Elsevier

Autoimmunity Reviews

Volume 13, Issues 4–5, April–May 2014, Pages 379-382
Autoimmunity Reviews

Review
Diagnosis and classification of eosinophilic fasciitis

https://doi.org/10.1016/j.autrev.2014.01.019Get rights and content

Abstract

Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome with an unknown etiology and pathogenesis that should be considered an immune-allergic disorder. Painful swelling with progressive induration and thickening of the skin and soft tissues of the limbs and trunk are the clinical hallmarks of the disease. Peripheral blood eosinophilia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate are the main laboratory findings. Full-thickness wedge biopsy of the clinically affected skin showing inflammation and thickening of deep fascia is essential to establish the diagnosis. The differential diagnosis includes systemic sclerosis and other scleroderma subsets such as morphea, and epidemic fasciitis syndromes caused by toxic agents such as the myalgia–eosinophilia syndrome and toxic oil syndrome. Peripheral T cell lymphomas should also be ruled out. The diagnosis of EF can be established by clinical, laboratory and histological findings, but universally accepted international diagnostic criteria are lacking. Corticosteroids are efficacious and remain the standard therapy for EF, although some patients may improve spontaneously.

Introduction

Eosinophilic fasciitis (EF) is an uncommon scleroderma-like disorder first described by Shulman [1] in 1974 and characterized by induration of the skin, peripheral eosinophilia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate (ESR). The disease is present almost equally in both sexes [2], [3], [4], in sporadic rather than in epidemic form. The mean age at onset has consistently been reported as between 40 and 50 years, with a wide range from childhood to advanced age [5]. It remains unclear whether race and family history are risk factors for developing eosinophilic fasciitis; anecdotal evidence suggests familial aggregation with a predominance of HLA-A2 [5], [6], [7].

Section snippets

Etiology and pathogenesis

The etiology of EF remains uncertain. Hematological, infectious, and autoimmune diseases, as well as intense physical exertion, chemical compounds, drugs, solid neoplasms, and physical factors have been proposed as possible triggers and associated factors [2], [5], [6] (Table 1). Some authors have proposed an aberrant immune response as the main pathogenetic mechanism, a notion supported by findings of hypergammaglobulinemia in peripheral blood and IgG and C3 deposition in fascia of some

Clinical manifestations

EF is characterized by abrupt onset of painful and erythematous swelling of the affected extremities. Although the condition is mainly symmetrical, unilateral disease also occurs [13]. Involvement of the extremities is the rule, but any other skin areas can be affected [2], [5], [6]. Trunk involvement is a recognized risk factor for refractory fibrosis [4]. Edema is progressively replaced by thickening of the skin, which is firmly bound to the underlying tissue. Peau d'orange appearance (Fig. 1

Additional examinations

The following complementary tests can be of help in the diagnosis of EF.

Pathological features

A full-thickness wedge biopsy of the affected skin usually reveals characteristic findings. The muscle fascia shows accumulation of lymphocytes, mainly CD8 + lymphocytes (CD4/CD8 ratio < 1) [11], macrophages, and plasma cells. Eosinophils or major basic eosinophilic proteins are not always present in affected tissues [24]. The fascia is usually 2 to 15 times thicker than normal and firmly adherent to the subjacent skeletal muscle, whereas the dermis and epidermis are usually normal. Nevertheless,

Diagnosis and differential diagnosis

Universally accepted diagnostic criteria in patients with EF are lacking. Most physicians consider that the diagnosis of EF can be established when characteristic skin lesions are present, after excluding the various subsets of scleroderma, toxic oil syndrome, silica exposure, gadolinium administration in patients with renal failure, and l-tryptophan-induced eosinophilia–myalgia epidemic syndrome [4], [24], [25]. It is our opinion that some of the features related to EF are more important than

Treatment

Some EF patients improve spontaneously without treatment, but in those who do not, glucocorticoids (0.5–1 mg/kg/d) are the mainstay therapy. Clinical improvement can take weeks or months and is heralded by resolution of peripheral blood eosinophilia. Methotrexate at low doses (15–25 mg once weekly) is probably the most favored second-line treatment, especially in patients with morphea-like skin lesions [2], [3].

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  • Cited by (0)

    Funding: This study was funded in part by a grant (FIS/2012 PI12-01320) from the Spanish Ministry of Health and Consumer Affairs.

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