Tumorous variant of scleromyxedema. Successful therapy with intravenous immunoglobulins

Abstract

We present an unusual tumorous variety of scleromyxedema mimicking facies leonina in lymphoma. In spite of pronounced and widespread cutaneous changes, hypergammaglobulinaemia and paraproteinaemia, the general condition of the patient was satisfactory, there was no internal involvement and no symptoms of any malignancy. Initially, melphalan and corticosteroids were applied but were not effective. High-dose intravenous immunoglobulin (IVIG) therapy had dramatic effect, and after five 5-day monthly courses the tumours almost regressed and the skin became less hard. After a further five courses in the following year there was complete clearance, which was sustained without any therapy for 1 year (until now). IVIG appears to be the therapy of choice for scleromyxedema. We stress, however, that at the start of therapy, IVIG applications should be supplemented with small doses of melphalan and/or corticosteroids.

Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin

“…5,[31][32][33] At the time of this writing, 24 cases were published showing clinical J AM ACAD DERMATOL VOLUME 60, NUMBER 6 improvement of scleromyxedema treated with IVIG. 4,5,8,9,11,15,31,[34][35][36][37][38] In the majority of studies, patients were treated with high-dose IVIG (2 g/kg/mo), although improvement has been reported with doses as low as 0.5g/kg/mo. 37 Furthermore, it appears that some patients require maintenance IVIG therapy to control symptoms whereas others only need a few infusions to achieve a sustained remission.…”

“…3 In severe cases, infiltration may produce leonine-like facies. 4 In addition to cutaneous findings, other organ systems may be affected in scleromyxedema. Musculoskeletal involvement is one of the most common extracutaneous manifestations with up to 90% of patients reporting arthralgia, myalgia, or muscle weakness, which may be associated with elevation of muscle enzymes and inflammatory electromyographic findings.…”

Scleromyxedema with Subcutaneous Nodules: Successful Treatment with Thalidomide and Intravenous Immunoglobulin

“…Monoclonal gammopathy has been found in 88–100% of patients and has usually been of IgG γ light chains [3, 7, 9, 12]. Paraproteinemia is generally present at the beginning of disease, though a delayed appearance should also be considered [1, 6, 13]. Other reported associations include biclonal gammopathy [3, 12, 14].…”

Evidence for the Use of Intravenous Immunoglobulins—A Review of the Literature

“…Case report [322] Marked clinical improvement, maintained with repeated IVIg infusions A 56-year-old woman with scleromyxedema IVIg (Sandoglobulin®) 2 g/kg over 5 days as monotherapy Case report [323] Complete clearance of skin lesions, sustained effect after 1 year without treatment Case report [324] Clinic Rev Allerg Immunol (2010) 38: Kaposi's sarcoma…”