The eosinophilic fasciitis (EF) is a rare scleroderma-like disorder, characterised by the sudden onset of symmetrical painful, tender, oedematous and erythematous extremities with progressive thickening of the skin and soft tissues. The diagnosis of EF is often based on the association of cutaneous abnormalities with histological examination showing a thickened fascia with an inflammatory infiltration constituted by lymphocytes and eosinophils. Peripheral eosinophilia is common at least at the early stages but not mandatory for the diagnosis. Imaging is under development; ultrasound but mostly magnetic resonance imaging than can objectify fascia involvement with signal enhancement after gadolinium administration can be of value. Differential diagnoses are mainly systemic sclerosis, localised scleroderma, inflammatory myositis, hypereosinophilic syndromes, eosinophilia–myalgia syndrome after l-tryptophan exposure and graft versus host disease. Despite the scarcity of this condition and lacking clinical trials, there is substantial agreement among published cases or case series that corticosteroids, initially at high dose (more than 20 mg prednisone/day and often 0.5–1 mg/kg as a starting dose), are the first-line treatment. Methotrexate seems to be the first-line immunosuppressive drug given in addition to corticosteroids or for some authors only in case of an unsatisfactory response to corticosteroids alone. For refractory diseases, other immunosuppressive drugs may be offered after ruling out associated neoplasia.
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
This is a preview of subscription content, log in to check access.
Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians. 1975;88:70–86.PubMedGoogle Scholar
Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after-what do we really know. Report of 11 patients and review of the literature. Dermatology. 2006;213:93–101.PubMedCrossRefGoogle Scholar
Rodnan GP, Di Bartolomeo A, Medsger Jr TA. Proceedings: eosinophilic fasciitis: report of six cases of a newly recognized scleroderma-like syndrome. Arthritis Rheum. 1975;18:525.PubMedCrossRefGoogle Scholar
Bischoff L, Derk CT. Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature. Int J Dermatol. 2008;47:29–35.PubMedCrossRefGoogle Scholar
Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1998;17:221–31.CrossRefGoogle Scholar
Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai Y, Ishikawa O. Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol. 2007;26:1445–51.PubMedCrossRefGoogle Scholar
Agnew KL, Blunt D, Francis ND, Bunker CB. Magnetic resonance imaging in eosinophilic fasciitis. Clin Exp Dermatol. 2005;30:435–6.PubMedCrossRefGoogle Scholar
Schaffer JV, McNif JM, Seropian S, Cooper DL, Bolognia JL. Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum. J Am Acad Dermatol. 2005;53:591–601.PubMedCrossRefGoogle Scholar
Khanna D, Agrawal H, Clements PJ. Infliximab may be effective in the treatment of steroid-resistant eosinophilic fasciitis: report of three cases. Rheumatology (Oxford). 2010;49:1184–8.CrossRefGoogle Scholar