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

Carolin Kurz,Christian Andres,Claudia Traidl-Hoffmann,Benedikt J Schwaiger,Derek Spieler,Silke Wunderlich,Rüdiger Ilg

doi:10.1186/1756-0500-7-580

Carolin Kurz, Christian Andres + Show 5 more

Open Access

https://doi.org/10.1186/1756-0500-7-580

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Abstract

BackgroundClinical 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 presentationHere 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.ConclusionSevere 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.

Highlights

Clinical complications of Sjoegren’s syndrome include myelitis and skin manifestations
Intensive immunosuppressant treatment resulted in significant improvement of both symptom clusters
We report a patient with biopsy-proven s syndrome (SS) who presented with an acute rash, acute transverse myelopathy and coincident cranial neuropathy

Summary

Conclusion

We report a patient with biopsy-proven SS who presented with an acute rash, acute transverse myelopathy and coincident cranial neuropathy. Of note recent findings demonstrate a T cell epitope mimicry between Sjögren’s syndrome Antigen A (SSA)/Ro60 and skin bacteria [14]. Author details 1Department of Neurology, Klinikum rechts der Isar, Technische Universität, Ismaninger Strasse 22, Munich 81675, Germany. Szymula A, Rosenthal J, Szczerba BM, Bagavant H, Fu SM, Deshmukh US: T cell epitope mimicry between Sjogren’s syndrome Antigen A (SSA)/Ro60 and oral, gut, skin and vaginal bacteria. Doi:10.1186/1756-0500-7-580 Cite this article as: Kurz et al.: Acute transverse myelitis and psoriasiform dermatitis associated with Sjoegren’s syndrome: a case report.

Background

Findings