No side-effects were observed. patient remains in remission on monthly maintenance intravenous immunoglobulins, 2 years after initial treatment. Keywords:High-dose intravenous immunoglobulin, modified Rodnan score, scleromyxoedema == Introduction == Scleromyxoedema is a rare disease involving dermal deposition of mucin and fibroblast proliferation; it is characterized clinically by a diffuse papular eruption, skin thickening, oedema and decreased skin flexibility, especially of the face and hands, with a progressive and disabling disease course.14Patients with scleromyxoedema can also have systemic manifestations.5Many treatments have been proposed Baclofen for scleromyxoedema, including corticosteroids, retinoids, cyclophosphamide, plasmapheresis, bortezomib, melphalan, thalidomide, chloroquine, extracorporeal photopheresis and photochemotherapy such as psoralen plus ultraviolet A radiation Baclofen therapy (P-UVA), cyclosporine and autologous stem-cell transplantation. High doses of intravenous immunoglobulins have been proposed as a therapy, based on multiple case reports that support the efficacy and safety of Rabbit Polyclonal to OR2AG1/2 such treatment.57 == Case report == A 64-year-old man presented to Baclofen the Department of Dermatology, University-Hospital of Modena and Reggio Emilia, Modena, Italy, in November 2012 with scleromyxoedema, with severe involvement of the skin of the face, arms and hands, decreased mouth opening and hypomotility of the fingers (Figure 1). Dysphagia, asthenia and monoclonal gammopathy of immunoglobulin G lambda-type were also present. == Figure 1. == (a) Left hand of a patient with scleromyxoedema before treatment, showing oedema, stiffness, erythema and ulceration. (b) The same hand after 3 months high-dose intravenous immunoglobulin therapy, showing resolution of the oedema, erythema and ulceration, good mobility and visible skin folds and wrinkles. The patient had previously been treated with topical and systemic corticosteroids, P-UVA therapy, plasmapheresis, extracorporeal photochemotherapy, hydroxychloroquine and cyclophosphamide, without any benefit. Disease severity was assessed using a modified Rodnan score, which evaluates skin thickness and papular involvement.8,9Skin thickness is rated by clinical palpation using a 03 point scale (0, normal skin; 1, mild thickness; 2, moderate thickness; 3, severe thickness with inability to pinch the skin into a fold) for 17 surface anatomical areas: face, anterior chest; abdomen; fingers; forearms; upper arms; thighs; lower legs; dorsum of hands and feet bilaterally. Papular involvement is rated according to the presence of papules (0, no papules; 1, presence of papules) in each of same 17 anatomical areas. The patients total score was 53 out of a maximum score of 182*. Treatment with intravenous immunoglobulins at a dosage of 2 g/kg administrated over 5 days once a month was started. After the first infusion cycle, a substantial improvement was observed. After seven cycles of therapy, recovery of skin elasticity, an increase in facial mimic movement and restoration of joint function was observed (Figure 2). == Figure 2. == Face of a patient with scleromyxoedema: (a) before treatment, showing severe erythema, papules and plaques, especially on the glabella, thinning of the eyebrows and reduced mobility; (b) after 3 months high-dose intravenous immunoglobulin therapy, showing improvement in erythema and oedema and improved mobility; (c) after 5 months high-dose intravenous immunoglobulin therapy, showing further improvements in erythema and mobility, with disappearance of the papules and plaques on the glabella. The modified Rodnan score dropped from 53 to 31. No side-effects were observed. After 2 years, the patient remains in clinical remission on maintenance therapy with intravenous immunoglobulins Baclofen (2 g/kg once per month). The patient gave consent for their Baclofen case to be published. == Discussion == Scleromyxoedema is a progressive disease with a disabling and unpredictable course; in a few cases it is fatal due to heart or kidney failure.4,10The aetiology of scleromyxoedema is still unknown. Therapeutic management is based on data from reported cases. P-UVA therapy, hydroxychloroquine and cyclophosphamide pulsed therapy were all unsuccessful in the patient presented here. A considerable improvement was obtained with intravenous immunoglobulin 2 g/kg, administered over 5 days once a month. The use of intravenous immunoglobulins as a treatment for scleromyxoedema has been well documented.5,11,12The action of intravenous immunoglobulins is not yet completely understood, but they seem to act through an immunomodulatory mechanism blocking Fc receptors on splenic macrophages, inhibiting complement, modulating cytokines, neutralizing autoantibodies, blocking CD95 and inhibiting apoptosis.6,13,14Side-effects are rare and normally mild.15,16Some patients require maintenance intravenous immunoglobulin therapy; others need only a few infusions to obtain permanent remission.5,15 In the patient presented here, the efficacy of intravenous immunoglobulin therapy was demonstrated in a reduction in the modified Rodnan score. The.
