ReviewDiagnosis and classification of eosinophilic fasciitis☆
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].
References (25)
- et al.
Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases
Semin Arthritis Rheum
(1988) - et al.
Eosinophilic fasciitis
Semin Arthritis Rheum
(1980) - et al.
Eosinophilic fasciitis and eosinophilic cellulitis in a patient with abnormal circulating clonal T cells: increased production of interleukin 5 and inhibition by interferon alfa
J Am Acad Dermatol
(2003) - et al.
Eosinophilic fasciitis: analysis of a series of 10 patients
Med Clin (Barc)
(2005) - et al.
Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma
Am J Med
(1990) Diffuse fasciitis with eosinophilia: a new syndrome?
Trans Assoc Am Physicians
(1975)- et al.
Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients
Rheumatology (Oxford)
(2012) - et al.
Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome
Clin Rheumatol
(2007) - et al.
Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature
Int J Dermatol
(2008) - et al.
Eosinophilic fasciitis (Shulman disease)
Best Pract Res Clin Rheumatol
(2012)
Eosinophilic fasciitis in siblings
J Rheumatol
Serum levels of tissue inhibitor of metalloproteinase-1 and 2 in patients with eosinophilic fasciitis
Br J Dermatol
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.