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Vol. 213, No. 2, 2006
Issue release date: August 2006
Section title: Clinical and Laboratory Investigations
Dermatology 2006;213:93–101
(DOI:10.1159/000093847)

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

Report of 11 Patients and Review of the Literature

Antic M.a · Lautenschlager S.b · Itin P.H.c
aDepartment of Internal Medicine, General Hospital Waid, and bOutpatient Clinic of Dermatology, General Hospital Triemli, Zurich; cDepartment of Dermatology, University Hospital Basel, Basel, and Kantonsspital Aarau, Aarau, Switzerland

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Article / Publication Details

First-Page Preview
Abstract of Clinical and Laboratory Investigations

Received: 7/22/2005
Accepted: 2/13/2006
Published online: 8/28/2006
Issue release date: August 2006

Number of Print Pages: 9
Number of Figures: 5
Number of Tables: 2

ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)

For additional information: http://www.karger.com/DRM

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.

© 2006 S. Karger AG, Basel


  

Author Contacts

Peter H. Itin
Department of Dermatology, University of Basel
Petersgraben 4
CH–4031 Basel (Switzerland)
Tel. +41 61 265 4086, Fax +41 61 265 4200, E-Mail peter.itin@unibas.ch

  

Article Information

Received: July 22, 2005
Accepted: February 16, 2006
Number of Print Pages : 9
Number of Figures : 5, Number of Tables : 2, Number of References : 66

  

Publication Details

Dermatology

Vol. 213, No. 2, Year 2006 (Cover Date: August 2006)

Journal Editor: Saurat, J.-H. (Geneva)
ISSN: 1018–8665 (print), 1421–9832 (Online)

For additional information: http://www.karger.com/DRM


Article / Publication Details

First-Page Preview
Abstract of Clinical and Laboratory Investigations

Received: 7/22/2005
Accepted: 2/13/2006
Published online: 8/28/2006
Issue release date: August 2006

Number of Print Pages: 9
Number of Figures: 5
Number of Tables: 2

ISSN: 1018-8665 (Print)
eISSN: 1421-9832 (Online)

For additional information: http://www.karger.com/DRM


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