Essential thrombocytosis (ET) has rarely been reported with autoimmune rheumatic disorders. We report a case of young female, diagnosed case of Rheumatoid arthritis (RA), who had been overlooked for her raised platelet counts. Later her symptoms of impending digital gangrene led to an active search for her thrombocytosis. JAK2 mutation came out to be positive and she was diagnosed as ET associated with RA. She was treated with Hydroxyurea and Aspirin, in addition to her RA treatment. Patient responded well to the treatment and her platelet counts have been gradually improved, however, she developed gangrene of toe, for which amputation of distal phalanx of toe and nail excision was done, later in the disease course.
Keywords: Erythromelalgia; Essential thrombocytosis; Raynaud’s phenomenon; Rheumatoid arthritis.