Eosinophilic fasciitis: Current concepts

Nicole Fett, Megan Arthur

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by abrupt onset of edema, followed by progressive induration of primarily the distal extremities. Patients may exhibit inflammatory arthritis, joint contractures, decreased mobility, and nerve entrapment. Almost half of patients with EF may have coexisting morphea plaques. Classic laboratory studies display peripheral eosinophilia, hypergammaglobulinemia, and elevated inflammatory markers. EF is included in the spectrum of scleroderma like disorders and may be difficult to distinguish from other sclerosing skin disorders. Full-thickness biopsy containing muscle and fascia is considered the gold standard for diagnosis and reveals sclerosis of the middeep dermis, subcutaneous fat, and thickening of the fascia. Magnetic resonance imaging (MRI) has been increasingly utilized to augment diagnostic capabilities. Ultimately, the diagnosis of EF relies upon the combination of characteristic clinical, laboratory, imaging, and histologic findings. Although some patients experience spontaneous remission, systemic corticosteroids (SCS) are the mainstay of treatment. Patients who fail to improve with SCS alone require the addition of a second immunosuppressive drug. Additionally, although data are limited, there is evidence to suggest that initial combination therapy with SCS and methotrexate (MTX) may be most beneficial.

Original languageEnglish (US)
Pages (from-to)487-497
Number of pages11
JournalClinics in Dermatology
Volume36
Issue number4
DOIs
StatePublished - Jul 1 2018

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Adrenal Cortex Hormones
Fascia
Nerve Compression Syndromes
Localized Scleroderma
Spontaneous Remission
Hypergammaglobulinemia
Connective Tissue Diseases
Subcutaneous Fat
Eosinophilia
Sclerosis
Contracture
Immunosuppressive Agents
Dermis
Methotrexate
Arthritis
Edema
Extremities
Joints
Magnetic Resonance Imaging
Biopsy

ASJC Scopus subject areas

  • Dermatology

Cite this

Eosinophilic fasciitis : Current concepts. / Fett, Nicole; Arthur, Megan.

In: Clinics in Dermatology, Vol. 36, No. 4, 01.07.2018, p. 487-497.

Research output: Contribution to journalArticle

Fett, Nicole ; Arthur, Megan. / Eosinophilic fasciitis : Current concepts. In: Clinics in Dermatology. 2018 ; Vol. 36, No. 4. pp. 487-497.
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