Cyclosporine Reduces Sclerosis in Morphea: A Retrospective Study in 12 Patients and a Literature Review

Abstract

Background: The treatment of severe morphea is challenging, and treatment experience concerning the use of immunosuppressive agents for this condition is limited. Objective: The purpose of this study is to analyze the use of cyclosporine, its tolerability, and its effect on skin sclerosis. Materials and Methods: Patients with severe morphea who underwent treatment with cyclosporine were studied retrospectively. Results: Five of 12 patients with morphea showed complete remission and 6 patients had partial remission at the end of therapy (9-46 months, median 14) under a median cyclosporine dose of 2.4 mg/kg. The mean affected body surface area fell from 50% (2-80, median 65) to 17% (0-40, median 18). Side effects were hypertension, elevated transaminases, cholesterol, and weight gain. Conclusion: Cyclosporine can be effective in morphea. The side effects were reversible. However, the duration of treatment with cyclosporine is limited because of its potential permanent side effects. Prospective placebo-controlled studies are needed to establish the superiority of cyclosporine over other immunosuppressive drugs in this setting.

Juvenile case of multiple morphea profunda resulting in joint contracture that was successfully treated with cyclosporin A: A case report and review of the published works

“…In a retrospective study of 12 patients with severe morphea who were treated with CyA, complete remission was seen in half of the cases and only one case had no response. 12 However, as that previous report had no morphea profunda cases, our report is the first one that demonstrates CyA efficacy for morphea profunda. Thus, it should be noted that CyA can be a treatment option to reduce oral steroid doses in juvenile cases with morphea profunda to avoid oral steroid inhibition of bone growth.…”

“…In addition, our case suggested that administration of CyA is also effective at resolving even subcutaneous nodules. In a retrospective study of 12 patients with severe morphea who were treated with CyA, complete remission was seen in half of the cases and only one case had no response . However, as that previous report had no morphea profunda cases, our report is the first one that demonstrates CyA efficacy for morphea profunda.…”

Development of keloidal morphea after treatment with cyclosporine in a case of recalcitrant generalized morphea

“…The authors stated that the side effects of cyclosporine were reversible. 9 Secondly, despite the skin induration had a favorable response to cyclosporine in our patient, she later developed keloid-like nodules. This finding may be due to changes in the microenvironment of skin with cyclosporine therapy that led to a fibrosing skin reaction.…”

“…7,8 Since the lesions can occur at high-risk sites (such as the trunk) for the development of keloid and hypertrophic scar formation in susceptible patients, a possible role of genetic predisposition can exist. [8][9][10][11][12] Some remarkable findings in this case are as follows: Firstly, our patient with generalized morphea was refractory to different treatments for progressive morphea, but her lesions had a favorable response to cyclosporine. In line with our findings, a retrospective study indicated that cyclosporine is efficient against severe morphea.…”

“…According to recent studies, cyclosporine can be effective in the management of hardto-treat cases of morphea. 9 Herein, we report a case of recalcitrant progressive generalized morphea that responded to treatment with cyclosporine but later complicated with nodular lesions.…”