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

Abstract

Morphea profunda refers to inflammatory and sclerotic lesions that start primarily from the deep dermis, subcutaneous fat and fascia. Its pediatric case published work is limited. Here, we report the case of an 8‐year‐old girl with a 5‐year history of multiple subcutaneous nodules on her extremities and a right wrist joint contracture who had been previously diagnosed with juvenile idiopathic arthritis and treated with salazosulfapyridine, low‐dose prednisolone (PSL) and methotrexate. We performed biopsies of two subcutaneous nodules, which revealed the typical morphology of morphea profunda. She was administrated a tapered course of oral PSL then cyclosporin A (CyA) for 20 weeks which completely resolved her joint contracture and subcutaneous nodules. We reviewed 11 previously reported cases of morphea profunda and found that some include circumscribed/linear morphea that develop into subcutaneous tissues, indicating that “classical” morphea profunda arising within the deep tissues has rarely been reported. Our report is the first to demonstrate the efficacy of CyA for treatment of morphea profunda, and the possibility of CyA as a treatment option to reduce oral steroid doses in juvenile cases.

Pediatric‐onset solitary morphea profunda

“…To date, juvenile‐onset morphea profunda, which presents as linear or plaque type, has only rarely been reported 1,2 . No triggering events, such as vaccination and trauma, were observed in our patient in whom a plaque developed on the back.…”

Morphea: The 2023 update

“…Other agents including cyclosporine, hydroxychloroquine, azathioprine, retinoids, intravenous immunoglobulins, rituximab and infliximab, have all been shown effective in various case reports of severe morphea (151)(152)(153)(154)(155)(156)(157). However, their routine use is pending more definitive evidence of efficacy.…”

Treatment of juvenile localized scleroderma: current recommendations, response factors, and potential alternative treatments

“…Since 2019, here have been three reports on other synthetic molecules used to treat localized scleroderma (Table 2). A case report on cyclosporine A (CSA) included only one jLS patient, so findings from this and a 2016 study on 12 jLS and aLS patients [35,36] were collated. Patients were treated with CSA because of an inadequate response to prior treatment; more than 30% had pansclerotic morphea or generalized morphea.…”