Schwerer Verlauf einer mutilierenden pansklerotischen zirkumskripten Sklerodermie im Kindesalter

Abstract

Disabling pansclerotic morphea of childhood is the most severe variant of localized scleroderma. It is characterized by rapid progression of deep cutaneous fibrosis expanding over large areas of body surface. The prognosis in terms of normal life activity is poor and the disease may even take a fatal course. Presented is a case with extremely severe and rapidly progressive lesions resulting in cutaneous ulcerations joint contractures, and multilaing deformities of the extremities. Histopathological analysis revealed extensive intravascular calcinosis of the small vessels, which may be an important factor in the pathogenesis of this poorly understood disease. UVA1-phototherapy was performed and induced a softening of sclerosis and a distinct decrease of skin thickness. Based on these observations UVA1-phototherapy may be promising in the treatment of extensive sclerotic disease of this kind, and possibly other diseases accompanied by excessive sclerosis.

S1‐Leitlinie zur UV‐Phototherapie und Photochemotherapie

“…Auch die Necrobiosis lipoidica kann auf eine UV‐A1‐Phototherapie ansprechen . Kasuistisch wird über die erfolgreiche Behandlung einer pansklerotischen Morphae der Kindheit berichtet.…”

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

“…A relapse-free follow-up period of 12 months or even longer has been reported for morphea [12,13,14]. We observed long-term remission after conclusion of the treatment in 4 of our 12 morphea patients followed for 2, 10, and 14 years.…”

“…Except of one case report [11], morphea patients were treated only for 1-4 months [11,12,13,14]. On the other hand, in published studies, morphea patients received cyclosporine at a higher dose (3-5 mg/kg) compared to our patients (mean 2.40 mg/kg).…”

“…Creatinine elevation was found only in 1 of 12 patients. According to the literature, cyclosporine is well tolerated by patients with morphea, although it should be noted that the evidence level is based only on case reports [11,12,13,14,15]. …”

“…A PubMed search from 2015 revealed only five case reports of 1 or 2 patients [11,12,13,14,15]. With the exception of one published case [13], cyclosporine exerted a beneficial effect in all other patients. The use of cyclosporine is well established in stem cell transplanted patients, and approved by the EMA [16], but not by the FDA [17], for the prevention and treatment of graft-versus-host disease.…”