Successful treatment of scleromyxedema with plasmapheresis and immunosuppression

Successful Treatment of Scleromyxedema With Autologous Peripheral Blood Stem Cell Transplantation

“…The fact that many patients have a poor response is reflected in the large number of treatments that have been tried. These include chemotherapeutic agents, such as the alkylators, 3,5,6,27 methotrexate, 7 cladribine, 8 glucocorticoids, 9 isotretinoin, 28,29 interferon alfa, 15 dimethyl sulfoxide, 30 extracorporeal photochemotherapy, 12 plasmapheresis, 31,32 thalidomide, 10 intravenous immunoglobulin, 11,33 and psoralen-UV-A. 13 In 1969, Feldman et al 5 described a dramatic response to melphalan therapy, and a 1979 report on a series of 8 patients confirmed that melphalan therapy could be effective in some patients (4 patients had an excellent response).…”

Papulöse Muzinose - Erfolgreiche Therapie mit Plasmapherese

“…Hier beruht der Effekt auf der Entfernung zirkulierender Antikörper oder Immunkomplexe [11]. Im Falle der papulösen Muzinose und seiner Sonderform des Skleromyxödems werden die offensichtlich ätiologisch bedeutsamen Serumfakatoren sowie, falls vorhanden, Paraproteine mittels der Plasmapherese entfernt und somit die Produktion und Ablagerung bestimmter, saurer Mucopolysaccharide reduziert [1,7].…”

Scleromyxedema, a Positive Effect with Thalidomide

“…Corticosteroids[7,8,9], PUVA [10], electron beam therapy [11], retinoids[ 12, 13], plasmapheresis[ 14], high-dose intravenous immunoglobulin[ 15], extracorporeal photochemotherapy[ 16], dermabrasion[ 17] and topical dimethyl sulfoxide [18] have all produced some symptomatic improvement; however, none of them have yielded long-term beneficial results. Several chemotherapeutic and modulatory agents such as mephalan[ 2, 18, 19], cyclosporine[ 20, 21], 2-chlorodeoxyadenosine[ 22], cyclophosphamide[ 23, 24], methotrexate[ 25, 26], chlorambucil[ 27] and a-interferon [28] have also been used in an attempt to interfere with the plasma cell dyscrasia.…”