Asymptomatic diffuse fasciitis with eosinophilia

The case of a 70-year-old woman with asymptomatic diffuse fasciitis with eosinophilia, confirmed by biopsy, is presented.     

An overlap of diffuse fasciitis with eosinophilia and scleroderma

Summary A case with overlapping features of scleroderma and diffuse fasciitis with eosinophilia is presented.     

Eosinophilia-myalgia syndrome with fasciitis and interstitial myositis after L-tryptophan administration]

We describe a 53-year-old women with eosinophilia-myalgia syndrome who suddenly developed severe persistent myalgias of her arms, legs, back, and shoulder after a 5-month period of daily L-tryptophan ingestion, associated with fever, progressive stenocardia and left-sided congestive heart failure. Laboratory tests showed a leukocytosis of 11.2/nl with 3.14/nl eosinophils and an elevated erythrocyte sedimentation rate. There was a marked, predominantly proximal sclerosis of her arms, legs and trunk with a brownish discoloration. The skin of her arms and legs appeared dimpled (peau d'orange). Findings of the electrophysiological examinations were consistent with sensory neuropathy and myositis. Remarkable fasciitis and interstitial myositis were present in a biopsy specimen (from skin to muscle) taken from her thigh. However, eosinophilic infiltrates were rare. Angiography revealed an apical obstructive cardiomyopathy. In this paper, we describe the clinical findings, the course over 2 years, as well as the therapeutic management. Furthermore, the most important differential diagnoses are discussed and the literature is reviewed with special attention given to more recent pathogenic insights into this newly recognized multisystem disease.     

Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome?

Two male patients (53 and 19 years old) have been seen with a diffuse scleroderma-like illness with firm taut skin bound down to underlying structures (sparing the face in both patients and the hands and feet in one). Flexion contractures of elbows and knees and limitation of abduction at the shoulders developed in a few weeks after onset. Raynaud's phenomenon was absent and thorough evaluation failed to reveal any evidence of myositis or the visceral manifestations of systemic sclerosis. There was no loss of skin appendages. Both had circulating eosinophils (12% and 37%) without drug ingestion or parasitic infestation. Both had elevated sedimentation rates and hypergammaglobulinemia (in one, IgG = 4.1 g.%). Serologic tests for syphilis, rheumatoid factor, LE cells, antinuclear antibodies, complement and cryoglobulins were negative or normal in both patients. Bone marrow examination revealed plasmacytosis and eosinophilia. Biopsies revealed striking thickening of the fascia between the subcutis and muscle. Within the thickened connective tissue there was intense infiltration with plasma cells and lymphocytes, at times in follicles, both about and separate from vessels; eosinophils were absent. Skin biopsies revealed no changes of scleroderma; and muscle biopsies, no evidence of myositis. Prednisone therapy over 15 months in decreasing dosage, using laboratory parameters, induced in the first patient a full remission which has persisted for 5 years without therapy. In the second (more severely involved) patient prednisone therapy has reversed laboratory abnormalities; but clinical improvement to date has been minimal. The pathogenesis of this diffuse fasciitis is obscure, although unusual physical exertion antedated the onset of illness in each case.     

Diffuse fasciitis and eosinophilia with symmetric polyarthritis

Articular involvement has been infrequently reported in diffuse fasciitis with eosinophilia. We report a patient with diffuse fasciitis with eosinophilia, concurrent symmetrical polyarthritis, and bilateral carpal tunnel syndrome. Light microscopy and immunofluorescence studies of synovium and full-thickness calf biopsy were done. Synovial fluid analyses showed mildly inflammatory fluid. Response to prednisone, clinically and by laboratory indicators, was prompt and dramatic.     

Eosinophilia-myalgia syndrome associated with L-tryptophan use

The eosinophilia-myalgia syndrome associated with the use of oral preparations of the amino acid L-tryptophan was recognized in late 1989. We describe the clinical and laboratory manifestations, pathological findings and early clinical course of 20 patients with the eosinophilia-myalgia syndrome. Prominent clinical findings included severe myalgias limiting function, fatigue, rashes, edema and weight gain, weight loss, muscle weakness and shortness of breath. Laboratory findings included eosinophilia (often marked), normal erythrocyte sedimentation rate, and elevated aldolase with normal or low creatine kinase values. On biopsy fascial inflammation was always seen consisting of lymphocytes, histiocytes and eosinophils in a perivascular distribution. Invasion of the vascular wall by lymphocytes was seen in 20%. Capillary and arteriolar endothelial cell thickening was found in most cases on electron microscopy and endothelial cell necrosis or mural invasion by lymphocytes was seen in 25% of cases. Two patients improved with no therapy. Ten patients responded to therapy with prednisone alone. Three patients have had progressive disease and one of these died. The relationship of this syndrome to previously described disease entities associated with eosinophilia is discussed.     

Diffuse fasciitis with eosinophilia: A clinicopathologic study

Three cases of diffuse fasciitis with eosinophilia are presented, and their clinical, laboratory and histologic follow-up are outlined. Furthermore histologic comparison with scleroderma is attempted and the literature is reviewed. From our findings, we conclude that diffuse fasciitis with eosinophilia is a distinct clinicopathologic entity.     

Eosinophilia-myalgia syndrome. Natural history in a population-based cohort.

BACKGROUND--To determine the natural history of eosinophilia-myalgia syndrome, we followed up all patients with eosinophilia-myalgia syndrome reported to the Oregon Health Division, Portland, during the recent epidemic caused by contaminated tryptophan. METHODS--Patients were interviewed by telephone from 1 to 5 months after illness onset and again at least 12 months after onset. Symptoms (type, onset, and duration), overall disability, treatment, and tryptophan lot and dose were assessed for each patient. RESULTS--Information was obtained for 55 (96%) of 57 case-patients: 53 patients completed interviews and two patients had died. For the 53 patients who were interviewed, symptoms with onset more commonly during the first 3 months of illness included severe myalgias, fatigue, generalized weakness, edema, and rash. Symptoms with later onset included paresthesias, muscle cramps, extremity weakness, and alopecia. At 12 months, 41 patients (77%) continued to report fatigue, 36 (68%) weakness, and 34 (64%) myalgias; 26 patients (49%) had difficulty climbing stairs, 23 (43%) had difficulty getting up from a chair, and 15 (28%) had difficulty holding a cup. Higher doses of tryptophan were correlated with more severe disability, both initially (rs = .33) and at follow-up (rs = .42). Although most patients reported improvement in symptoms at 12 months, only 14 (26%) patients reported that they were able to perform all normal daily activities. CONCLUSIONS--Most patients with eosinophilia-myalgia syndrome in this population-based cohort are still symptomatic 1 year after onset, primarily with the complaints reported early in the illness. The association between degree of disability and daily tryptophan dose suggests that ingestion of varying amounts of contaminant may be responsible, in part, for the severity of symptoms experienced by individual patients.     

Eosinophilia-myalgia syndrome. Recognition of a distinct clinicopathologic entity

The eosinophilia-myalgia syndrome is a newly described disorder related to the ingestion of L-tryptophan-containing products. Its presentation may mimic other disorders characterized by eosinophilia and muscle pain and/or weakness, but can be differentiated by certain characteristic laboratory and pathologic findings. We report two such cases, describe their features, and review similar syndromes.     

The eosinophilia-myalgia syndrome and eosinophilic fasciitis.

The scientific excitement that follows the recognition of a new disease has been reflected in the numerous publications describing the clinical, histopathologic, and pathogenetic aspects of the eosinophilia-myalgia syndrome (EMS) during the period covered by this review. The clinical picture that has emerged during the past 2 years indicates that EMS is a multisystemic disease with prominent cutaneous, hematologic, and visceral manifestations that frequently evolves into a chronic course and can occasionally be fatal. Considerable progress has been made toward understanding the etiology and pathogenesis of EMS. The demonstration of an association with the ingestion of L-tryptophan-containing products originating from a single source has led to the identification and characterization of a putative etiologic agent present as a contaminant in these preparations. Although the accumulation of eosinophils, lymphocytes, macrophages, and fibroblasts in the affected tissues suggests that these cells play important roles in the pathogenesis of EMS, the precise mechanisms of their involvement have not been established. Several studies have demonstrated the activation of eosinophils and the deposition of eosinophil-derived toxic proteins in affected tissues. Fibroblast activation and increased expression of genes coding for various connective tissue macromolecules have been demonstrated employing in situ hybridizations with complementary DNAs. Furthermore, interleukin-5 and transforming growth factor-beta have been implicated as potential mediators in the pathogenesis of EMS. The explosive epidemic of EMS has emphasized the importance of chemical and environmental factors in the development of systemic disorders characterized by chronic inflammation and fibrosis. It is expected that further study of the pathogenesis of EMS will provide valuable information regarding the mechanisms responsible for these obscure disorders.     

Nonallergic rhinitis with eosinophilia syndrome

Nonallergic rhinitis with eosinophilia syndrome (NARES) is a clinical syndrome comprising symptoms consistent with allergic rhinitis in which an absence of atopy has been demonstrated by allergen skin testing, and nasal cytology analysis demonstrates more than 20% eosinophils. Anosmia is a prominent feature not shared with allergic rhinitis. The pathophysiology of NARES is poorly understood, but a key component involves a self-perpetuating, chronic eosinophilic nasal inflammation with development of nasal micropolyposis and polyposis. Mast cells likely play an important role as well. NARES is a risk factor for the development of nasal polyposis and aspirin sensitivity, as well as obstructive sleep apnea. Treatment consists mainly of intranasal corticosteroids with or without the addition of second-generation antihistamines and/or leukotriene-receptor antagonists.     

Treatment with cimetidine of atypical fasciitis panniculitis syndrome.

Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid antibodies, improved. It is concluded that features of eosinophilic fasciitis or localised scleroderma and certain additional atypical elements should be categorised as atypical fasciitis-panniculitis syndrome.