Ungueal capillaroscopy in fasciitis with eosinophilia: a distinctive feature of systemic scleroderma. Apropos of 15 cases

Eosinophilic fasciitis (EF) is a recently described disease whose distinction from progressive systemic sclerosis (PSS) is still being discussed. PSS has a characteristic microcirculation pattern. We performed nailfold microscopy on 15 patients with EF and compared the results to those of 98 PSS patients and 75 normal control subjects. EF patients have a normal microcirculation pattern (13/15) or discrete, non-specific anomalies: none had the typical capillary pattern associated with PSS and associated diseases. The findings of this study justify making a distinction between EF and PSS and demonstrate that nail fold microscopy can be a useful tool for an early differential diagnosis between these two disorders.     

Tuberculous fasciitis in scleroderma

A 25-year old woman visited the hospital because of a painful swelling for 20 days in the midposterior portion of her right thigh. She had been diagnosed with systemic sclerosis and treated for 10 months. An MRI scan of the right thigh showed diffuse fascial thickening and involved the superficial portion of thigh muscles, which were hyperintense on the T₂-weighted image. A biopsy of the involved muscles revealed chronic granulomatous inflammation with several acid-fast bacilli on a Ziehl–Neelsen stain. We here report a case of tuberculous fasciitis manifest with painful swelling of the midposterior muscles of the right thigh without pulmonary tuberculosis in a patient with scleroderma.Abbreviations HIV Human immunodeficiency virus     

L-tryptophan ingestion associated with eosinophilic fasciitis but not progressive systemic sclerosis

OBJECTIVE: To assess the use of L-tryptophan by patients with eosinophilic fasciitis and compare this with its use by patients with progressive systemic sclerosis (scleroderma). DESIGN: Retrospective and prospective analysis. Six patients with eosinophilic fasciitis were identified retrospectively and two prospectively. Retrospective identification of patients was done by questioning hospital-affiliated rheumatologists and dermatologists and by searching the hospital dermatopathology database. The patients with scleroderma or morphea were prospectively identified by questioning consecutive office patients with these established diagnoses. SETTING: University of Pennsylvania rheumatology and dermatology practices. PATIENTS: Eight patients with eosinophilic fasciitis; 40 consecutive patients with scleroderma (27 with diffuse cutaneous and 13, limited cutaneous disease); 3 patients with morphea. RESULTS OF DATA ANALYSIS: All eight patients with eosinophilic fasciitis had taken L-tryptophan before the onset of their disease. All had myalgias and high peripheral eosinophil counts (most greater than 5000 cells/mm3). Only 1 of 40 patients with scleroderma (no patients with morphea) had used L-tryptophan preceding illness (P less than 0.001 compared with eosinophilic fasciitis). Six patients with eosinophilic fasciitis had taken L-tryptophan for less than 8 months. One patient had taken it for 9 years before developing skin induration. Two patients were newly identified as having hypothyroidism; two developed neuropathy; and two had severe flexion contractures (several occurring in areas without skin induration). Five patients had low-titer antinuclear antibodies, indicating a possible autoimmune process. Most patients had only a partial response to systemic corticosteroid therapy. One patient has had important disease regression in response to isotretenoin therapy that was evident even while she continued to take L-tryptophan. CONCLUSIONS: L-Tryptophan use can lead to eosinophilic fasciitis whereas it does not appear to cause classic scleroderma. The disease process does not automatically remit after discontinuation of L-tryptophan-containing products.     

Dystrophic calcinosis in eosinophilic fasciitis

Clinical Rheumatology -     

Diagnostic problems in eosinophilic fasciitis

We presented two cases with symptoms of diffuse swelling of subcutaneous tissue, stiffness and tenderness of involved areas, fever, eosinophilia and hypergammaglobulinemia. The inflammatory infiltrates consisting of lymphocytes, plasma cells and eosinophils were yielded in fascia. The difficulties in differentition of the symptoms between eosinophilic fasciitis and "eosinophilia-myalgia syndrome" are discussed.     

Paraneoplastic eosinophilic fasciitis: a case report

A 60-year-old white woman with polycythemia rubra vera post splenectomy in November 2001 was found to have peripheral white blood cell counts increasing over 3 months. Cytogenetics revealed trisomy of chromosomes 8 and 9, and bone marrow biopsy showed hypercellular, fibrotic bone marrow consistent with myelofibrosis of polycythemia rubra vera. Two months later, the patient developed acute swelling and pain in her lower extremities. The clinical symptoms along with confirmatory histology supported the diagnosis of eosinophilic fasciitis. This is the first reported case in the English literature of an association between polycythemia vera and eosinophilic fasciitis.     

The groove sign in eosinophilic fasciitis

Clinical Rheumatology -     

Synovial fluid lymphocytes in eosinophilic fasciitis

Arthritis is an uncommon manifestation of eosinophilic fasciitis. We report a patient who developed arthritis of his left knee, followed 2 weeks later by skin lesions which evolved into histologically proven eosinophilic fasciitis. Synovianalysis on 2 occasions demonstrated a mildly inflammatory fluid with more than 90% lymphocytes. These lymphocytes were shown to be primarily activated suppressor/cytotoxic T cells.     

Serum eosinophilotactic activity in eosinophilic fasciitis

Serum eosinophil chemotactic activity was determined by the Boyden chamber technique in 20 patients with eosinophilic fasciitis. Increased eosinophil chemotactic activity (>125% of background) was found in all 20 patients (mean 297% ± 129), whereas sera of 20 controls with systemic sclerosis and diffuse scleroderma had increased eosinophil chemotactic activity in only 6 (30%) instances (mean 96% ± 47, P<0.001). Although serum eosinophil chemotactic activity decreased over time in all 8 eosinophilic fasciitis patients studied longitudinally, this activity returned to normal in only 3, all of whom received prolonged courses of corticosteroids. Preliminary data suggest the existence of a eosinophilotactic serum factor common to patients with eosinophilic fasciitis.     

Circulating immune complexes in eosinophilic fasciitis

Serum immune complexes were measured in 22 patients with eosinophilic fasciitis, 8 of whom had serial determinations. Elevated levels were found by Raji cell radioimmunoassay in 14 (64%) patients, by agarose gel electrophoresis in 13 (59%), and by C1q agglutination-inhibition in 9 (41%). Elevated levels by Raji cell assay were present more frequently at times of active disease [17 of 22 sera (77%)] than at times of inactive disease [2 of 24 (8%) (P<0.0005)] and were more closely correlated with disease activity than were eosinophilia, hypergammaglobulinemia, or increased erythrocyte sedimentation rate.     

Magnetic resonance imaging in the evaluation of patients with eosinophilic fasciitis

Two cases of eosinophilic fasciitis are described, in which magnetic resonance imaging (MRI) clearly demonstrated thickening of the fascia and increased signal intensity in the superficial muscle fibers correlating with inflammation. MRI is a useful noninvasive imaging technique in the diagnosis of eosinophilic fasciitis and can guide biopsy of selected abnormal areas.     

Pulmonary involvement in juvenile eosinophilic fasciitis: A case report

Introduction: Eosinophilic fasciitis (EF) is a rare scleroderma-like disease, characterized by acute onset of symmetrical swelling, induration and thickness of the skin. Laboratory findings include peripheral eosinophilia, elevated inflammatory markers and increased gammaglobulemia. Although, the diagnosis is mainly clinically, a full thickness skin/fascia/muscle biopsy remains the gold standard for the definite diagnosis. Unlike systemic sclerosis, visceral involvement, such as pulmonary affection, is rare in EF. Although, few cases of systemic involvement in adult EF have been presented, we report a rare case of juvenile EF associated with pulmonary involvement. Case report: A 12-years old boy presented with symmetrical skin thickening of bilateral upper extremity, back, trunk, neck, face, and scalp of 2-weeks duration. The laboratory tests revealed marked peripheral eosinophilia 61.9% (normal 1–3%) and elevation of the acute phase reactants (erythrocyte sedimentation rate 40?mm/1st hour and C-reactive protein 15?mg/dL). Lung imaging study showed bilateral extensive pulmonary nodules. A full thickness skin/fascia/muscle biopsy revealed an inflammatory infiltration, fibrosis in the fascial and muscle tissues. These findings were concordant with EF. An initial treatment of intravenous (IV) methylprednisolone 30?mg/kg/day for 3 successive days was started followed by oral steroid (2?mg/kg/day) plus methotrexate (20?mg/week). Follow up revealed complete improvement in the skin thickening, pulmonary affection and systemic inflammation. Conclusion: To the best of our knowledge this is the first reported co-existence of pediatric eosinophilic fasciitis with pulmonary affection. Systemic involvement should be screened in EF cases, as it may have consequences in the management and outcome.