Abstract
A 23-year old female patient presented with a 14 year history of cutaneous mucinosis. Her hands showed indurated edema with acrocyanosis and severe reduction in motility, while her face was red, edematous and revealed numerous small angiomas. In the sacral region, she had a large elevated skin colored plaque. On the basis of clinical findings, histology and further laboratory tests the disease was classified as papular mucinosis (lichen myxoedematosus). In view of the severe suffering of the young patient and an unsuccessful previous therapy with chloroquine, we decided to employ plasmapheresis as a single therapy. After the first treatment course there was an improvement in her skin condition. After a total of four plasmapheresis courses over a period of 18 months (total exchange volume of 38.4 l) there was an objective flattening of the plaques and reduction of the finger swelling. This case demonstrates that monotherapy of plasmapheresis is an effective mode of treatment for cutaneous mucinosis, a disease which is extremely difficult to treat.
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