TY - JOUR
T1 - Eosinophilic fasciitis
T2 - Current concepts
AU - Fett, Nicole
AU - Arthur, Megan
N1 - Publisher Copyright:
© 2018
PY - 2018/7/1
Y1 - 2018/7/1
N2 - 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.
AB - 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.
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U2 - 10.1016/j.clindermatol.2018.04.006
DO - 10.1016/j.clindermatol.2018.04.006
M3 - Article
C2 - 30047432
AN - SCOPUS:85049456705
SN - 0738-081X
VL - 36
SP - 487
EP - 497
JO - Clinics in Dermatology
JF - Clinics in Dermatology
IS - 4
ER -