Thrombocytopenia in the context of systemic sclerosis (SSc) is rare. It should primarily raise the possibility of scleroderma renal crisis. Immune thrombocytopenia (ITP) is another cause of low platelets that is common in systemic lupus erythematosus, but tremendously rare in patients with SSc. We herein report two cases of severe ITP in patients with SSc. The first case is a 29-year-old woman with very low platelet counts (2×10⁹/L) that did not increase despite receiving corticosteroids, intravenous immunoglobulins (IVig), rituximab and romiplostim. Because of a symptomatic acute subdural haematoma, emergency splenectomy was performed and subsequently platelet counts normalised without neurological sequelae. The second case is a 66-year-old woman in whom self-limited mild epistaxis revealed low platelet counts (8×10⁹/L). The patient did not improve after the use of IVig and corticosteroids. Secondarily rituximab and romiplostim normalised the platelet counts after 8 weeks. To the best of our knowledge this is the first reported case of severe ITP in a patient with diffuse cutaneous SSc and anti-topoisomerase antibodies.