Approximately 10-20% of the reported patients with acute febrile neutrophilic dermatosis (Sweet's syndrome) have an associated neoplasm. Oral findings of Sweet's syndrome are rarely reported, and no cases in patients with oral cancer have been reported to date. This report describes the clinico- and histopathological findings of Sweet's syndrome in ...

Thirty-nine patients with Sweet's syndrome seen at the National Skin Centre in Singapore between 1982 and 1994 were reviewed. Diagnosis was based on the presence of acute tender, erythematous plaques and nodules, and histologically on a dense dermal infiltrate composed predominantly of neutrophils. The female to male ratio was 1.6:1. ...

Sweet first described acute febrile neutrophilic dermatosis in 1964. Since then, more than 425 cases of this typically benign, steroid-responsive disease have been recorded. Although often associated with myelodysplasic syndromes or hematologic malignancies, Sweet's syndrome has also been related to pregnancy, autoimmune disorders, and many drug therapies. Although it is ...

A case is presented of Churg-Strauss syndrome in a young man in whom the definitive diagnostic procedure was a full thickness sigmoidoscopic rectal biopsy, with submucosal sampling. Gastrointestinal changes in Churg-Strauss syndrome, a rare systemic illness characterised by asthma, blood and tissue eosinophilia, vasculitis, and granulomatous inflammation are common but ...

Wegener's granulomatosis and Churg-Strauss syndrome are the predominant pulmonary vasculitides. Next in frequency are the various diffuse alveolar haemorrhage syndromes, which may be related to the antineutrophil cytoplasmic autoantibody (ANCA)-associated diseases, such as Wegener's granulomatosis and Churg-Strauss syndrome, or may be a part of a collagen vascular disease, such as ...

Taken together, these studies suggest that many different etiologic agents alone or together may initiate the common final pathways of tissue pathologic response resulting in the clinical syndrome of eosinophilia, myalgias and fasciitis. Tryptophan itself may contribute to some of the scarring features of the illness, while impure L-tryptophan, and ...

BACKGROUND: Schnitzler's syndrome is a rare disorder characterized by chronic urticaria and monoclonal IgM gammopathy. The mechanisms of the urticarial flares remain poorly understood. OBJECTIVE: To more accurately define the histopathologic changes in urticarial lesions, we reviewed 25 original biopsies from 15 cases of Schnitzler's syndrome, 11 of which have ...

This article examines the methodology of the epidemiological studies of the association between L-tryptophan and eosinophilia-myalgia syndrome (EMS) and evaluates the validity of the conclusions from these studies. In the initial case-control studies of L-tryptophantryptophan and EMS there were a variety of methodological problems, including different sources and different exclusion ...

We describe a 7-year-old boy with Churg Strauss syndrome who presented with a 3-month history of cough, wheeze, fever, weight loss, abdominal pain, skin lesions, proteinuria and pulmonary infiltrates with eosinophilia. He showed a good response to corticosteroid treatment and is currently doing well. The case illustrates the difficulty and ...

The pathogenesis of vasculitis syndromes is via a diverse array of mechanisms that disrupt vessel integrity and produce clinical signs and symptoms of tissue ischemia. In many patients with vasculitis, it is not possible to directly classify the syndrome according to a particular schema, and attempts to define the pathogenic ...

The similarity of eosinophilia-myalgia syndrome (EMS) and toxic-oil syndrome (TOS) to systemic sclerosis and diffuse fasciitis with eosinophilia (DFE) highlights the potential for environmental agents to induce autoimmune disease. Further, a candidate etiologic agent for EMS, 3-(phenylamino)alanine, is chemically similar to the aniline derivative identified in samples of oil implicated ...

A 50-year-old man with Churg-Strauss syndrome showed granulocytes (GNLs) which generated more superoxide anion (O2-) than GNLs from healthy subjects following in vitro stimulation with C5a or C3a. Production of O2- subsided as the clinical symptoms improved with steroid treatment. A hyperresponsiveness of GNLs may be involved in this disorder.

A 53-year-old man with acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA) developed fever and small erythemas on his arms and legs when the peripheral blood neutrophil count had increased. Erythemas gradually enlarged and painful subcutaneous nodules appeared. Skin biopsy revealed dense neutrophil infiltration in the dermis without ...

We report a 58-year-old patient with acute myeloid leukaemia who developed an oedematous erythema resembling Sweet's syndrome, accompanied by atypical erythema nodosum and bullous pyoderma gangrenosum. Examination of skin biopsies showed dense infiltration with mature neutrophils, although there was peripheral blood leucocytopenia. The oedematous erythema worsened after he was treated ...

Although the tropical pulmonary eosinophilia (TPE) syndrome of filarial aetiology has very distinctive clinical and immunological features, its clinical profile is not unique; other helminths sometimes induce similar presentations. We carefully evaluated 7 individuals with non-filarial TPE-like syndromes and found that serological tests based on detection of 'antifilarial' immunoglobulin (Ig) ...

Vasculitis represent a broad spectrum of disorders involving vessels of different types and characterized by various clinical manifestations. The diagnosis of cutaneous vasculitis is usually easy and based on clinical and histological features. A number of classification systems of vasculitis have been proposed. In this review we define small vessel ...

The hypereosinophilic syndrome is a multisystem syndrome characterized by peripheral blood eosinophilia and eosinophil infiltration of bone marrow, heart, and other organs. The syndrome is associated with cardiac, hematological, pulmonary, neurological, and cutaneous involvement and, if untreated, has a high fatality rate. Criteria for the diagnosis of hypereosinophilic syndrome include ...

Sweet, in 1964, described a skin condition characterized by fever, leukocytosis, tender erythematous plaques, and, histopathologically, a predominantly neutrophilic dermal inflammation. However, other dermatologic conditions can present with similar clinical and histological features. Therefore, diagnostic criteria are important for the correct diagnosis. Use of systemic steroids is the treatment of ...

We present the rare case of a 62-year-old woman with Churg-Strauss syndrome associated with a left third nerve palsy and a mononeuritis multiplex. Cranial nerve palsies are unusual in patients with Churg-Strauss syndrome, and the most frequent cranial nerve lesion observed is an ischemic optic neuritis. Third nerve involvement secondary ...

We report the case of a 52-year-old woman with a non-tuberculous (atypical) mycobacterial cervical lymphadenitis, caused by Mycobacterium fortuitum, in association with Sweet's syndrome. The cervical lymphadenitis was resistant to medical treatment, and the Sweet's syndrome occurred intermittently. Systemic steroid treatment was required to control the cutaneous symptoms.

A variety of musculoskeletal syndromes has been described in association with numerous parasitic infestations. Arthritis, enthesitis, myositis and vasculitis have been described in infected individuals resident in, or visitors to, endemic areas. The diagnosis of parasitic rheumatism is supported by poor response to anti-inflammatory drugs and improvement following antiparasitic treatment.

A series of 10 cases of Sweet′s syndrome observed during a period of 3 years in Eastern parts of Libya is reported. All the patients were females. Fever and peripheral neutrophilia were not found in all these cases. Dapsone was found to be very effective in the cases in which ...

Urticarial vasculitis is difficult to treat. We report here on a 40-year-old woman with a 16-year history of idiopathic hypocomplementemic urticarial vasculitis syndrome. Her disease had been resistant to treatment with H1- and H2-blockers, indomethacin, dapsone and interferon alpha but responded to > 25 mg/day prednisolone. Monotherapy with pentoxifylline was ...

We report a case of Sweet's syndrome in a patient in whom an acute myeloblastic leukemia was subsequently diagnosed. Approximately 10-20% of the reported cases of Sweet's syndrome occur in patients with a malignancy, the neoplastic condition most commonly reported being acute myelogenous leukemia. This case is of interest because ...

The eosinophilia-myalgia syndrome (EMS) is a rare systemic disease caused by presumably contaminated L-tryptophan. Thirteen outpatients with EMS were found to have a high degree of depression, anxiety, and difficulty adjusting to illness. Pre-EMS history of major depression but not EMS severity predicted poor adjustment to illness.

The hypereosinophilic syndrome is characterized by a long-lasting increase in circulating eosinophils in the absence of a definable etiology and by manifestations of multisystem involvement. It must be differentiated from the eosinophilia-myalgia syndrome related to the ingestion of L-tryptophan, although the clinical features may be similar. Two patients with hypereosinophilia ...

Eosinophilia-myalgia syndrome, a recently described illness, reached epidemic proportions in 1989 and was linked to the ingestion of L-tryptophan containing trace amounts of several contaminants. Eosinophilia-myalgia syndrome shares many clinical and pathologic similarities with toxic-oil syndrome, an epidemic linked to the ingestion of adulterated cooking oil that occurred in Spain ...

Acute febrile neutrophilic dermatosis, first described in 1964 by Robert Douglas Sweet, has been termed Sweet's syndrome. Classic Sweet's syndrome occurs in middle-aged women after a nonspecific infection of the respiratory or gastrointestinal tract. Raised erythematous plaques with pseudoblistering and occasionally pustules occur on the face, neck, chest, and extremities, ...

Minocycline is a tetracycline agent frequently used for acne therapy. It has a few rare but severe side effects that are not widely known but should be recognized early as drug related. These include acute hepatitis and liver failure; a Löffler-like syndrome with pulmonary infiltrates, wheezing, fever, and eosinophilia; skin ...

An epidemic of a new disease, termed eosinophilia-myalgia syndrome, occurred in the USA in 1989. This syndrome was linked to the consumption of L-tryptophan manufactured by a single company utilizing a fermentation process. All the findings indicate that the illness was probably triggered by an impurity formed when the manufacturing ...

A recently recognized disease, the eosinophilia-myalgia syndrome, is described and presented as a new condition attributable to nutritional toxicology. Its etiology is related to the ingestion of L-tryptophan, manufactured by a single Japanese supplier who had modified its production system, which, though of high purity, contained in minute concentrations a ...

A 2.5-month-old infant had Sweet syndrome. Chronic granulomatous disease was subsequently diagnosed by the nitroblue tetrazolium reduction test. To date, this infant is the youngest reported with Sweet syndrome. Moreover, the association of chronic granulomatous disease with this syndrome has not been previously described. The precise relationship between the conditions ...

The eosinophilia-myalgia syndrome associated with L-tryptophan-containing products is highlighted by eosinophilia, incapacitating myalgias, and diverse multisystemic manifestations. In addition to involvement of the skin, skeletal muscle, and peripheral nerves, visceral damage has been quite prominent, particularly affecting the lungs, the heart, and the liver. Hepatic involvement has been manifested by ...

BACKGROUND: The clinicopathologic manifestations of Sweet's syndrome associated with malignancy have been rarely studied in a large series. METHODS: We describe the clinical features of Sweet's syndrome in 11 patients seen during the past 12 years. Histopathologic findings of these patients are discussed. The clinicopathologic manifestations of 249 cases of ...

We report on a case of Churg-Strauss syndrome in a 30 year old male with a history of perennial rhinitis. The syndrome began nine months earlier with clinico-radiological features typical of chronic eosinophilic pneumonia. Diagnosis of vasculitis was made by skin biopsy, and at that time there was multisystemic involvement ...

Eosinophilic pulmonary infiltration is an uncommon presentation from cocaine abuse. We present a patient with migratory pulmonary infiltrates and eosinophilia consistent with Löffler's syndrome. Our review uncovered two other similar patients. The importance of early recognition is stressed in our report. Either abstinence from cocaine usage and/or steroids result in ...

A remarkable infiltration of eosinophils was observed in skin biopsy specimens in two cases of Sweet's syndrome. One patient was a 29-year-old woman, whose clinical and histological features included associated asthma, a prior respiratory tract infection, red plaques, blood eosinophilia, and a dense dermal infiltrate of neutrophils and eosinophils, without ...

A case of Churg-Strauss syndrome with multiple perforations of the small intestine is described. A 31-year-old woman was admitted with a complaint of epigastric pain. She had a history of bronchial asthma. One week before admission, white blood cell count was 20,800/mm3 with 59% eosinophils. Neurological examination on admission disclosed ...

A patient with aplastic anaemia developed Sweet's syndrome (a febrile neutrophilic dermatosis) during granulocyte colony-stimulating factor (G-CSF) therapy. Three repeated episodes of appearance and disappearance of erythematous nodules after administration and withdrawal of G-CSF confirmed that G-CSF induced Sweet's syndrome in the patient. Sweet's syndrome has been reported in patients ...

Three patients with eosinophilia-myalgia syndrome linked to consumption of L-tryptophan supplement developed a severe sensorimotor axonal neuropathy. All three had myalgia, elevated eosinophil count, and later developed fasciitis. Neuropathy was found at all stages of the illness and resulted in disability which was irreversible despite cessation of L-tryptophan. Nerve conduction ...

The classic (giant cell) temporal arteritis occasionally coexists with other systemic vasculitides or one of the connective tissue diseases, and occasionally, necrotizing vasculitis has been diagnosed serendipitously in temporal artery biopsies. The association of the classic (giant cell) temporal arteritis with Churg-Strauss syndrome in the same patient occurs only rarely. ...

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a rare inflammatory disease of unknown cause characterized by painful, erythematous plaques and a dermal infiltrate of mature neutrophils. Associated systemic disease is a common finding in patients with Sweet's syndrome, and in fact determines the prognosis; therefore, the diagnosis of Sweet's syndrome ...

We report a 59-year-old male with Sweet's syndrome who developed small cell carcinoma of the lung one year after the initial diagnosis. To the best of our knowledge, this is the first report of Sweet's syndrome in conjunction with lung cancer. In cases of Sweet's syndrome, a search for not ...

We report a case of leukemia-associated acute febrile neutrophilic dermatosis (Sweet's syndrome) that is unique because its initial histologic findings mimicked leukemia cutis. Otherwise, the clinical manifestations and response to corticosteroid therapy were typical of Sweet's syndrome. The onset of the dermatosis coincided with the onset of neutrophilic differentiation induced ...

A case of acute promyelocytic leukaemia is reported in which Sweet's syndrome developed at the time of an improvement in white blood cell count due to transretinoic acid. Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a disorder characterized by acute onset of inflammatory skin nodules associated with systemic features which ...

A 66-year-old male patient presented, over a 10-year period, polymorphic cutaneous manifestations with extensive neutrophilic infiltration which supports the diagnosis of overlapping syndrome of neutrophilic dermatoses. This was associated with a benign monoclonal gammopathy of IgA lambda type that had antineutrophil cytoplasmic autoantibody (ANCA) activity. Neutrophilic dermatoses may be associated ...

A case of Behçet's disease associated with Sweet's syndrome is presented. The onset was neurological symptoms and oral aphthae. Subsequently, genital ulcers developed. Coexistence of Sweet's syndrome and Behçet's disease has been reported only in few patients. On reviewing the literature, we propose that Sweet's syndrome is one of the ...

As a poorly understood group of disorders of unknown etiology, the idiopathic hypereosinophylic syndrome (HES) is defined by the common clinical features of prolonged eosinophilia of unknown cause and organ system dysfunction due to the eosinophilia. An allergic asthmatic presented with HES involving the submandibular and sublingual glands and the ...

BACKGROUND: Sweet syndrome (acute febrile neutrophilic dermatosis) may occur as a cutaneous paraneoplastic syndrome. This condition has been associated with hematologic malignancies and, to a lesser extent, with solid tumors. METHODS: The authors report two patients with malignancy-associated Sweet syndrome: a 66-year-old man in whom the onset of Sweet syndrome ...

Sweet's syndrome is a rare dermatosis characterized by fever, neutrophilia, raised painful erythematous plaques and a dense dermal infiltrate consisting of mature neutrophils. About 15% of published cases occurs in association with hematologic malignancies, so that the syndrome is considered a paraneoplastic phenomenon; conversely, the association with solid tumors is ...