Monoclonal gammopathy of thrombotic significance

A 64-year-old man presented with monoclonal gammopathy of thrombotic significance (MGTS): progressive painful purpuric plaques, progressive thrombocytopenia and acral necrosis. Recognition of MGTS by dermatologists as part of the differential diagnosis of microvascular occlusion syndromes is crucial to guide patient care. Management should focus on early recognition of the underlying haematological neoplasm, surveillance of thrombotic complications and initiation of cytoreductive therapy.