Sweet's syndrome with pregnancy

A 31-year-old pregnant woman had eruptions on her wrist, face and neck. We diagnosed her as having Sweet's syndrome from clinical symptoms, histopathological and laboratory findings. We successfully treated her with prednisolone and there are no relapses after delivery. We studied her polymorphonuclear leukocyte activity by the polarization assay with N-formyl-methionyl-leucyl-phenylalanine as a chemoattractant and it is higher than that of control. It is known that Sweet's syndrome is accompanied with collagen diseases and malignant diseases or others as underlying diseases or conditions. Three cases of Sweet's syndrome associated with pregnancy have been reported before and this is the first one in Japan.     

Sweet's syndrome with neurologic manifestations in a patient with esophageal adenocarcinoma: case report and review of the literature

Background  Sweet's syndrome, also known as febrile neutrophilic dermatosis, can occur in patients with an underlying malignancy and can present with extracutaneous manifestations, including neurologic symptoms.
Methods  This report describes a 62-year-old man with adenocarcinoma of the esophagus who developed Sweet's syndrome and whose postoperative course was complicated by encephalitis.
Results  A diagnosis of Sweet's syndrome with neurologic manifestations was made, and the patient was treated with oral corticosteroids. His symptoms improved markedly within 12 h.
Conclusion  Neurologic symptoms in Sweet's syndrome are infrequently reported and have not been described previously in a patient with adenocarcinoma of the esophagus.     

SWEET''S SYNDROME ASSOCIATED WITH ADENOCARCINOMA OF THE PROSTATE

Sweet's syndrome can occur in association with malignancy, in particular acute myeloid leukaemia. It has rarely reported with solid tumours. A case of recurrent Sweet's syndrome in a 75 year old man with metastatic prostatic carcinoma is presented.     

Sweet’s Syndrome

Sweet's syndrome was originally described in 1964 by Dr Robert Douglas Sweet as an 'acute febrile neutrophilic dermatosis'. The syndrome is characterized by pyrexia, elevated neutrophil count, painful red papules, nodules, plaques (which may be recurrent) and an infiltrate consisting predominantly of mature neutrophils that are diffusely distributed in the upper dermis. In addition to skin and mucosal lesions, Sweet's syndrome can also present with extra-cutaneous manifestations. Sweet's syndrome can be classified based upon the clinical setting in which it occurs: classical or idiopathic Sweet's syndrome, malignancy-associated Sweet's syndrome and drug-induced Sweet's syndrome. Systemic corticosteroids have been considered the 'gold standard' for the treatment of patients with Sweet's syndrome; in addition, treatment with topical and/or intralesional corticosteroids may be effective as either monotherapy or adjuvant therapy. However, spontaneous resolution of the symptoms and lesions has occurred in several patients with Sweet's syndrome for whom disease-specific therapeutic intervention was not initiated and in some of the patients with drug-induced Sweet's syndrome after withdrawal of the dermatosis-causing medication. Oral therapy with either potassium iodide or colchicine typically results in rapid resolution of Sweet's syndrome symptoms and lesions; therefore, in patients with Sweet's syndrome who have a potential systemic infection or in whom corticosteroids are contraindicated, it is reasonable to initiate treatment with these agents as a first-line therapy. Indomethacin, clofazimine, dapsone, and cyclosporine have also been effective therapeutic agents for managing Sweet's syndrome. However, indomethacin and clofazimine appear less effective than corticosteroids, potassium iodide, and colchicine. Appropriate initial and follow-up laboratory monitoring is necessary when treating with either dapsone or cyclosporine because of the potential for severe adverse drug-associated effects. Systemic antibacterials with activity against Staphylococcus aureus frequently result in partial improvement of Sweet's syndrome lesions when they are impetiginized or secondarily infected. In some patients with dermatosis-associated bacterial infections, organism-sensitive specific systemic antibacterials have been helpful in the management of their Sweet's syndrome. Although patients with hematologic malignancy-associated Sweet's syndrome often receive cytotoxic chemotherapy agents and antimetabolic drugs for the treatment of their underlying disorder, these agents are seldom used solely for the management of the symptoms and lesions of Sweet's syndrome. The treatment of patients with Sweet's syndrome with either etretinate or interferon-alpha have been reported as single case reports; both patients had improvement of not only their Sweet's syndrome lesions, but also their associated hematologic disorder.     

Acute febrile neutrophilic dermatosis (Sweet's syndrome): a study of 15 cases in Iran

BACKGROUND: Sweet's syndrome is well recognized by dermatologists and is not infrequently diagnosed in Iran; however, few case series have been reported. METHODS: A clinicopathologic study was carried out on 15 cases of Sweet's syndrome in Hamedan, in the west of Iran, between 1994 and 2004. RESULTS: The incidence of Sweet's syndrome was 3 per 10,000 amongst new dermatologic patients. All were women with a mean age of 58 years. All 15 cases were of the classic type, with no associated diseases. Fever was seen in only six (40%) patients. CONCLUSIONS: Sweet's syndrome has a broad spectrum of clinical and pathologic findings in various areas of the world. Further investigation is necessary to determine the etiology and effect of environmental factors in this disease.     

Acute febrile neutrophilic dermatosis--a marker of malignancy?

A retrospective survey during a 2-year period disclosed 18 patients with acute febrile neutrophilic dermatosis (Sweet's syndrome). An associated lympho- or myeloproliferative malignancy was found in 6 patients. Attacks of Sweet's syndrome preceded the diagnosis of neoplasia in 4 patients (3 months to 6 years). Some differences in symptoms and signs were found in the group of patients with associated malignancy compared with the group without, that is, male predominance, mucosal symptoms, anemia, and frequent recurrence of skin symptoms. The onset of Sweet's syndrome indicates an acute infectious disease, and the patients are frequently referred to departments of internal medicine and infectious diseases. In addition, the skin lesions may mimic those which often accompany a generalized infection (erythema multiforme, erythema nodosum, vasculitis, pustular eruptions and urticaria). Since Sweet's syndrome may precede the possibly associated malignant disease, the initial diagnosis of the syndrome is important and should be made with confidence with increasing awareness of the characteristic symptoms.     

Herpetiform Sweet's syndrome and leukaemia

A patient who developed atypical vesicular Sweet's syndrome (SS) resembling herpes simplex virus infection, during relapse of acute myeloid leukaemia is presented. The morphologic and histologic spectrum of SS is discussed, alerting dermatologists to atypical varients. The association with malignancy, particularly of myeloprliferative origin, is highlighted, as are features of SS that suggest the presence of an underlying malignancy.     

Sweet's syndrome with bullous lesions

We present a case of Sweet's syndrome with atypical lesions, characterized by erythematous plaques, vesicles and bullous lesions. Skin lesions in patients with an underlying malignancy are more frequently atypical and with vesicular, bullous or even ulcerative characteristics, in addition to the typical plaques and nodules. However, the case presented is not associated with malignancy, despite the fact that these processes, particularly hematologic ones, should be suspected.     

Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.     

Sweet's syndrome revisited: a review of disease concepts

Sweet's syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by a constellation of symptoms and findings: fever, neutrophilia, erythematous and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, and prompt improvement of both symptoms and lesions after the initiation of treatment with systemic corticosteroids. Hundreds of patients with this dermatosis have been reported. The manifestations of Sweet's syndrome in these individuals have not only confirmed those originally described by Dr Robert Douglas Sweet in 1964, but have also introduced new features that have expanded the clinical and pathologic concepts of this condition. The history, clinical characteristics, laboratory findings, associated diseases, pathology, and treatment options of Sweet's syndrome are reviewed. The evolving and new concepts of this dermatosis that are discussed include: (i) Sweet's syndrome occurring in the clinical setting of a disease-related malignancy, or medication, or both; (ii) detection of additional sites of extracutaneous Sweet's syndrome manifestations; (iii) discovery of additional Sweet's syndrome-associated diseases; (iv) variability of the composition and/or location of the cutaneous inflammatory infiltrate in Sweet's syndrome lesions; and (v) additional efficacious treatments for Sweet's syndrome.     

Acute febrile neutrophilic dermatosis (Sweet's syndrome). Response to dapsone

A 33-year-old woman had many tender, erythematous and pustular plaques on her face, neck, and extremities. Biopsy findings were consistent with a diagnosis of Sweet's syndrome. A chemotaxis test disclosed increased chemotactic activity of the peripheral blood polymorphonuclear neutrophil leukocytes. The patient was successfully treated with dapsone. To the best of my knowledge, this is the first reported case of Sweet's syndrome in which dapsone therapy was used successfully.     

Sweet's syndrome: clinicopathological features of patients treated from 1997 to 2009 at Cassiano Antonio Moraes University Hospital - Vitoria (Espirito Santo)

Sweet's syndrome or acute febrile neutrophilic dermatosis is rare in Brazil. It is clinically characterized by painful erythematous nodules, papules or plaques that occur mainly on the neck and upper limbs. Its cause may be unknown (idiopathic form) or it may be associated with malignancies, usually hematologic, or drugs. The authors describe 16 cases of the syndrome. The median age was 36 years, and all patients were white and presented solid lesions, predominantly on the upper limbs and trunk. Histopathological examination of the dermis of all patients revealed predominance of moderate to intense, superficial and deep, diffuse inflammatory infiltrate, mainly consisting of polymorphonuclear neutrophils, with leukocytoclasia. It also revealed changes in the epidermis and hypodermis (neutrophilic hypodermitis), but with no signs of vasculitis in most patients, which was not considered an important finding for diagnosis. Presence of exocytosis of neutrophils was common, favoring the diagnosis of Sweet's syndrome when accompanied by diffuse interstitial neutrophilic dermatitis.     

Sweet's syndrome and polycythaemia rubra vera

Sweet's syndrome is associated with haematological malignancy, particularly acute myelogenous leukaemia, but there are few reports of its association with polycythaemia rubra vera. We describe an 85-year-old man with polycythaemia rubra vera who developed Sweet's syndrome and review the literature of this association.     

Sweet's syndrome associated with Crohn's disease

The first known case involving an association of Sweet's syndrome with Crohn's disease is described. A 36-year-old woman developed a diarrhea, fever, and infiltrated erythematous cutaneous plaques on neck and limbs, consistent with a presumptive diagnosis of Sweet's syndrome. This was confirmed by a skin biopsy showing a dense dermal infiltrate of polymorphonuclear leukocytes. Crohn's disease, extending from the anus to the terminal ileum, was diagnosed as well. Prednisolone treatment resulted in the improvement of both the bowel disease and skin lesions.     

Lymphocytic infiltrates as a presenting feature of Sweet's syndrome with myelodysplasia and response to cyclophosphamide

Sweet's syndrome has a well-recognized association with malignancies, around half of which have been acute myelogenous leukaemia. There are also numerous reports of Sweet's syndrome in association with myelodysplasia. We report two patients with Sweet's syndrome in whom the classical histological appearances were preceded by dermal lymphocytic infiltrates. A literature search using PubMed indicates that this phenomenon has not been previously reported. The cases demonstrate the chronicity of Sweet's lesions in association with haematological disease and the need for repeat biopsies to make the diagnosis. We also describe successful treatment with cyclophosphamide, which adds to the list of second-line drugs that may be used in Sweet's syndrome.     

Scrofuloderma and Sweet's syndrome

BACKGROUND: In recent years, the rare association of Sweet's syndrome with nontuberculous mycobacterial lymphadenitis has been reported. OBJECTIVE: To report the clinical, demographic, and bacteriologic data and association with Sweet's syndrome of 18 patients with scrofuloderma and scrofuloderma-like condition caused by nontuberculous mycobacterial infections seen during the past 7 years (1994-2000). METHODS: In all patients, a biopsy specimen was obtained for histopathologic and microbiologic studies. Patients from whom Mycobacterium tuberculosis and nontuberculous mycobacteria were isolated from the culture of skin biopsy specimens were included. Deep fungal infection was excluded by the lack of a fungal element in histologic section and cultural methods. The patients were treated with antimicrobials or antituberculous drugs according to the causative species. RESULTS: Eighteen cases of scrofuloderma (nine male, nine female; mean age, 36.9 years) were found among 104 patients with cutaneous tuberculosis and nontuberculous mycobacterial cutaneous infections. Sixteen of the 18 cases had lymphadenitis as the underlying focus of scrofuloderma: 15 cases occurred in the cervical group and one case in the inguinal area. One case drained from the soft tissue and one from the paranasal air sinus. Five cases had multiple episodes of Sweet' s syndrome during the course of treatment. Most cases in this group (four of the five) were middle-aged women with cervical lymphadenitis, and the most common species were rapid growers. CONCLUSIONS: Age, sex, and the site of infection may have some influence on the association with Sweet's syndrome in nontuberculous mycobacterial infections.     

Histiocytoid neutrophilic dermatoses and panniculitides: variations on a theme

Requena et al, in their article titled "Histiocytoid Sweet syndrome," in 2005, established that the dermal infiltrate in some patients with Sweet's syndrome is composed of histiocyte-like immature myeloid cells, not polymorphonuclear leukocytes as is the norm. With this premise in mind, we report on 6 cases of inflammatory skin disease in which the common denominator was a dermal and/or subcutaneous infiltrate of histiocytoid myeloid cells in patients with new-onset cutaneous eruptions and systemic symptoms. The cases were diverse clinically and microscopically, fell short of the criteria necessary for a diagnosis of classical Sweet's syndrome, and were difficult to categorize at the outset. The systemic manifestations ranged from malaise alone to a combination of fever, chills, night sweats, and polyarthralgia. The clinical morphology of the cutaneous eruptions varied from being papulovesicular in 1 patient to mainly consisting of erythematous plaques and nodules in the remainder. The dermatologists' differential diagnoses included Sweet's syndrome in 3 cases, a drug eruption in 2, and other entities such as erythema nodosum and Well's syndrome. Biopsies in all cases revealed a dermal and/or subcutaneous infiltrate composed predominantly of mononuclear histiocytoid cells of myeloid origin. With the benefit of detailed clinicopathologic correlation, the cases were classified for the purpose of this report as follows: Sweet's-like neutrophilic dermatosis, histiocytoid (3 cases); subcutaneous Sweet's syndrome, histiocytoid (2 cases); histiocytoid neutrophilic dermatosis, unspecified (1 case). In addition, we describe a further instructive case that exhibited overlap with those in the series but proved ultimately to represent leukemia cutis. The spectrum of observations in this report supports and expands the original concept of histiocytoid Sweet's syndrome.     

Sweet''s Syndrome

Sweet's syndrome (acute neutrophilic dermatosis) is characterized by fever; polymorphonuclear neutrophilic leukocytosis; elevated ESR; and characteristic, raised, painful red plaques on the face and limbs. Since Sweet first described the syndrome in 1964, more than 150 cases have been reported, but it is assumed that the disorder is far more common than this number would indicate.     

Sweet's syndrome in patients with kidney and liver disorders.

Two patients with Sweet's syndrome are described. In the first patient the disorder was associated with urinary stone disease and in the second with chronic hepatitis. We compared skin lesions in patients with kidney and liver diseases with those in Sweet's syndrome. In both patients all skin manifestations disappeared when the underlying disorders were treated. No corticosteroids were used.     

Impairment of some granulocyte functions in Sweet's syndrome.

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 5/7 patients. Phagocytosis and oxidase activities were within normal levels. These results point to an alteration of some PMNL functions in the acute phase of Sweet's syndrome.