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Kurz, C.* ; Wunderlich, S.* ; Spieler, D. ; Schwaiger, B.J.* ; Andres, C.* ; Traidl-Hoffmann, C. ; Ilg, R.*

Acute transverse myelitis and psoriasiform dermatitis associated with Sjoegren's syndrome: A case report.

BMC Res. Notes 7:580 (2014)
Verlagsversion DOI
Open Access Gold
Creative Commons Lizenzvertrag
BACKGROUND: Clinical complications of Sjoegren's syndrome include myelitis and skin manifestations. There is scarce observational data and a lack of randomised controlled studies regarding the treatment of Sjoegren's syndrome in the presence of such complications. CASE PRESENTATION: Here we report the case of a 41-year-old Caucasian female patient with biopsy-proven Sjoegren's syndrome who initially presented with generalized exanthema and subsequently developed acute extensive transverse myelitis. In view of the rapid deterioration we opted for an intensive treatment using a combination of corticosteroid pulse therapy, plasmapheresis and cyclophosphamide, which we later changed to rituximab. Under that treatment the skin manifestations resolved entirely whereas transverse myelitis showed incomplete remission. CONCLUSION: Severe neurological and dermatological complications may occur in Sjoegren's syndrome. This suggests a close yet currently unclear pathogenetic relationship. Intensive immunosuppressant treatment resulted in significant improvement of both symptom clusters. Skin manifestations may precede other severe complications in Sjoegren's syndrome and therefore require particular attention.
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Publikationstyp Artikel: Journalartikel
Dokumenttyp Wissenschaftlicher Artikel
Schlagwörter Cyclophosphamide ; Myelitis ; Psoriasiform Dermatitis ; Psoriasis ; Rituximab ; Sjoegren's Syndrome ; Sjögren's Syndrome
e-ISSN 1756-0500
Zeitschrift BMC Research Notes
Quellenangaben Band: 7, Heft: 1, Seiten: , Artikelnummer: 580 Supplement: ,
Verlag BioMed Central
Begutachtungsstatus Peer reviewed