1/36. Multifocal peripheral neuropathy in eosinophilic fasciitis.Polyneuropathy may complicate eosinophilia-associated connective tissue disease. We report a multi-focal neuropathy in a patient with eosinophilic fasciitis proven by demonstrating an eosinophilic cellular infiltrate in a fascial biopsy specimen from the forearm. sural nerve biopsy revealed lymphocytic cuffing of epineural arterioles. Although described in the L-tryptophan-related eosinophilia-myalgia syndrome, peripheral neuropathy with these features has not been previously noted in a patient with eosinophilic fasciitis who had not consumed L-tryptophan.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
2/36. Miliary tuberculosis in a patient with eosinophilic fasciitis.The fibrosing disorders represent a diverse group of uncommon chronic diseases that include systemic sclerosis, eosinophilic fasciitis, eosinophilia-myalgia syndrome, toxic oil syndrome, and localized forms of fibrosis. A rare case of eosinophilic fasciitis is reported. The patient was a 61-year-old female who presented with generalized massive edema and eosinophilia. Signs of common edema-producing diseases, such as heart failure, were absent. Corticosteroids were initiated with slow improvement in edema. Over the ensuing months, miliary tuberculosis and tenosynovitis of her left hand and left foot developed, which responded to appropriate treatment. Moreover, prominent induration, hyperpigmentation, and coarse puckering of the skin gradually took place. Other conditions, especially neoplasms and infections, have been described in association with eosinophilic fasciitis, but this is the first reported case of miliary tuberculosis associated with the disease.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
3/36. Scleroderma, fasciitis, and eosinophilia associated with the ingestion of tryptophan.An association between the ingestion tryptophan and a syndrome characterized by scleroderma-like skin abnormalities, fasciitis, and eosinophilia has recently been recognized in the united states. We report the clinical and histopathological findings in nine patients and the results of biochemical analyses of tryptophan metabolism in seven patients with this syndrome. edema of the extremities, frequently accompanied by pruritus, paresthesia, and myalgia, developed in the nine patients (six women and three men; age range, 30 to 66 years) 1 to 18 months after the start of therapy with tryptophan (1.5 to 3.0 g daily) for insomnia, depression, or obesity. Five patients were taking drugs (benzodiazepines) known to inhibit hypothalamic-pituitary-adrenal function, and one had adrenal insufficiency. All had blood eosinophilia in the acute phase of their illness (mean eosinophil count [ /- SD], 3.62 /- 2.87 X 10(9) cells per liter). All had histopathological changes in the dermis and subcutaneous tissue typical of scleroderma, and seven patients had eosinophils. The fascia was inflamed and fibrotic, and adjacent skeletal muscle often showed perifascicular inflammation. tryptophan was discontinued in all patients, and eight received prednisone. The cutaneous symptoms improved, but only two patients had complete resolution of their illness. The patients had plasma levels of tryptophan before and after an oral dose of tryptophan that were similar to those in normal subjects. Plasma levels of L-kynurenine and quinolinic acid, which are metabolites of tryptophan, were significantly higher in four patients with active disease than in three patients studied after eosinophilia had resolved or in five normal subjects (P less than 0.001)--findings consistent with the activation of the enzyme indoleamine-2,3-dioxygenase. This illness resembles eosinophilic fasciitis and probably represents one aspect of the recently reported eosinophilia-myalgia syndrome. The development of the syndrome may result from a confluence of several factors, including the ingestion of tryptophan, exposure to agents that activate indoleamine-2,3-dioxygenase, and possibly, impaired function of the hypothalamic-pituitary-adrenal axis.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
4/36. L-tryptophan-induced eosinophilia-myalgia syndrome.This report describes three Belgian cases of the eosinophilia-myalgia syndrome associated with the use of L-tryptophan-containing products. Three women, aged 51, 53 and 73 years, were taking L-tryptophan for 2 months to 2 years, at 500, 1500, and 2250 mg d-1, respectively. All developed disabling myalgias, fatigue, and a variable skin rash, in association with marked eosinophilia. In one patient, symptoms and eosinophilia reappeared after rechallenge with L-tryptophan. Discontinuation of the drug resulted in gradual disappearance of the symptoms, signs and laboratory abnormalities in two patients. One patient was treated with corticosteroids because of persisting myalgias. Because of the non-specific clinical manifestations, clinicians from all subspecialties of internal medicine might be confronted with such patients and should be aware of this new entity.- - - - - - - - - - ranking = 5keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
5/36. plasmapheresis in a case of eosinophilia-myalgia syndrome with ascending polyneuropathy.eosinophilia-myalgia syndrome complicated by ascending polyneuropathy in a 40-year-old woman is described. High-dose intravenous steroids had no beneficial effect on the clinical course. Dramatic and rapid clinical improvement occurred with the use of plasmapheresis. The use of this therapeutic modality should be considered in patients with a similar clinical presentation.- - - - - - - - - - ranking = 4keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
6/36. Scleroderma-like fasciitis without eosinophilia after L-tryptophan ingestion.A 53-year-old man developed severe sclerodermatous skin changes and a neuromyopathic process, consistent with the eosinophilia-myalgia syndrome, 2 months after discontinuation of L-tryptophan (L-Try). His peripheral eosinophil count was within normal limits upon presentation and remained so throughout the illness. Currently the Centers for disease Control surveillance definition requires peripheral eosinophilia greater than 1000 cells/mm3. eosinophilia-myalgia syndrome may need to be part of a differential diagnosis even in the absence of peripheral eosinophilia.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
7/36. Papular mucinosis in L-tryptophan-induced eosinophilia-myalgia syndrome.Five patients with the L-tryptophan-related eosinophilia-myalgia syndrome had a generalized eruption of flesh-colored papules. In all patients, histologic examination revealed a focal accumulation of mucin in the upper mid dermis, associated with increased dermal cellularity. The mucin was composed predominantly of hyaluronic acid, with small amounts of sulfated acid mucopolysaccharides. The cells within the lesion were fibroblasts. The lesions slowly regressed after L-tryptophan was discontinued. Proposed explanations for the L-tryptophan-related eosinophilia-myalgia syndrome have centered on contaminants, chemically related to L-tryptophan, introduced in the manufacturing process. tryptophan metabolites have been linked with sclerotic cutaneous diseases but have not been previously implicated in cutaneous mucinoses.- - - - - - - - - - ranking = 6keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
8/36. neuromuscular manifestations of L-tryptophan-associated eosinophilia-myalgia syndrome: a histomorphologic analysis of 14 patients.The recent delineation of a clinical syndrome marked by eosinophilia, myalgia, and scleroderma-like skin changes associated with L-tryptophan use has necessitated the Centers for disease Control to initiate a health alert. The likely association of L-tryptophan ingestion with a syndrome that mimics eosinophilic fasciitis (Shulman's syndrome) further identifies an environmental agent associated with an inflammatory sclerosing rheumatic disease process. In this report, we present the clinical, morphologic, and enzyme histochemical findings in muscle, skin, and fascia biopsies from 14 cases fulfilling the Center for disease Control diagnostic criteria for L-tryptophan-associated eosinophilia-myalgia syndrome. The clinical syndrome reveals a high incidence of arthralgia, elbow contracture, and clinical neuropathy. The absence of significant change in creatine kinase or sedimentation rate allows for diagnostic separation from other inflammatory myopathies. Histoenzymatic features in muscle biopsies reveal a preferential epimysial-perimysial noneosinophilic infiltration characterized by acid phosphatase reactive histiocytosis, nonnecrotizing venulitis, perineural inflammation within dermis and perimysium, type II fiber atrophy with superimposed denervation features, and perifascicular alkaline phosphatase reactivity representing early neofibroplasia. The constellation of changes in skin, fascia, and muscle, with the defined clinical syndrome, allows for accurate differentiation from allied syndromes, including eosinophilic polymyositis, scleroderma, idiopathic polymyositis/dermatomyositis, polyarteritis nodosa, and toxic oil syndrome. Accurate differentiation from eosinophilic fasciitis still rests on a history of L-tryptophan ingestion.- - - - - - - - - - ranking = 5keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
9/36. Pulmonary involvement in the eosinophilia-myalgia syndrome.We describe the cases of three women with pulmonary involvement in the eosinophilia-myalgia syndrome. The illness was characterized by elevated peripheral blood eosinophil counts, myalgias, fatigue, and dyspnea. Two of three patients had bilateral infiltrates on chest roentgenograms. All three had markedly decreased carbon monoxide diffusing capacities and pulmonary hypertension. High-dose prednisone therapy provided only partial resolution of the pulmonary symptoms. Open lung biopsy specimens showed chronic interstitial and perivascular infiltrates in two of the patients and moderate fibrointimal hyperplasia of pulmonary vasculature in the third. High-dose prednisone therapy prior to the biopsies may have modified the original histologic features.- - - - - - - - - - ranking = 5keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
10/36. eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan: muscle biopsy findings in 4 patients.Muscle biopsies of 4 patients with the eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan showed lymphocytic infiltrates with occasional eosinophils largely restricted to interstitial fibrous tissue and perivascular areas. There was inflammation and fibrosis of muscle spindle capsules in 3 patients. In the 2 sickest patients, there was profound muscle atrophy, affecting both muscle fiber types.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
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