Scleromyxedema is characterized by a generalized papular and sclerodermatous eruption, monoclonal gammopathy in the absence of thyroid disease, and evidence of mucin deposition, fibrosis, and particularly skin involvement.Scleromyxedema is similar to scleroderma, but has predominantly mucin deposition and is usually associated with a serum IgG monoclonal paraprotein. High-dose chemotherapy with stem cell rescue is feasible for patients with scleromyxedema and, although not curative, offers durable remission in most patients. The available evidence is that of case reports and series, which is understandable given the rarity of the disease.
There are no guidelines, consensus sttements or review articles that recommend this therapy. However, the provided plan language does not contain anything that excludes this procedure. It is apparently effective for pallation but not cure based on the current literature, albeit this literature is limited.It is not in trials and could never be.
However, this condition can be seen as a form of scleroderma. Stem cell transplantation in the hope of modifying disease activity in scleroderma is under active clinical investigationl.As previously pointed out, the low ejection fraction makes this procedure riskier.
Adrienne M. Feasel, MD; Michele L. Donato, MD; Madeleine Duvic, MDComplete Remission of Scleromyxedema Following Autologous Stem Cell Transplantation Arch Dermatol. 2001;137:1071-1072.
C . Cokonis Georgakis , G . Falasca , A . Georgakis , W . Heymann Scleromyxedema .
Clinics in Dermatology , Volume 24 , Issue 6 , Pages 493 – 497 2006
Lacy MQ, Hogan WJ, Gertz MA, Dispenzieri A, Rajkumar SV, Hayman S, Kumar S, Litzow MR, Schroeter AL.Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation.Arch Dermatol. 2005 Oct;141(10):1277-82
Complete Remission of Scleromyxedema Following Autologous Stem Cell Transplantation Am Acad Dermatol. 2004 Jul;51(1):126-31 Adrienne M. Feasel, MD; Michele L. Donato, MD; Madeleine Duvic, MD
van Laar JM, Farge D, Tyndall A. Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial: hope on the horizon for patients with severe systemic sclerosis. Ann Rheum Dis 2005; 64:1515.
van Laar JM, Tyndall A. Adult stem cells in the treatment of autoimmune diseases. Rheumatology (Oxford) 2006; 45:1187-1193.
Alan Tyndall; Daniel E. Furst Adult Stem Cell Treatment of Scleroderma Curr Opin Rheumatol. 2007;19(6):604-610