vasculare as a pointer to reticulosis of the skin

AN important location of reticulo-endothelial tissue is the dermis of the skin (Robb-Smith, 1944). As in other sites, this tissue can undergo proliferation, ultimately to give a lymphoma, the clinical presentation being termed mycosis fungoides by dermatologists. Evolution is slow, 20 years or more usually elapsing before frank malignancy occurs. At this stage the fungating skin tumours which Alibert first described in the early 19th century may appear. Poikiloderma atrophicans vasculare of the skin, defined as pigmentation, telangiectasia, light scaling and epidermal atrophy giving so-called cigarettepaper skin, was reported by Lane in 1923 to precede Hodgkin's disease. Another case mentioned by him in 1921, also with poikiloderma, died eventually from lymphosarcoma. Increasing evidence would suggest that while poikiloderma may be found in one or two inherited disorders, in dermatomyositis and as a consequence of radiotherapy or of actinic damage, its main importance is as an indicator of present or future disorder of the reticulo-endothelial system of the skin. Samman (1964) found that of twenty-five cases with poikiloderma, twelve were showing progression towards a reticulosis. In 1968, he reported that fourteen of the twenty-five were showing such progress while of thirty-five additional cases eighteen were following a similar course towards reticulosis. We wish to report a case where poikiloderma atrophicans vasculare was present for 40 years, to be followed by mycosis fungoides and terminal reticulum cell sarcoma.