1/7. The many faces of scleroderma.This review integrates the clinical aspects of systemic sclerosis (SSc; scleroderma) and scleroderma-like conditions with new knowledge of the control of blood vessel tone and the role of anoxia in the activation of connective tissues leading to fibrosis. serologic tests, high resolution computed tomographic scanning, bronchoalveolar lavage, and physiologic assessment of pulmonary gas diffusion are compared as diagnostic tools and as means of quantitating internal organ involvement. Treatment of Raynaud's disease and phenomenon, management of scleroderma renal crisis, and new means for improving gastrointestinal function with octreotide, the somatostatin analogue, also are discussed. The relationship between idiopathic forms of SSc and eosinophilic fasciitis/eosinophilia-myalgia syndrome caused by L-tryptophan ingestion and the scleroderma-like disease associated with silicone breast implants also is discussed.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
2/7. Cutaneous manifestations of the eosinophilia-myalgia syndrome.We report the cutaneous manifestations of the eosinophilia-myalgia syndrome in 10 patients, with specific reference to their clinical course, histopathological features, and immunogenetic studies. Cutaneous manifestations could be classified into three groups: morphoea-like sclerosis, urticarial and papular lesions, and generalized sclerosis. Despite this polymorphic clinical presentation, the histopathological abnormalities in all cases were strikingly similar, and consisted of superficial and deep perivascular lymphocytic dermal infiltrates, mucin deposition, and fascial inflammation (often in the absence of sclerosis). Immunoperoxidase studies revealed increased numbers of factor xiiia- and MAC 387-positive cells in the inflammatory infiltrate. Immunogenetic studies demonstrated that 77% (7/9) of patients possessed the HLA-DR3 or HLA-DR4 phenotypes. Mean follow-up of 24 months after discontinuation of L-tryptophan revealed the presence of persistent severe disabling disease in 30% of patients.- - - - - - - - - - ranking = 5keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
3/7. L-tryptophan syndrome: histologic features of scleroderma-like skin changes.The eosinophilia-myalgia syndrome (EMS) associated with the ingestion of L-tryptophan (LT) containing products has recently been recognized in the United States. We report the histologic features of the cutaneous scleroderma-like changes in four patients. All of the patients met the Center for disease Control criteria for EMS and had a history of LT ingestion. Skin biopsies showed increased dermal mucin and dermal sclerosis, with trapping of adnexal structures. There are clinical and histologic similarities between EMS, scleroderma, the toxic oil syndrome, and fasciitis with eosinophils.- - - - - - - - - - ranking = 1keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
4/7. 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) |
5/7. 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) |
6/7. Postmortem studies of the heart in three fatal cases of the eosinophilia-myalgia syndrome.OBJECTIVE: To examine the hearts of individuals who died from the eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan, with particular attention paid to the coronary arteries, the neural structures, and the conduction system of the heart because of reported terminal disturbances of cardiac rhythm and conduction. STUDY MATERIAL: Three hearts fixed in neutral formalin and well preserved with all the relevant areas of conduction system intact. methods: light microscopic examination of subserial sections of the sinus node, atrioventricular node and His bundle, coronary chemoreceptor and regional nerves, ganglia, and small coronary arteries. Routine stains used were Goldner trichrome and Verhoeff-van Gieson. RESULTS: Arterial abnormalities were numerous and primarily of two types: focal fibromuscular dysplasia causing moderate to severe narrowing, as well as endarteritis and panarteritis. Extensive examples of neuritis and ganglionitis were present throughout the heart, including the conduction system, where arterial abnormalities were also abundant. In the coronary chemoreceptor there were both old and new lesions comprising focal inflammation with degeneration as well as older areas of fibrotic destruction. Within the sinus node, areas of dense fibrosis replaced all nodal tissue. These abnormalities were similar in nature and extent in all three hearts. CONCLUSIONS: The pathologic lesions present in the coronary arteries, neural structures, and conduction system of the heart in patients who died from the eosinophilia-myalgia syndrome provide a suitable anatomic substrate for substantial cardiac electrical instability, including the occurrence of sudden death. In cases of unexplained cardiac electrical instability or sudden unexpected death an inquiry should be made about previous use of L-tryptophan. In patients with the eosinophilia-myalgia syndrome, the possibility of cardiac electrical instability should be considered as part of long-range clinical management.- - - - - - - - - - ranking = 4.2892847425336keywords = eosinophilia-myalgia syndrome, eosinophilia-myalgia (Clic here for more details about this article) |
7/7. Scleroderma and L-tryptophan: a possible explanation of the eosinophilia-myalgia syndrome.Scleroderma developed in six women who were taking L-tryptophan. fasciitis and morphea were most common, but one patient had pleural effusion, hypertension, and signs of cardiac and kidney failure. In five patients the biopsy findings were characteristic of scleroderma; the sixth patient had Crohn's disease and developed fasciitis; her biopsy specimen showed inflammatory arteritis. All patients' conditions improved after cessation of their L-tryptophan intake, initiation of corticosteroid therapy, or both. These findings confirm previous data that show altered tryptophan-kynurenine metabolism in some patients with scleroderma and fasciitis, particularly with tryptophan loading.- - - - - - - - - - ranking = 1.2892847425336keywords = eosinophilia-myalgia (Clic here for more details about this article) |