Poikiloderma vasculare atrophicans is a cutaneous reaction pattern characterised by mottled hyper- and hypomelanosis, telangiectasia and progressive dermal and epidermal atrophy. It may manifest as a component of established mycosis fungoides but may precede the development of the latter by many years and, in some cases, may persist indefinitely without progression to frank lymphoma. It should be distinguished from other causes of poikiloderma such as may be seen with dermatomyositis.