Eosinophilic Fasciitis: Clinical, Biological, Histological and Therapeutic Features from a Series of 28 Patients

OBJECTIVE: To determine the clinical, biological, histological and therapeutic features of patients with eosinophilic fasciitis (EF).
METHODS: Monocentric study including patients with biopsy proven EF. Diagnosis was based on the presence of a non neoplastic inflammatory infiltrate in the fascia. Main features were retrospectively collected and analyzed.
RESULTS: 28 patients: 17 females (61%), 11 men (39%), mean age at diagnosis: 53±17 yrs.
At diagnosis, cutaneous manifestations were present in 24 patients (86%): deep cutaneous sclerosis in 21 (75%), arm oedema in 14 (50%), leg oedema in 13 (46%), an aspect of sunken veins in 14 (50%) and an associated morphea in 7 (25%) patients. Myalgia were present in 23 patients (82%), arthralgia in 11 (39%), a carpal tunnel syndrome in 7 (25%) patients and Raynaud’s syndrome in 2 (7%). The muscle-fascia-skin biopsy showed fascia inflammatory infiltrates composed of lymphocytes (100%) and eosinophils in 21 patients (78%) with a perivascular tropism. Muscle inflammatory infiltrates were present in 13 patients (46%). Fibrosis was present in 1 case.
Biology: blood eosinophilia (>0.5 G/L) in 23 patients (82%) [Mean: 1.5±1.1 G/L], inflammatory syndrome 13/25 (52%), raised CPK 3/22 (14%).
Muscle MRI (12 patients): abnormal in 8 (83%) with fascia thickening, abnormal signal intensity and gadolinium contrast enhancement (7/8), fat infiltration (2/8) and muscular abnormal signal intensity (2/8).
Nailfold capillaroscopy (22 patients): normal in 20 (91%) and disorganization of the capillary bed without enlarged loops in 2.
Treatment and Evolution: 26 patients (93%) received a treatment comprising in all cases steroids (0.5 to 1 mg/kg/day). Ten patients received prednisolone pulses at treatment initiation. For 11 patients (42%) (6 females, 5 males), immunosuppressive drugs (ID) were introduced due to an incomplete clinical response: methotrexate (n=9) and azathioprine (n=2). The interval between the introduction of steroids and of ID was 20.9±21 months (range: 3 - 67 mo; median 10 mo), higher in females (32±23 mo) than in males (7.8±7 mo) [P=0.04]. The whole course duration was 34±28 months (median 24 mo) for patients who received steroids alone (n=15) versus 48±30 months (median 44 mo) for those with ID. A complete response was achieved in 17 patients (65%), tendon retractions and or local cutaneous indurations were still present in 6 patients (23%) and 3 patients (12%) still need high steroid doses (>20 mg/d). No hematological complications were observed during the follow-up.
Conclusion: EF is a rare disease characterized by cutaneous and muscular symptoms, a frequent blood eosinophilia. The mainstay treatment is steroids alone or in combination with an immunosuppressive drug, mainly methotrexate, allowing a satisfying response in more than 80% of patients.

 D. Sène, None; D. Lebeau, None; C. Francès, None; B. Wechsler, None; Z. Amoura, None; T. Maisonobe, None; S. Barete, None; O. Benveniste, None; P. Cacoub, None; J.C. Piette, None.