules over the knuckles of the fingers (Gottron’s papules). The skin signs had appeared simultaneously with muscle symptoms (weakness of proximal muscles). No underlying tumour was found. Investigations revealed: Negative ANA , Elevated CPK and abnormal EMG.