Eosinophilic fasciitis 30 years after - What do we really know? : Report of 11 patients and review of the literature

• Published in: Dermatology (Basel) Vol. 213; no. 2; pp. 93 - 101
• Main Authors: ANTIC, Milos, LAUTENSCHLAGER, Stephan, ITIN, Peter H
• Format: Journal Article
• Language: English
• Published: Basel Karger 01.08.2006; S. Karger AG
• Subjects: Adult; Aged; Bacterial diseases; Biological and medical sciences; Biopsy; Borrelia infections; Dermatology; Diagnosis, Differential; Drug therapy; Eosinophilia - complications; Eosinophilia - drug therapy; Eosinophilia - pathology; Fascia - pathology; Fasciitis - complications; Fasciitis - drug therapy; Fasciitis - pathology; Female; Follow-Up Studies; Glucocorticoids - therapeutic use; Human bacterial diseases; Humans; Infant; Infectious diseases; Leukocytes; Male; Medical diagnosis; Medical sciences; Middle Aged; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Skin diseases; Steroids; Tropical bacterial diseases; Human; Immunopathology; Connective tissue disease; Skin disease; Dermatology; Borrelia infection; Autoimmune disease; Borreliosis; Review; Eosinophilic fasciitis; Infection; Case study; Spirochaetosis; Systemic disease; Bacteriosis; Eosinophilic fasciitis, pathomechanism; Scleroderma; Shulman syndrome
• ISSN: 1018-8665; 1421-9832
• DOI: 10.1159/000093847

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. 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. 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. 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.