PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV
PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV
PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV
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Poster Arzneimittel: Wirkung und Nebenwirkung<br />
P 11<br />
Treatment with Extracorporal Photochemotherapy in Patients Suffering from<br />
Pansclerotic Morphea: Report of Two Cases<br />
Sabine Moser-Oberthaler 1<br />
Laura Kuehnelt-Leddihn 1<br />
Gudrun Ratzinger 1<br />
Walter Nussbaumer 2<br />
Norbert Sepp 1<br />
1 Medical University Innsbruck, Department of Dermatology and Venerology,<br />
6020 Innsbruck<br />
2 Medical University Innsbruck, Department of Transfusion Medicine, 6020 Innsbruck<br />
Introduction: Morphea, the localized form of scleroderma, is an inflammatory<br />
autoimmune disorder that causes fibrotic changes in the skin. It is distinguished from<br />
generalized scleroderma by its lack of systemic involvement. Patients with morphea<br />
show single or multiple sclerotic or inflammatory plaques. Therapeutic options include<br />
observation, topical medication and phototherapy. Depending on disease activity,<br />
more aggressive systemic immunosuppressant therapies are required. Though the<br />
therapy with extracorporal photopheresis (a leukapheresis-based therapy combining<br />
UVA and 8-methoxypsoralen irradiation) has been reported in literature only in rare<br />
case series, it has been applied in this disease since 2009 at the University Hospital of<br />
Innsbruck, as when all other therapeutic options lack in efficacy. We report 2 cases of<br />
generalized deep pansclerotic morphea with good clinical response to treatment with<br />
extracorporal photopheresis.<br />
Design: At the University Hospital of Innsbruck, extracorporal photopheresis has been<br />
applied in this disease since 2009. Our aim was to objecitfy the results of this<br />
therapeutic option by using the modified Rodnan Skin Score. Skin thickening is<br />
assessed through palpation of the skin in 17 areas of the body using a 0-3 scale, where<br />
0 is normal, 1 is mild thickness, 2 is moderate thickness, and 3 is severe thickness. Total<br />
skin score can range from 0 (no thickening) to 51 (severe thickening in all 17 areas).<br />
Results: One female and one male patient (born 1943 and 1940) suffering from a<br />
histologically proven pansclerotic morphea, received regular photopheresis in addition<br />
to their basic therapy with low dose methylprednisolon. They were treated with a<br />
regimen of two consecutive treatments every four weeks - in total 56 and 34 times<br />
respectively. Before the first treatment and then every three months the modified<br />
Rodnan Skin Score was performed. The female patients initial score was 37, after 56<br />
treatments it decreased to 32; the male patient started with a score of 19 and after 34<br />
treatments it declined to 14. The improvement of the score was in both patients<br />
remarkable (13,51% versus 26,32%) without suffering from any side effects.<br />
52