Scleroderma encompasses a group of disorders that can be broadly divided in to 3 groups: systemic sclerosis, localized scleroderma, and sclerodermatous disorders (including conditions such as scleredema diabeticorum and scleromyxoedema). There are different clinical presentations of localized scleroderma, including plaque morphea, linear morphea, generalized morphea, and eosinophilic fasciitis. Treatment with localized scleroderma has been tried with topical and systemic corticosteroids, calcitriol, phototherapy, and systemic agents such as cyclosporine, D-penicillamine, or methotrexate, but there are no large series or trials with any of these agents.

In a recent report published in the British Journal of Dermatology, a group of investigators from the Netherlands describe their experience with the use of methotrexate (used in combination with systemic corticosteroids in some patients) in a large series of patients with localized scleroderma or sclerodermatous disorders. There were 27 patients with generalized morphea, 13 with linear morphea, 9 with plaque morphea, 5 with eosinophilic fasciitis, 2 with scleredema diabeticorum, and 2 with scleromyxoedema. Patients were treated methotrexate 15 mg per week, and if there was a partial or no response it was increased to 25 mg per week. In some patients with more acute disease, systemic corticosteroids were added to this regimen. There were 47 patients treated with methotrexate and 81% showed improvement. There were also 11 patients treated with methotrexate in combination with systemic corticosteroids, and 100% of these showed improvement. Many patients reached a clinical remission, and relapses were mainly seen in patients who had received less cumulative doses of methotrexate. It was only effective in one out of four patients with scleredema diabeticorum or scleromyxoedema Side effects were frequent (around 50% of patients) but treatment had to be discontinued only in 6, and in 4 of these it was restarted without recurrence of side-effects.

Although this study is not a randomized prospective study the results of this large series suggest that methotrexate could be of of the first-line therapies in localized scleroderma as it seems to be very effective and the side-effects are mild.

Effectiveness, side-effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: an inception cohort study
Kroft EBM, et al.

Br J Dermatol 2009; 160:1075–1082.

Summary. Background Detailed information is lacking on effectiveness of methotrexate (MTX) in sclerotic skin diseases, side-effects, and duration of remission after discontinuation.
Objectives To determine effectiveness, side-effects and period of remission gained by use of MTX in sclerotic skin diseases.
Methods All patients with a sclerotic skin disease who were treated with MTX (group A) or MTX with corticosteroids (CS) (group B) between 1995 and 2007 were evaluated. Detailed information was collected on dosage and duration of MTX treatment, concomitant immunosuppressive medication and CS treatment, effectiveness, side-effects, duration of the remission period, and time until restart.
Results Fifty-eight patients (A, n = 47; B, n = 11) were evaluated. Clinical assessment revealed that 38 patients (81%) treated with MTX and 11 patients (100%) treated with MTX + CS showed improvement of sclerotic skin. After one treatment course 51% of the patients treated with MTX and 73% treated with MTX + CS reached remission status with a median follow-up time of 55 and 58 months. Patients showing relapse still responded to a second and even to a third course of MTX. Patients who showed a relapse had received a lower cumulative dose, due to a shorter period of treatment with MTX in the first course. Serious side-effects were seen in six patients (10%).
Conclusions MTX was an effective treatment for various sclerotic skin diseases with a long period of remission and relatively low toxicity. Patients showing relapse still responded to a second and third course of MTX.

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