Eosinophilic Fasciitis 30 Years after – What Do We Really Know?

Abstract

Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.

From Furuncle to Axillary Web Syndrome: Shedding Light on Histopathology and Pathogenesis

“…Eosinophilic fasciitis or Shulman syndrome is another rare entity usually presenting with bilateral swelling of the extremities, followed by peau d’orange changes and finally woody induration, thickening and tightness of the skin [9,10]. Retraction of the superficial veins within the indurated skin results in linear depressions described as the ‘groove sign’.…”

Espessamento da pele: desafio diagnóstico

“…A maioria dos pacientes se encontra entre a terceira e sexta década de vida, mas o acometimento da população pediátrica também tem sido relatado. 2 A etiologia da FE é desconhecida. Alguns estudos sugerem que doenças auto-imunes, hematológicas, infecciosas, fármacos, traumatismos, esforço físico intenso ou picadas de inseto podem ser fatores associados e desencadeadores da doença, 3 mas não foi identificado nenhum destes fatores no presente caso.…”

‘Venous Furrowing’: A Clue to the Diagnosis of Eosinophilic Fasciitis

“…simvastatin [7] , atorvastatin [8] , lansoprazol [9] , phenytoin [10] ), myeloproliferative illnesses [11] , thyroid diseases [12] , extreme physical activity, bone marrow transplantation [13] , haemodialysis [14] and arthropod bites have all been described as causative agents.…”