Inversion of chromosome 16, inv(16)(p13q22), is characteristic of acute myeloid leukaemia (AML) with eosinophilia and is rarely found in the myelodysplastic syndrome (MDS). We report three cases of MDS in which inv(16) was observed. They were classified to FAB subtypes RA, RARS and RAEBT; eosinophilia or abnormal eosinophils were not ...

Eosinophilia-myalgia syndrome (EMS) has been linked to ingestion of tryptophan contaminated with 1,1'-ethylidene-bis[L-tryptophan] (EBT), but other contaminants have received little study. The authors identified 101 lots of L-tryptophan that had been consumed either by persons with EMS or by asymptomatic tryptophan users and quantified the amounts of EBT and five ...

Sweet's syndrome (acute febrile neutrophilic dermatosis) is a steroid-responsive dermatosis characterized by pyrexia, neutrophilia, and painful erythematous plaques that histologically show a dense dermal infiltrate of neutrophils. Pregnancy-associated Sweet's syndrome is defined as the initial appearance or recurrent episode of Sweet's syndrome in a pregnant woman. Sweet's syndrome occurring during ...

The clinical and hematological characteristics of ten children with myelodysplastic syndromes diagnosed and followed up over a 3 year period are presented. All of them had anemia and a low platelet count whilst the white blood cell count was variable. Presentation with bilateral proptosis and acute febrile neutrophilic dermatosis (Sweet's ...

Eosinophilia-myalgia syndrome is an idiopathic disorder characterized by myalgia, especially of the extremities and peripheral eosinophilia. It is found in some patients as a result of tryptophan ingestion. We examined four patients showing clinical features and muscle biopsy changes consistent with eosinophilia-myalgia syndrome. The results of conventional histology were compared ...

The clinical course of a 56-year-old female patient with Sweet's syndrome (SS) preceded by a myelodysplastic syndrome (MDS) is described. During the acute phase of the disease with high remittent fever, painful skin lesions and maximal leucocytosis IL-6 and G-CSF serum levels were extremely high, while TNF-alpha was only slightly ...

The Churg-Strauss syndrome is a rare but treatable clinical entity. Its clinical presentation is thought to be stereotypic but is not. The authors report the first instance of a patient with the Churg-Strauss syndrome with hemodynamically significant pericardial effusion but without concurrent active pulmonary, renal or neurological involvement. Pericardial biopsy ...

BACKGROUND: Sweet's syndrome is well recognized and not infrequently diagnosed in Spain; however, the range of clinical and pathologic expression may not have been fully realized. METHODS: We reviewed 30 consecutive Spanish cases of Sweet's syndrome diagnosed in our department from 1979 to 1990, with special attention to clinical and ...

We describe the long-term follow-up of two patients who experienced diffuse fasciitis and eosinophilia while ingesting L-tryptophan for treatment of insomnia. This is one of the first reports in the dermatologic literature of the long-term follow-up of eosinophilic fasciitis in association with L-tryptophan ingestion. Both patients fit the Centers for ...

Neurologic symptoms are common in patients with the Churg-Strauss syndrome and often manifest as a peripheral neuropathy of the mononeuritis multiplex type secondary to vasculitis. Reports of central nervous system involvement is less frequent and ranges from psychiatric disturbances to cerebral hemorrhage. We report the first case of pathologically documented ...

Vasculitic lesions are not generally associated with eosinophilic fasciitis. Eosinophilic fasciitis is reported to be a syndrome distinct from progressive systemic sclerosis (PSS). More recent studies, however, note overlapping features in the clinical, pathologic, and laboratory findings of eosinophilic fasciitis and scleroderma. We report a typical presentation of eosinophilic fasciitis ...

Eosinophilia-myalgia syndrome (EMS) is thought to be caused by the intake of contaminated L-tryptophan products. 1,1'-Ethyliden bis[L-tryptophan] (EBT) is one of the contaminants in the L-tryptophan products and is thought to be a possible cause of EMS. In the present study we report that anti-EBT antibodies are generated by immunizing ...

A sixty-six-year-old white man presented with a five-day history of painful erythematous papules, plaques, pustules, and hemorrhagic bullae on both hands. His history was remarkable for having prepared and pickled fifteen quarts of home-grown jalapeño peppers several days before the eruption occurred. The light microscopic examination of sections of lesional ...

We report the case of a 67-year-old man with allergic granulomatosis and angiitis (AGA; Churg-Strauss syndrome) who developed nephrotic syndrome during his clinical course and demonstrated membranous nephropathy on renal necropsy by electron microscopy. Following the development of symptoms of bronchial asthma accompanied by eosinophilia and mononeuritis multiplex, transbronchial lung ...

The CHSS was first described as an allergic granulomatosis and angiitis. This syndrome is characterized by pulmonary and systemic vasculitis, extravascular granulomas and eosinophilia which occur in patients with bronchial asthma and allergy. The CHSS in limited form may be present in an isolated organ or tissue. Despite increased awareness ...

The L-tryptophan eosinophilic myalgia syndrome (EMS) clinically has some similarities with idiopathic eosinophilic fasciitis (EF). In order to study the pathology of both syndromes, we analyzed 21 biopsies of patients with EMS and 8 with idiopathic EF. In both diseases there is dermal and fascial mucin and dermal edema, but ...

We report two cases of simultaneous Sweet's syndrome and erythema nodosum. We believe this to be a real association rather than the extension of the neutrophilic infiltrate of Sweet's syndrome into the subcutaneous fat. In one of our cases, concurrent erythema nodosum and Sweet's syndrome was a presentation of sarcoidosis ...

A cutaneous or systemic vasculitis occurs in myelodysplasia as well as in myeloproliferative and lymphoproliferative disorders. The most common lesion is a leucocytoclastic vasculitis, with neurological or joint involvement occurring less often. The vasculitis may appear contemporaneously with or precede the clinical onset of the blood dyscrasia. Occasionally the lesions ...

The eosinophilia-myalgia syndrome, which is associated with the ingestion of L-tryptophan that contained products, occurred as an epidemic in the United States in 1989. Eosinophilia-myalgia syndrome is similar in many ways to the toxic-oil syndrome, which occurred in Spain in 1981, and to diffuse fasciitis with eosinophilia, which has been ...

This is the second reported case of minocycline-induced Sweet's syndrome (and the first such case to appear in the American literature). The syndrome developed in a 32-year-old man 10 days after minocycline therapy for acne was begun and resolved rapidly after discontinuation of the medication and start of oral prednisone ...

A 62-year-old woman who had a history of asthma and allergic rhinitis developed bilateral pulmonary infiltrates and marked eosinophilia. A transbronchial biopsy specimen did not help clarify the diagnosis. She later developed erythematous nodules on her feet; results of a biopsy specimen revealed necrotizing extravascular granulomas and marked infiltration of ...

We report a case of hypereosinophilic syndrome (HES) with marked eosinophilia (59.7%) and mononeuritis multiplex (upper limbs and buttocks). Necrosis of the finger tips was the primary manifestation which simultaneously occurred on both sides. These clinical manifestations were improved dramatically by subsequent steroid therapy. Interestingly, an elevation of serum tumor ...

A case of childhood hemiplegia due to idiopathic hypereosinophilic syndrome is reported. There was no cardiac lesion. The neurological complications associated with hypereosinophilic syndrome and the pathophysiological mechanism of neurotoxicity of human eosinophils are discussed. It is likely that the neurological deficit was due to eosinophilic neurotoxicity.

A patient with eosinophilia-myalgia syndrome developed progressive central nervosa system involvement that did not improve despite discontinuation of L-tryptophan therapy. Neurologic impairment was manifested initially by spastic monoparesis, which was improved by treatment with methyl-prednisolone and hydroxyurea. Recurrence of weakness was accompanied by gait ataxia, dysphagia, and complaints of a ...

The few studies already published about phagocyte functions in Chediak-Higashi syndrome (CHS) has stated that neutrophils present slow rate of bacterial killing but normally ingest microorganisms. In the present study, both phagocytosis and killing of Staphylococcus aureus were verified to be delayed in neutrophils from two patients with CHS when ...

A 7-week-old infant with antecedent otitis media, upper respiratory infection, and aseptic meningitis was diagnosed as having Sweet syndrome. Although this disease usually affects adults, it has been reported in 17 children. This is the youngest reported patient with the disorder to date, and the first in whom the syndrome ...

The authors report a case of eosinophilia-myalgia syndrome with a progressive neuromyopathy. Progressive weakness, myalgia, and dermatitis developed in the patient described after chronic ingestion of high-dose L-tryptophan for insomnia. Laboratory, electrophysiologic, and muscle biopsy results support the diagnosis of an inflammatory myopathy consistent with that of eosinophilia-myalgia syndrome. The ...

A patient with Sweet's syndrome and leukopenia is reported. Hematological evaluation revealed hairy cell leukemia (HCL). The clinical picture was dominated by persistent fever, which is a common feature of both Sweet's syndrome and HCL. Since fever frequently reflects concomitant infection in HCL, a thorough search for infectious disease was ...

We report a case of Sweet's syndrome which responded to treatment with cyclosporin 4 mg/kg/day. Cyclosporin is effective in the treatment of many dermatoses, and although the immunosuppressive effects of cyclosporin are mainly attributed to inhibition of T-lymphocyte proliferation, it has also been shown to affect the functions of both ...

In a case of Sweet's syndrome (acute febrile neutrophilic dermatosis) neutrophilic alveolitis was found by lung biopsy. Pulmonary disease in this condition may be underrecognised yet, like the dermatological features, it responds rapidly to corticosteroids.

Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, ...

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 ...

We report a 45-year-old female who had symptomatic gastrointestinal involvement, eosinophils in the cellular infiltrate, and who proved to have L-tryptophan-associated eosinophilia-myalgia syndrome. This case illustrates that gastrointestinal disease can be a major, seemingly primary clinical presentation in this syndrome, and that a drug history, specifically L-tryptophan, needs to be ...

Eosinophilia-myalgia syndrome (EMS) is a disorder characterized by generalized muscle pain and eosinophilia. The etiology of this syndrome appears to be related to the ingestion of L-tryptophan. Most studies to date describe an associated peripheral neuropathy or combined myopathy and peripheral neuropathy. This report presents 2 EMS patients with myopathy, ...

Eosinophilia-myalgia syndrome (EMS) is a newly described syndrome associated with use of L-tryptophan. A neuropathy with features of axonal degeneration has also been described in conjunction with EMS. Demyelinating polyneuropathy is not a well recognised association of the syndrome. The two patients with EMS reported presented with profound weakness and ...

Sweet's syndrome (acute febrile neutrophilic dermatosis) and erythema nodosum are reactive dermatoses whose pathogenesis is currently undefined. Although there are several associated conditions that both of these disorders have in common, the appearance of Sweet's syndrome and erythema nodosum in the same person is rare. Only 8 individuals with biopsy ...

A 34-year-old man with Sweet's syndrome associated with subacute necrotizing lymphadenitis is reported. Histological examination of an erythematous, painful, cutaneous plaque revealed a dermal interstitial neutrophilic infiltrate. A biopsy specimen obtained from an inguinal lymph node showed granulomatous formation, consisting of histiocytes, with central necrosis in the paracortex and macrophages ...

We describe the histopathologic changes of skin, muscle, vessels, and fascia in 11 patients with eosinophilia myalgia syndrome, a newly described entity that has been linked to the ingestion of L-tryptophan. This syndrome is defined clinically by severe incapacitating myalgias and a peripheral eosinophilia. Arthralgias, edema of the extremities, morbilliform ...

Here we describe four young Japanese women aged 25-33 years, whose clinical findings are characterized by episodic angioedema, marked leukocytosis with eosinophilia, benign course with spontaneous remission or low-dose prednisolone treatment. The recognized causes of eosinophilia, such as allergy, parasite, and collagen diseases, and the causes of edema, such as ...

OBJECTIVE: To investigate the relationship between L-tryptophan (LT) ingestion and eosinophilic fasciitis (EF) occurring prior to the outbreak of eosinophilia-myalgia syndrome in 1989. METHODS: Interviews and record reviews of 45 EF case-patients and 126 polymyositis patients (controls) diagnosed prior to 1988. RESULTS: Nine case-patients (20%) and no controls recalled taking ...

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 ...

Antineutrophil cytoplasmic autoantibodies are a useful diagnostic serologic marker for a variety of well-known vasculitic syndromes, including Wegener's granulomatosis, polyarteritis nodosa (especially microscopic polyarteritis nodosa), Churg-Strauss syndrome, and pulmonary-renal syndrome with alveolar capillaritis. Although most patients with antineutrophil cytoplasmic autoantibody-associated disease have systemic disease, disease limited to one organ does ...

OBJECTIVE: To review recent advances in the understanding of the cause and pathogenesis of the eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan. DATA SOURCES: Studies published from 1989 to 1991 were identified using a MEDLINE literature search. Additional references were selected from the bibliographies of identified articles. DATA SYNTHESIS: The ...

The iatrogenic L-tryptophan-induced eosinophilia-myalgia syndrome, often considered to be a "new" disease, has proven to be a remarkable mimic of the classic sclerosing rheumatologic disorders. Although subacute cutaneous lupus erythematosus remains a clinically defined entity, supportive histologic and immunopathologic findings have recently been proposed. Rheumatoid neutrophilic dermatitis needs to be ...

Chemotaxis, phagocytic and intracellular killing activities of polymorphonuclear leukocytes (PMNL) were investigated in vitro in 7 patients suffering from the acute phase of Sweet's syndrome. A moderate but consistent impairment of neutrophilic chemotactic activity (NCA) was revealed in all patients. Intracellular killing of blastospores of Candida albicans was diminished in ...

A comparison of the histopathologic features of Shulman's syndrome (diffuse fasciitis with eosinophilia) and the fasciitis associated with the eosinophilia-myalgia syndrome is presented. The study population consisted of eight biopsy specimens of seven patients with Shulman's syndrome and 11 specimens from 10 patients with eosinophilia-myalgia syndrome. Both groups exhibited inflammatory ...

A 61-year-old Japanese female on hemodialysis developed marked eosinophilia induced by nafamostat mesilate as an anticoagulant for hemodialysis. This is the first case of hypereosinophilic syndrome induced by nafamostat mesilate in a hemodialysis patient. The elevated eosinophil counts (51,900/mm3) are the highest for hemodialysis-associated eosinophilia. This eosinophilia was eliminated after ...

The eosinophilia-myalgia syndrome (EMS) is a recently described systemic disorder distinguished by the development of characteristic muscle spasm, myalgia, neuropathy, and scleroderma-like cutaneous induration. Life-threatening manifestations have included cardiopulmonary and acute ascending neuropathic syndromes. Current evidence indicates that this is a severe illness with the potential for long-term disability. Careful ...

We report a 52-yr-old man with ulcerative colitis who developed sulfasalazine-induced pulmonary infiltration with eosinophilia (PIE syndrome), which resolved completely after withdrawal of this drug. Desensitization to sulfasalazine was successful, and allowed the patient to receive this drug without recurrence of the pulmonary toxicity. This is the first case of ...

OBJECTIVES: To study the incidence of eosinophilia-myalgia syndrome, the risk factors associated with the syndrome, and the clinical spectrum of illness associated with L-tryptophan use in an exposed population. DESIGN: Retrospective cohort and nested case-control studies of risk factors for eosinophilia-myalgia syndrome using inpatient and outpatient chart reviews, telephone interviews, ...