Combination Oral Prednisone and Intravenous Immunoglobulin in the Treatment of Scleromyxedema

Abstract

IVIg is being used for a growing number of inflammatory and immune disorders. It is being increasingly reported as a successful treatment for scleromyxedema. Although our patient succumbed to the disease, combination therapy with prednisone and IVIg provided temporary symptomatic, laboratory, and clinical improvement of the condition. Optimization of this therapeutic strategy is thus indicated for the management of scleromyxedema.

Arndt–Gottron scleromyxoedema: successful therapy with intravenous immunoglobulins

“…In our extensively pretreated patient the IVIg therapy induced a sustained improvement of scleromyxoedema. Our experience with IVIg treatment of scleromyxoedema together with all the other published reports 2,4–10 suggest, that IVIg is an excellent therapeutic option for scleromyxoedema through its high effectiveness and excellent safety profile.…”

“…Several authors describe successful treatment of scleromyxoedema with IVIg 2,4–10 . In our extensively pretreated patient the IVIg therapy induced a sustained improvement of scleromyxoedema.…”

Scleromyxedema without paraproteinemia

“…These include a generalized papular and sclerodermoid rash, mucin deposits, fibroblast proliferation and fibrosis, monoclonal gammopathy, and the absence of thyroid disease . The pathogenesis of this disease remains uncertain; however, the high prevalence of monoclonal gammopathy, predominantly of immunoglobulin G (IgG), which presents in up to 80% of patients, suggests a possible immune response of B cells to “antigenic” mucin deposits in the dermis . Although a slight plasmacytosis has been detected in bone marrow, monoclonal gammopathy progresses to multiple myeloma in only 10% of cases …”

“…8 The pathogenesis of this disease remains uncertain; however, the high prevalence of monoclonal gammopathy, predominantly of immunoglobulin G (IgG), which presents in up to 80% of patients, suggests a possible immune response of B cells to antigenic mucin deposits in the dermis. 3,5,[9][10][11][12] Although a slight plasmacytosis has been detected in bone marrow, monoclonal gammopathy progresses to multiple myeloma in only 10% of cases. 3 Many treatment options have been described in various case reports but have shown limited efficacy in the management of these patients.…”

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

“…Case report [319] Improvement after the first treatment, continuous skin softening and reduction of induration, sustained response to treatment after 20 courses A 48-year-old female with long-standing scleromyxedema unresponsive to highdose CS and immunosuppressive (CP, melphalan) treatment IVIg 2 g/kg in 2 days (Intratect®; Biotest, Dreieich, Germany) every 4 weeks, adjunctive to PD 10 mg/day Case report [320] Progressive clinical improvement over several months allowing discontinuation of PD but rapid deterioration afterwards with lethal CVA despite reinstating PD and CP An 81-year-old female with scleromyxedema and IDDM IVIg (Gamimune N, 10%, Bayer) 1.5 g/ kg (dose reduced due to CHF) every 4 weeks and oral PD 30 mg/day Case report [321] A positive response after 2 cycles An 82-year-old female with a 12-year history of scleromyxedema unresponsive to various preceding therapies Venimmun® N (ZLB Behring, Germany) 0.5 g/kg for 5 days at 4-week intervals as monotherapy…”