PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV

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