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Successful treatment of scleromyxoedema with high dose intravenous immunoglobulin

We report a 53-year-old female patient with scleromyxoedema who has demonstrated and maintained a significant improvement in her condition when treated with high dose intravenous immunoglobulin (IVIG) therapy. She presented with a 1-year history of a progressive, infiltrating, papular eruption affecting her face, trunk and limbs which exhibited the Koëbner phenomenon and coalesced into indurated areas of sheeted erythema (see Fig. 1a). The presence of a monoclonal IgG paraprotein with lambda light chains in the blood and a skin biopsy which showed a marked proliferation of fibroblasts with prominent collagen and mucin deposition confirmed the diagnosis. Scleromyxoedema is a difficult condition to treat as all recommended treatment options have serious potential complications and relapse is frequent. The use of melphalan is restricted by toxicity, and cyclophosphamide, cyclosporin, photophoresis, PUVA and total skin electron beam therapy have all met with limited overall improvement. Interferon-a has resulted in deterioration in some cases, and plasma exchange is reserved for patients with central nervous system involvement. Systemic steroids have been used concurrently in many patients and their individual efficacy is difficult to determine. Autologous stem cell transplantation is described as an emerging therapy for this condition. IVIGs are used as treatment in a range of immunemediated diseases. Their efficacy has been shown in a variety of autoimmune medical and dermatological conditions, and they are a relatively safe, albeit expensive, treatment option. Our patient commenced IVIG as a firstline therapy. She received Vigam-S at a dose of 2 g ⁄ kg given as an infusion over a 5-day period on a monthly basis (0.4 g ⁄ kg ⁄ day) and noticed an improvement within 8 weeks. She has shown marked functional improvement with clinical resolution of the erythema and induration of her skin (Fig. 1b). She continues on 6-weekly IVIG infusions as maintenance therapy and her condition has currently been maintained in remission for 12 months. There are five case reports of scleromyxoedema responding successfully to IVIG. One patient showed an initial improvement in skin scores but subsequently relapsed with severe neuromuscular disease progression which failed to respond to ongoing maintenance therapy; another showed a dramatic improvement in skin scores and sustained his remission over a 10-month follow-up with ongoing 10-weekly IVIG infusions. The other three patients responded well to therapy, with one showing resolution of disease-associated encephalopathy; interestingly, while one of these patients required ongoing monthly treatment to maintain remission during a 2-year follow-up, the other two maintained remission with no ongoing therapy over a 3-year period, suggesting that maintenance therapy may not be necessary in a subset of patients. The mechanism behind the response of scleromyxoedema to IVIG is unclear. However, the ability of scleroderma and pretibial myxoedema to also respond favourably to IVIG suggests a common pathway, and there is some evidence suggesting that IVIG affects the process of matrix turnover and fibrosis through alterations in levels of matrix metalloproteinases. In summary, we feel that IVIG should be considered as both first-line treatment and maintenance therapy for scleromyxoedema. Its safety profile is favourable in Figure 1 Scleromyxoedema (a) before and (b) after treatment with high dose intravenous immunoglobulin Correspondence