Sweet's syndrome with gastric cancer

Solid tumors are found in about 15% of malignancy-associated Sweet's syndrome, but only 3 cases of Sweet's syndrome with gastric cancer have been reported. We describe a 59-year-old Japanese man with gastric cancer associated with Sweet's syndrome.     

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.     

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.     

A continuum of neutrophilic disease occurring in a patient with ulcerative colitis

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders, Aiypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological) dyscrasias but not. to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum,; Sweet's syndrome, erythema elevatum diutinum and. subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.     

A case of Sweet's syndrome developed after the treatment of herpes simplex infection in a metastatic breast cancer patient

BACKGROUND: Sweet's syndrome or acute febrile neutrophilic dermatosis is associated with several systemic diseases such as malignancies and infectious diseases. METHODS: We present a 34-year-old woman with Sweet's syndrome associated with both herpes infection and metastatic disease. RESULTS: Skin biopsy showed neutrophilic infiltrates in the dermis confirming the diagnosis of Sweet's syndrome. CONCLUSIONS: To our knowledge, this is the second case of Sweet's syndrome associated with herpes simplex infection in the literature. Further observations are required to determine the relationship between Sweet's syndrome and herpetic infection.     

Diagnostic criteria for Sweet's syndrome

Five patients with Sweet's syndrome with typical clinical and histologic features were reviewed. Attention is drawn to the possible association of drug use, venipuncture and insect bite, resection of colon, exacerbation of sinusitis, and acute myelocytic leukemia with the onset of the skin eruptions in our patients. The question of whether Sweet's syndrome is just a reactive phenomenon or a specific entity is raised. Our opinion is that Sweet's syndrome is a reaction to many different antigens. However, characteristic clinical and histologic features are present to allow a definite diagnosis of Sweet's syndrome. We propose two major criteria and four minor criteria for the diagnosis of Sweet's syndrome. Findings in patients must fulfill both of the major criteria and at least two of the minor criteria to allow a diagnosis of Sweet's syndrome.     

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.     

Acute febrile neutrophilic dermatosis and abnormal bone marrow chromosomes as a marker for preleukemia

Acute febrile neutrophilic dermatosis or Sweet's syndrome is a rare disease, which occasionally is seen in patients with myeloid leukemia. We present a case of Sweet's syndrome in a patient with an abnormal chromosome pattern in bone marrow aspirate. Initially the patient had flu-like symptoms with high fever. Two weeks later raised, erythematous and painful plaques appeared on the skin. Various antibiotics were ineffective, but the symptoms vanished after administration of prednisone. Six months later a fulminant acute myeloid leukemia developed, the course of which was complicated by a fatal subdural bleeding. It is concluded that Sweet's syndrome may be a cutaneous sign of a neoplastic myeloid proliferation and that a complete hematological examination including chromosome analysis is mandatory in these patients.     

Sweet's syndrome and non-Hodgkin's lymphoma: the first report of this association

A 58-year-old woman developed Sweet's syndrome one week after a flu-like illness. She was later found to have a centrocytic/centroblastic non-Hodgkins lymphoma. Six courses of chemotherapy were given during which the lesions of Sweet's syndrome resolved completely. As far as we are aware this is the first report of the association of Sweet's syndrome with a lymphoma.     

Lymphocytic infiltrate of the dermis preceding typical Sweet's syndrome

BACKGROUND: The histologic criteria for Sweet's syndrome consist in prominent oedema of the dermis and a diffuse infiltrate of numerous neutrophils with leukocytoclasis without vasculitis in the superficial and the deep dermis. Several comorbidities have been observed in patients with Sweet's syndrome, particularly hemo-proliferative diseases. PATIENTS: We report the cases of two men aged 60 and 75 years with Sweet's syndrome associated in one case with myelodysplasia and in the other with chronic lymphocytic leukemia. These two patients had typical edematous plaques highly evocative of Sweet's syndrome. However, histological examination revealed superficial and deep perivascular lymphocytic infiltrate in the dermis on 5 occasions before the typical neutrophilic dermatosis of Sweet's could be diagnosed after respectively 2 and 4 years of progression. DISCUSSION: Histological findings in Sweet's syndrome are characteristic and constitute a major diagnostic factor. However, these two cases show that a lymphocytic infiltrate can occur months or even years before the appearance of typical neutrophilic infiltrate in patients with Sweet's syndrome.     

Sweet''s syndrome in association with generalized granuloma annulare in a patient with previous breast carcinoma

We describe a case of Sweet's syndrome in association with generalized granuloma annulare (GA) which presented 3 years after a diagnosis of breast carcinoma. Both Sweet's syndrome and generalized GA have been described independently in association with breast carcinoma but have never been described together. Both conditions appeared to respond to oral clofazimine.     

Increased plasma chemoattractant in Sweet''s syndrome

The neutrophil function and plasma leukotactic activity of a patient with Sweet's syndrome and cystonodular acne were evaluated during a 2 1/2-year period. These studies demonstrated that chemotaxis was frequently slightly increased, especially during an exacerbation of Sweet's syndrome, but showed some decrease during isotretinoin therapy. Other functions, such as phagocytosis, metabolic activation, and bacterial killing, also were slightly increased. In addition, the patient's serum contained a heat-stable, nonlipid chemoattractant that was present at all times except during a course of isotretinoin. Although his symptoms responded to aspirin, the plasma continued to show this chemoattractant. These findings are consistent with the hypothesis that excess chemoattractant in Sweet's syndrome attracts neutrophils, which then mediate an inflammatory response. In addition, aspirin may be used to control Sweet's syndrome symptoms, although it does not suppress the plasma chemoattractant.     

Familial Sweet's syndrome in 2 brothers,both seen in the first 2 weeks of life

Sweet's syndrome (or acute febrile neutrophilic dermatosis) is a rare inflammatory disease that is characterized by fever, neutrophilia, and painful erythematous plaques that histologically show a dense dermal infiltrate of neutrophils without associated vasculitis. We describe 2 neonates (10 and 15 days of age) with Sweet's syndrome; to our knowledge, this is the first reported case of siblings with Sweet's syndrome.     

Myelofibrosis Discovered After Diagnosis of Sweet''s Syndrome

Multiple edematous plaques and nodules suddenly developed on the face and neck of a 26-year-old man whose physical examination was said to be normal Because the patient's condition fulfilled the diagnostic criteria of Sweet's syndrome, a further general medical examination and hematologic evaluation were performed. Extensive myelofibrosis was revealed by bone marrow biopsy. This case supports the belief that Sweet's syndrome is a reactive phenomenon. Because myelofibrosis was discovered in this patient after Sweet's syndrome was diagnosed, the importance of having the diagnostic criteria of Sweet's syndrome is emphasized. Careful systemic evaluation is indicated, especially when cutaneous lesions are severe or hematologic values are abnormal.     

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.     

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.     

Bullous Sweet's syndrome in congenital neutropenia: association with pegfilgrastim

Sweet's syndrome is an acute febrile neutrophilic dermatosis marked by attacks of painful, plaque-forming inflammatory papules accompanied by fever, arthralgias, peripheral leukocytosis, a diffuse dermal neutrophilic infiltrate, and prompt resolution of symptoms and lesions with glucocorticoid therapy. There are many reports of drug-induced Sweet's syndrome to various medications including all- trans -retinoic acid, carbamazepine, hydralazine, levonorgestrel/ethinyl estradiol, minocycline, trimethoprim/sulfamethoxazole, and granulocyte colony-stimulating factor. We describe the first known case of Sweet's syndrome induced by pegfilgrastim, a pegylated form of granulocyte colony-stimulating factor with unique pharmacologic properties that may induce Sweet's syndrome in patients with no history of neutrophilic dermatoses associated with granulocyte colony-stimulating factor therapy.     

An immunohistochemical study of the dermal infiltrate and epidermal staining for interleukin 1 in 12 cases of Sweet's syndrome

Summary Interleukin 1 (IL-1) has been proposed as a possible mediator in Sweet's syndrome. We examined all cases of Sweet's syndrome (M = 12) presenting to the department over a 10-year period, from 1982 to 1992, for the presence IL-l and also assessed the nature of the dermal inflammatory infiltrate in those cases. Staining for IL-1α and TL-1β was stronger in control tissues than in Sweet's syndrome. This may possibly be explained by the release of IL-1α and IL-1β into the dermis in Sweet's syndrome. Contrary to recent reports, we found that neutrophils predominated in all cases examined, although histiocytes were present in increased numbers indicating their possible role in the pathophysiology of Sweet's syndrome.     

Sweet's syndrome with abscess-like lesions in a patient with acute myelogenous leukemia

We describe a 49-year-old man with acute myelogenous leukemia associated with Sweet's syndrome and abscess-like lesions mimicking an infectious disease. Although blisters may be included in the clinical spectrum, frank non-infectious abscesses have not been reported as far as we know. Clinicians should be familiar with this clinical and histopathologic variant of Sweet's syndrome. It is mandatory to make every effort to find an infectious cause for abscesses before a diagnosis of Sweet's syndrome is made.     

Sweet's syndrome associated with ovarian carcinoma

Sweet's syndrome is sometimes associated with haematological malignancies and less frequently with solid tumors. We report a new case of association of a Sweet's syndrome with an ovarian carcinoma.