PAPULAR MUCINOSIS - SUCCESSFUL THERAPY WITH PLASMAPHERESIS

A 23 year old female patient presented with a 14 year history of cutan eous mucinosis. Her hands showed indurated edema with acrocyanosis and severe reduction in motility, while her face was red, edematous and r evealed numerous small angiomas. In the sacral region, she had a large elevated skin colored plaque. On the basis of clinical findings, hist ology and further laboratory tests the disease was classified as papul ar mucinosis (lichen myxoedematosus). In view of the severe suffering of the young patient and an unsuccessful previous therapy with chloroq uine,we decided to employ plasmapheresis as a single therapy. After th e first treatment course there was an improvement in her skin conditio n. After a total of four plasmapheresis courses over a period of 18 mo nths (total exchange volume of 38,4 I) there was an objective flatteni ng of the plaques and reduction of the finger swelling. This case demo nstrates that monotherapy of plasmapheresis is an effective made of tr eatment for cutaneous mucinosis, a disease which is extremely difficul t to treat.