Eosinophile Fasziitis (Shulman-Syndrom)

Abstract

Scleroderma like skin indurations without internal organ involvement, joint contractures, eosinophilia, and inflammatory infiltration of the fascia between subcutis and muscle are considered as characteristic features of eosinophilic fasciitis (EF). We report a further case of a 30 years old diabetic confirming to the syndrome defined by Shulman 1974. Corticosteroid therapy resulted in remission of disease symptoms, however a rebiopsy revealed no effect of therapy on histopathologic changes. A critical review of 118 cases in the literature is presented to explore the relationship of EF to scleroderma.

Hautmanifestationen rheumatischer Erkrankungen

“…Nur bei einem flüchtigen Blick kann eine eosinophile Fasziitis zu einer Verwechslung mit einer systemischen Sklerose führen (12). Dieses pseudosklerodermische Krankheitsbild, das nicht unbedingt den Kollagenosen zuzuordnen ist, manifestiert sich nicht an den Akren und ist nicht assoziiert mit einer Raynaud-Symptomatik.…”

Eosinophilic Fasciitis (Shulman Syndrome)

“…Raynaud’s phenomenon is normally not present [3], neither is organ involvement [4]. Associations with rheumatological and hematological illnesses are described [5]. The diagnosis is mainly based on the histology.…”